Glossary

Neurology

285 entries in this specialty.

Focal brain abscess Purulent infection localized and encapsulated within the brain parenchyma, secondary to hematogenous bacterial dissemination, otolaryngitis contiguity or penetrating head trauma. It manifests clinically with refractory progressive headache, fever and signs of neurological focality depending on the brain area involved.

Also: intracerebral purulent collection · encapsulated brain infection · brain abscess

Transient ischemic attack Brief episode of focal neurological dysfunction caused by focal cerebral, retinal, or spinal cord ischemia, with symptoms typically lasting less than an hour and without evidence of acute infarction on neuroimaging. It constitutes a critical and early predictor of imminent cerebral infarction.

Also: AIT · transient cerebral ischemia · transient ischemic attack

Broca's aphasia Language disorder of a non-fluent type characterized by a marked alteration in the expression of spoken and written language with reading comprehension preserved to a variable degree. It is associated with destructive lesions in the inferior frontal cortex of the dominant cerebral hemisphere.

Also: motor aphasia · non-fluent expressive aphasia · Broca's aphasia

Broca's aphasia with apraxia of speech Severe disorder of oral expression in which the inability to formulate complex grammatical sentences is associated with a marked deficit in voluntary motor planning to carry out the articulation of the phonemes required for fluent speech.

Also: expressive aphasia with articulatory apraxia · combined motor and articulatory impairment · Broca's aphasia with apraxia of speech

Broca's aphasia with a progressive course Non-fluent language disorder of a motor nature that gradually worsens in an insidious manner, characterized by increasing difficulty in the fluency of spoken speech that evolves towards mutism in the context of a neurodegenerative disorder of anterior lobar predominance.

Also: primary progressive motor aphasia · non-fluid variant of the APP · progressive Broca's aphasia

Broca's aphasia with a primary progressive course Non-fluent language disorder of a motor nature that gradually worsens in an insidious manner, characterized by increasing difficulty in the fluency of spoken speech that evolves towards mutism in the context of a neurodegenerative disorder of anterior lobar predominance.

Also: classical primary progressive motor aphasia · non-fluid variant of the progressive APP · primary progressive Broca's aphasia

Broca's aphasia with a classical primary progressive course Non-fluent language disorder of a motor nature that gradually worsens insidiously, characterized by increasing difficulty in the fluency of speech that evolves towards mutism in the context of anterior lobar atrophy of classic neurodegenerative cause.

Also: typical primary progressive motor aphasia · non-fluid variant of the classic APP · classic primary progressive Broca's aphasia

Conduction aphasia Language disorder characterized by a marked and selective difficulty in repeating previously heard words and phrases, in the presence of fluent spontaneous language and reasonably preserved listening comprehension. Pathophysiologically it is associated with lesions in the arcuate fasciculus that communicates the language areas.

Also: associative disconnection aphasia · associative aphasia · conduction aphasia

Wernicke's aphasia Fluid type language disorder characterized by a severe alteration in the understanding of spoken and written words, with fluent but incoherent spoken language plagued by paraphasias or neologisms. It is usually caused by cortical lesions in the posterior portion of the superior temporal gyrus of the dominant hemisphere.

Also: sensory aphasia · fluent receptive aphasia · Wernicke's aphasia

Transcortical Wernicke's aphasia Fluid type language disorder characterized by a severe alteration in language comprehension with fluent but semantically empty language and with the ability to repeat spoken words completely preserved. It is the result of temporo-parietal ischemic lesions that spare the posterior perisylvian area.

Also: sensory transcortical aphasia · receptive aphasia with preserved repetition · transcortical sensory aphasia

Agraphia Total or partial loss of the ability to express ideas or language in writing due to an acquired brain injury, in the absence of peripheral motor or sensory dysfunction of the limb. It is usually associated with damage to Exner's area or the dominant parietal lobe.

Also: acquired writing disorder · agraphia · loss of brain writing

Alexia Acquired loss of the ability to read and understand written language as a result of focal brain damage, despite maintaining normal visual acuity and auditory comprehension. It is classically caused by lesions in the angular gyrus of the dominant hemisphere.

Also: word blindness · acquired alexia · neurological reading disability

Cerebral amyloid angiopathy Disorder characterized by the progressive deposition of beta-amyloid peptide in the walls of the leptomeningeal and medium or small caliber blood vessels of the cerebral cortex. It is one of the most common causes of non-traumatic recurrent lobar hemorrhages in elderly patients.

Also: cerebral vascular amyloidosis · leptomeningeal amyloid angiopathy · AAC

Anosognosia Pathological lack of awareness or ability to recognize the patient's own functional, neurological or cognitive deficits. It is a common neuropsychological alteration after lesions of the right parietal lobe or extensive cerebrovascular accidents.

Also: imperception of the deficit · cognitive negligence · Babinski anosognosia

Friedreich's ataxia Autosomal recessive inherited neurodegenerative disease caused by the expansion of a GAA triplet in the frataxin gene, which causes mitochondrial dysfunction and iron overload. Clinically it presents with progressive gait ataxia, loss of proprioceptive sensitivity, progressive scoliosis and hypertrophic cardiomyopathy.

Also: Friedreich's spinocerebellar degeneration · Friedreich's atrophy · Friedreich ataxia

Friedreich's ataxia with heart disease Hereditary genetic disorder where frataxin deficiency causes neurodegenerative gait impairment systematically combined with progressive concentric hypertrophic cardiomyopathy. It is the most frequent source of mortality in these patients due to arrhythmias or heart failure.

Also: Friedreich's ataxia with myocardial involvement · Friedreich's ataxia with cardiomyopathy · FRDA with cardiomyopathy

Autosomal dominant spinocerebellar ataxia Genetically heterogeneous group of neurodegenerative disorders characterized by the progressive onset of progressive cerebellar ataxia of gait associated with dysarthria, nystagmus and pyramidal or extrapyramidal dysfunction with a disabling course of autosomal dominant transmission.

Also: Hereditary SCA · dominant spinocerebellar degeneration · autosomal dominant spinocerebellar ataxia

Progressive autosomal dominant spinocerebellar ataxia Genetically heterogeneous group of neurodegenerative disorders characterized by the progressive appearance of cerebellar gait ataxia associated with disabling dysarthria of autosomal dominant transmission and slow but continuous evolution.

Also: Progressive autosomal dominant SCA · progressive dominant spinocerebellar degeneration · progressive autosomal dominant spinocerebellar ataxia

Dominant hereditary spinocerebellar ataxia Genetically heterogeneous group of neurodegenerative disorders characterized by the progressive onset of progressive cerebellar gait ataxia of autosomal dominant inheritance.

Also: Autosomal dominant SCA · hereditary dominant spinocerebellar degeneration · hereditary dominant spinocerebellar ataxia

Progressive spinocerebellar ataxia Genetically heterogeneous group of neurodegenerative disorders of autosomal dominant inheritance characterized by slow and irreversible cerebellar atrophy, wide-based gait, cerebellar-type dysarthria and dysmetria in voluntary limb movements.

Also: hereditary spinocerebellar degeneration · spinocerebellar ataxia

Median nerve entrapment in the forearm Neuropathy characterized by focal compression of the median nerve at the level of the pronator teres muscle or at the exit of the pronator tunnel, which causes dull pain on the anterior surface of the forearm and weakness of the intrinsic flexor muscles without involvement of the carpal tunnel.

Also: pronator teres syndrome · proximal median compressive neuropathy · pronator teres syndrome

Common peroneal nerve entrapment Peripheral neuropathy due to mechanical compression of the common peroneal branch at the level of its path around the neck of the fibula bone, causing selective weakness for dorsiflexion and eversion of the foot with consequent walking in steppe.

Also: compression of the external popliteal sciatic nerve · common peroneus neuropathy · common peroneal nerve entrapment

Multiple system atrophy Neurodegenerative disease characterized by the pathological accumulation of alpha-synuclein in glial cells and clinically characterized by a variable combination of severe dysautonomia, parkinsonism and cerebellar signs. It is divided into subtypes depending on whether extrapyramidal or cerebellar symptoms predominate.

Also: AMS · Shy-Drager syndrome · MSA

Spinal muscular atrophy Group of hereditary neuromuscular disorders of autosomal recessive inheritance caused by mutations in the SMN1 gene, which lead to progressive degeneration of the motor neurons of the spinal anterior horn. It is characterized by predominantly proximal symmetric muscle weakness, generalized hypotonia, and osteotendinous areflexia.

Also: AME · spinal motor neuron atrophy · SMA

Distal spinal muscular atrophy Group of rare genetic neuromuscular disorders characterized by progressive weakness, amyotrophy and loss of reflexes located exclusively in the distal musculature of the upper and lower extremities due to symmetrical motoneuronal degeneration of the anterior horn.

Also: hereditary distal amyotrophy · dSMA · distal spinal muscular atrophy

Adult distal spinal muscular atrophy Rare neuromuscular genetic disorder characterized by progressive weakness, amyotrophy and loss of reflexes located exclusively in the distal musculature of the upper and lower extremities that debuts in the third decade of life.

Also: hereditary adult distal amyotrophy · adult dSMA · adult distal spinal muscular atrophy

Spinal muscular atrophy type I More severe and early-onset form of spinal muscular atrophy, which debuts before six months of age with severe generalized flaccid hypotonia, absence of cephalic control and manifest bulbar weakness that prevents oral feeding and prolonged spontaneous breathing.

Also: Werdnig-Hoffmann disease · severe spinal hypotonia of the infant · spinal muscular atrophy type 1

Childhood spinal muscular atrophy type I More severe and early form of neuromuscular atrophy that debuts before six months of age with severe generalized flaccid hypotonia, complete absence of head control and progressive respiratory difficulty due to the destruction of anterior motor neurons.

Also: Werdnig-Hoffmann disease of the infant · early spinal atrophy type I · spinal muscular atrophy type 1 of infancy

Early spinal muscular atrophy type I More severe and early form of neuromuscular atrophy that debuts before six months of age with severe generalized flaccid hypotonia, complete absence of head control and progressive respiratory difficulty due to the destruction of anterior motor neurons.

Also: Werdnig-Hoffmann disease of early infants · type I spinal atrophy of very early onset · early spinal muscular atrophy type 1

Early infantile spinal muscular atrophy type I More severe and earlier form of hereditary neuromuscular atrophy that debuts before six months of age with severe generalized flaccid hypotonia, complete absence of head control and progressive respiratory difficulty due to the destruction of the motor neurons of the spinal anterior horn.

Also: typical early infant Werdnig-Hoffmann disease · typical very early onset type I spinal atrophy · early infantile spinal muscular atrophy type 1

Spinal muscular atrophy type II Intermediate form of childhood neuromuscular atrophy that typically begins before eighteen months of age, in which affected children achieve the ability to sit independently independently but never achieve standing or spontaneous ambulation without support.

Also: chronic Werdnig-Hoffmann disease · infantile-onset spinal atrophy · spinal muscular atrophy type 2

Chronic spinal muscular atrophy type II A form of hereditary neuromuscular atrophy with childhood onset and prolonged course in which patients acquire the ability to sit independently but are unable to ambulate autonomously, requiring chronic mechanical assistance for mobility.

Also: chronic Werdnig-Hoffmann disease · progressive type II spinal atrophy · chronic spinal muscular atrophy type 2

Spinal muscular atrophy type III A form of hereditary neuromuscular atrophy with a more benign clinical course that begins after eighteen months of age, in which patients acquire the capacity for autonomous ambulation before developing progressive proximal muscle weakness and amyotrophy.

Also: Kugelberg-Welander disease · juvenile onset spinal atrophy · spinal muscular atrophy type 3

Childhood spinal muscular atrophy type III A form of hereditary neuromuscular atrophy with late onset during childhood, characterized by predominantly proximal weakness that is manifested mainly by progressive difficulty in getting up from the ground using the Gowers maneuver and waddling gait.

Also: classical Kugelberg-Welander disease · spinal atrophy type III · spinal muscular atrophy type 3 of childhood

Late-onset infantile spinal muscular atrophy type III A form of hereditary neuromuscular atrophy with late onset during childhood, characterized by predominantly proximal weakness that is manifested mainly by progressive difficulty in getting up from the ground using the Gowers maneuver.

Also: classical infantile Kugelberg-Welander disease · infantile spinal atrophy type III · spinal muscular atrophy type 3 of childhood of late onset

Progressive spinal muscular atrophy type III A form of hereditary neuromuscular atrophy with a juvenile onset and slow progressive course characterized by predominantly proximal weakness that gradually makes independent ambulation and autonomous climbing of stairs difficult.

Also: progressive Kugelberg-Welander disease · progressive spinal muscular atrophy type 3

Spinal muscular atrophy type IV Very rare, adult-onset form of hereditary progressive muscular atrophy with a slow and benign clinical course, characterized by debuting after thirty years of age with mild proximal muscle weakness of progressive progression and limb amyotrophy.

Also: adult spinal atrophy · adult motor neuron disease · spinal muscular atrophy type 4

Progressive muscular atrophy Subtype of motor neuron disease in which there is a selective and progressive degeneration of the lower motor neurons of the spinal anterior horn, respecting the pyramidal pathway. Clinically it manifests itself with predominantly distal weakness, asymmetric amyotrophy and marked fasciculations without signs of spasticity.

Also: AMP · selective lower motor neuron disease · progressive muscular atrophy

Progressive bulbar muscular atrophy Degenerative disease of the lower motor neuron that selectively compromises the motor nuclei of the brain stem, manifesting clinically with progressive flaccid dysarthria, severe dysphagia, atrophy with lingual fasciculations and weakness of the masticatory muscles.

Also: sporadic progressive bulbar palsy · pure bulbar motor neuron involvement · progressive bulbar spinal atrophy

Sporadic progressive bulbar muscular atrophy Degenerative lower motor neuron disease of a non-hereditary nature characterized by its debut in adulthood with exclusive involvement of the bulbar motor nuclei of the brain stem with progressive progression.

Also: sporadic progressive adult bulbar palsy · sporadic bulbar motor neuron involvement · sporadic progressive bulbar spinal atrophy

Progressive spinal muscular atrophy Progressive form of non-hereditary spinal lower motor neuron degeneration that characteristically affects adults, manifested semiologically by asymmetric muscle weakness, persistent flaccidity, massive atrophy and fasciculations with preservation of sensitivity.

Also: selective spinal lower motor neuron disease · progressive spinal amyotrophy · progressive spinal muscular atrophy

Adult progressive spinal muscular atrophy Sporadic degenerative disease of the lower motor neuron that debuts in adulthood and is located in the spinal cord, manifesting semiologically by asymmetric and progressive flaccid muscle weakness accompanied by marked muscular atrophy of a chronic course.

Also: sporadic adult spinal amyotrophy · sporadic lower motor neuron disease · adult progressive spinal muscular atrophy

Late-onset adult progressive spinal muscular atrophy Sporadic degenerative disease of the lower motor neuron that debuts in adulthood from the fifth decade of life and is located in the spinal cord, manifesting semiologically by asymmetric and progressive flaccid muscle weakness accompanied by chronic muscular amyotrophy.

Also: late-onset adult sporadic spinal amyotrophy · late sporadic lower motor neuron disease · late-onset adult progressive spinal muscular atrophy

Blepharospasm Focal dystonia characterized by spasmodic, involuntary, repetitive and bilateral contractions of the orbicularis oculi muscles. It can cause prolonged eye closure with secondary functional blindness despite the anatomical integrity of the eyeball.

Also: palpebral orbicularis dystonia · involuntary eyelid spasm · blepharospasm

CADASIL Autosomal dominant cerebral arteriopathy with subcortical infarcts and leukoencephalopathy caused by genetic mutations in the NOTCH3 gene. Clinically, it manifests itself with migraine with aura, recurrent ischemic strokes in middle age, and progressive vascular dementia.

Also: NOTCH3 cerebral arteriopathy · hereditary vascular dementia · dominant hereditary leukoencephalopathy

Chronic paroxysmal Horton's headache Trigemino-autonomic headache variant characterized by daily attacks of unbearable pain of a strictly unilateral nature that occur continuously for more than a year without significant periods of spontaneous remission.

Also: Chronic refractory Horton · chronic paroxysmal Horton's headache

Refractory chronic Horton's headache Variant of trigemino-autonomic headache characterized by daily attacks of unbearable pain of a strictly unilateral nature that occur continuously without response to the usual prophylactic pharmacological treatments.

Also: refractory chronic cluster headache · Chronic Horton that is difficult to control · chronic refractory Horton's headache

Severe refractory chronic Horton's headache Variant of trigemino-autonomic headache characterized by daily attacks of unbearable pain of a strictly unilateral nature that occur continuously without response to prophylactic pharmacological treatments or invasive blocking therapies.

Also: refractory severe chronic cluster headache · Chronic refractory difficult to control Horton · severe chronic refractory Horton's headache

Refractory episodic Horton's headache Clinical variant of trigemino-autonomic headache characterized by periods of daily attacks of unbearable pain of a unilateral nature that occur in cyclical periods that are difficult to control and without an adequate response to standard prophylaxis.

Also: refractory episodic cluster headache · Intractable episodic Horton · refractory episodic Horton's headache

Refractory Horton's headache Extremely painful primary headache, strictly unilateral in location and lancinating in nature, associated with severe ipsilateral autonomic symptoms of a daily nature that do not subside with the persistent administration of oxygen or triptans.

Also: cluster headache refractory to treatment · intractable Horton's headache · refractory cluster headache

Cluster headache Extremely painful primary headache, strictly unilateral in location and lancinating in nature, which is associated with ipsilateral autonomic symptoms such as conjunctival injection, lacrimation and nasal congestion. Attacks usually occur in grouped periods called clusters that last weeks or months.

Also: Horton's headache · migrainous neuralgia · cluster headache

Chronic cluster headache Clinical form of trigemino-autonomic headache in which daily attacks of unbearable unilateral pain occur continuously for more than a year without periods of remission or with remissions lasting less than three months.

Also: chronic Horton's headache · chronic cluster · chronic cluster headache

Episodic cluster headache Clinical variant of trigemino-autonomic headache characterized by periods of daily attacks of unbearable pain lasting weeks or months, characteristically alternating with periods of complete pain remission free of attacks lasting at least three months.

Also: episodic Horton's headache · episodic cluster · episodic cluster headache

Tension headache Very common primary headache of an oppressive nature, bilateral and of mild to moderate intensity, described as a band of pressure around the skull, which does not worsen with regular physical activity and is not associated with severe nausea or photophobia. It is usually linked to tension of the craniocervical muscles and emotional stress.

Also: tension headache · muscular headache · tension-type headache

Traumatic brain concussion Mild traumatic brain injury induced by transient biomechanical forces that functionally and non-structurally alter brain neurotransmission, clinically characterized by transient confusion, short-lived altered alertness, headache and self-limited post-traumatic amnesia.

Also: concussion · mild head trauma with functional impairment · concussion

Typical absence crisis Generalized epileptic manifestation characterized by a sudden, brief and involuntary episode of loss of the level of contact with the environment, of abrupt onset and end, usually accompanied by an electroencephalographic pattern of slow spike-wave discharges at three hertz frequency bilaterally and synchronously. It is not associated with loss of postural tone or relevant post-critical confusion.

Also: little epileptic evil · child absence crisis · typical absence seizure

Generalized tonic-clonic seizure Major motor epileptic event characterized by an initial phase of sustained muscle contraction of the entire body followed by rhythmic and bilateral jerks of the extremities, accompanied by complete loss of consciousness, cyanosis and urine emission. It usually ends with a prolonged postictal period of drowsiness, confusion, and generalized limpness.

Also: great evil · generalized convulsive epileptic seizure · generalized tonic-clonic seizure

Diffuse axonal damage Severe traumatic brain injury produced by high-speed shearing and stretching forces on axons due to angular acceleration and deceleration mechanisms of the skull. It causes immediate loss of consciousness with prolonged coma and is visualized microscopically as widespread axonal retractions.

Also: post-traumatic axonal shearing · diffuse axonal injury · diffuse axonal injury

Dementia with Lewy bodies Progressive degenerative encephalopathy characterized by the intraneuronal accumulation of alpha-synuclein aggregates in the cerebral cortex and subcortical structures. It manifests with fluctuating cognitive impairment, recurrent detailed visual hallucinations, severe dysautonomia, and early-onset parkinsonism.

Also: Lewy dementia · synucleinopathy with Lewy bodies · dementia with Lewy bodies

Dementia with diffuse Lewy bodies Degenerative synucleinopathy in which the widespread deposition of intraneuronal protein aggregates causes severe and fluctuating cognitive impairment associated early with symmetric parkinsonism and complex hallucinations with loss of awareness of the environment.

Also: cortical Lewy body dementia · diffuse Lewy body disease · diffuse Lewy body disease

Fluctuating Lewy body dementia Degenerative synucleinopathy clinically characterized by progressive cognitive impairment associated with marked and spontaneous variations in alertness, attention and executive functions from day to day or from hour to hour in a characteristic manner.

Also: fluctuating Lewy dementia · Lewy fluctuating cognitive impairment · fluctuating dementia with Lewy bodies

Frontotemporal dementia Clinically heterogeneous group of posterior non-cortical neurodegenerative disorders characterized by selective atrophy of the frontal and temporal lobes of the brain. It manifests predominantly with early and profound changes in personality, social disinhibition, and progressive language alterations before compromising memory.

Also: Pick's disease and variants · frontotemporal lobar degeneration · frontotemporal dementia

Cortical vascular dementia Progressive cognitive impairment secondary to the accumulation of multiple focal ischemic lesions of cortical location caused by thromboembolic occlusion of main arterial branches of the brain, characterized semiologically by a progression in steps.

Also: dementia due to multiple infarcts · cortical vascular dementia · multi-infarct dementia

Small vessel vascular dementia Progressive cognitive impairment of ischemic origin caused by a generalized deep ischemic leukoencephalopathy secondary to a microangiopathy of the small penetrating cerebral arteries associated with long-standing chronic arterial hypertension.

Also: microangiopathic subcortical vascular dementia · advanced Binswanger's disease · small vessel vascular dementia

Dysesthesia Sensory alteration of a neuropathic type in which a normal tactile stimulus produces a painful, unpleasant, burning or aberrant sensation in a specific body area, secondary to dysfunction of the peripheral or central somatosensory system.

Also: aberrant painful tactile sensation · qualitative alteration of sensitivity · dysesthesia

Dysesthesia with tactile allodynia Alteration of neuropathic sensitivity in which the patient experiences sensations of extreme burning or burning pain triggered involuntarily by minimal innocuous mechanical stimuli such as the rubbing of clothing or sheets against the skin.

Also: tactile hyperalgesia due to friction · dysesthetic allodynia · tactile allodynia with dysesthesia

Painful dysesthesia predominantly distal Type of chronic neuropathic pain characterized by an annoying and continuous sensation of burning or burning located symmetrically in the feet and hands, very characteristic of distal axonal involvement of fine fibers.

Also: symmetrical distal burning pain · painful distal axonal involvement · distal painful dysesthesia

Symmetrical distal painful dysesthesia Type of chronic neuropathic pain characterized by an annoying and continuous sensation of burning or burning located symmetrically in the feet and hands, very characteristic of distal axonal involvement of fine fibers.

Also: distal symmetric painful dysesthesia

Persistent symmetrical distal painful dysesthesia Type of chronic neuropathic pain characterized by an annoying and continuous sensation of burning or burning located symmetrically in the feet and hands that does not respond to usual neuromodulatory drugs.

Also: persistent symmetrical distal burning pain · refractory distal axonal painful involvement · persistent distal symmetric painful dysesthesia

Post-infectious painful dysesthesia Permanent alteration of somatic sensitivity characterized by burning-type painful sensations or spontaneous discharge located in specific dermatomes, secondary to direct damage to the sensory ganglia of the dorsal root after a viral infection.

Also: post-infectious neuropathic pain · post-herpetic dysesthesia · postinfectious painful dysesthesia

Persistent postinfectious painful dysesthesia Neuropathic-type sensitivity alteration characterized by persistent intense burning pain triggered by minimal tactile contact in specific dermatomes previously compromised by an acute viral infection.

Also: persistent postherpetic neuralgia · permanent post-infectious burning pain · persistent postinfectious painful dysesthesia

Distal burning dysesthesia Type of neuropathic pain characterized by a continuous sensation of heat or fire in the distal portion of the extremities, typically symmetrical and with a sock or glove distribution pattern, very characteristic of unmyelinated fine fiber polyneuropathies.

Also: neuropathic distal burning · distal small fiber pain · burning feet syndrome

Extrapyramidal tardive dyskinesia Movement disorder characterized by the appearance of involuntary choreic, athetotic or dystonic movements, predominantly orofacial and persistent, caused by prolonged and chronic exposure to drugs that block dopamine D2 receptors.

Also: neuroleptic-induced dyskinesia · delayed pharmacological chorea · tardive dyskinesia

Steinert myotonic dystrophy Inherited autosomal dominant neuromuscular disorder caused by abnormal expansion of CTG repeats in the DMPK gene. It is clinically characterized by progressive distal weakness, delay in voluntary muscle relaxation after contraction, early cataracts, frontal baldness and cardiac conduction disorders.

Also: myotonic dystrophy type 1 · Steinert disease · myotonic dystrophy type 1

Duchenne muscular dystrophy X-linked hereditary myopathy due to the absence or complete dysfunction of dystrophin, a structural protein of the sarcolemmal membrane of skeletal muscle. It causes progressive and symmetrical muscle weakness, calf pseudohypertrophy, and loss of ambulation at an early age.

Also: pseudohypertrophic muscular dystrophy · Duchenne myopathy · Duchenne muscular dystrophy

Herpetic encephalitis Focal, necrotizing viral infection of the central nervous system caused predominantly by herpes simplex virus type 1, which selectively affects the temporal lobes and orbital cortex. Clinically it presents with high fever of acute onset, focalized partial epileptic seizures, behavioral alterations and motor aphasia.

Also: herpes simplex virus encephalitis · temporary herpetic necrosis · herpes simplex encephalitis

Encephalitis due to antibodies against the NMDA receptor Severe neuropsychiatric autoimmune disorder characterized by the presence of antibodies directed against the GluN1 subunit of the NMDA receptor in the nervous system. It manifests clinically with acute psychosis, movement disorders with orofacial dyskinesias, epileptic seizures and autonomic instability.

Also: anti-NMDA receptor encephalitis · autoimmune limbic encephalitis · anti-NMDA receptor encephalitis

Encephalitis due to antibodies against LGI1 Specific form of autoimmune limbic encephalitis associated with antibodies directed against the LGI1 protein, clinically characterized by brief and recurrent faciobrachial dystonic crises, progressive loss of short-term memory and persistent hyponatremia.

Also: anti-LGI1 encephalitis · anti-LGI1 limbic condition · anti-LGI1 encephalitis

Refractory anti-LGI1 antibody encephalitis Severe form of autoimmune encephalitis associated with antibodies against the LGI1 protein that presents with frequent faciobrachial dystonic crises and persistent dementia that does not respond adequately to first-line immunosuppressive therapy.

Also: anti-LGI1 encephalitis that is difficult to control · refractory anti-LGI1 limbic involvement · refractory anti-LGI1 encephalitis

Anoxic-ischemic encephalopathy Severe diffuse neurological damage caused by prolonged oxygen deprivation combined with insufficient cerebral perfusion, usually after cardiorespiratory arrest or perinatal asphyxia. It can cause lamellar cortical necrosis and structural damage to the basal ganglia with permanent neurological sequelae or refractory coma.

Also: diffuse anoxic brain damage · hypoxic-ischemic encephalopathy · hypoxic-ischemic encephalopathy

Acute hypertensive encephalopathy Reversible and acute neurological syndrome caused by a sudden and sustained rise in blood pressure that exceeds the upper limits of autoregulation of cerebral blood flow, causing diffuse cerebral edema, severe pulsating headache, seizures and transient cortical blindness.

Also: encephalopathy due to hypertensive crisis · self-regulating cerebral edema · hypertensive encephalopathy

Progressive uremic encephalopathy Diffuse brain dysfunction of a metabolic nature caused by the systemic accumulation of nitrogenous toxins and organic waste products in the context of advanced renal failure. It presents with lethargy, flapping tremor, asterixis of the extremities, multifocal myoclonus, seizures and coma in terminal stages.

Also: metabolic uremic delirium · encephalopathy due to kidney failure · uremic encephalopathy

Alzheimer's disease Progressive neurodegenerative process and the most common cause of dementia worldwide, pathologically characterized by the extracellular deposition of beta-amyloid plaques and intracellular neurofibrillary tangles of hyperphosphorylated tau protein. Clinically, it begins with short-term episodic memory loss of insidious evolution.

Also: senile dementia of the Alzheimer type · neurodegenerative Alzheimer's disease · Alzheimer disease

Early onset familial Alzheimer's disease A form of dementia with autosomal dominant inheritance caused by rare mutations in the amyloid precursor protein genes, presenilin 1 or presenilin 2, characterized by debuting before the age of sixty-five with an accelerated clinical course.

Also: early hereditary amyloid dementia · Early familial Alzheimer's · early-onset familial Alzheimer's disease

Rapidly progressive familial Alzheimer's disease Autosomal dominant inherited form of dementia in which mutations in the PSEN1 or PSEN2 gene cause accelerated and progressive cognitive and behavioral deterioration that evolves into mutism and prostration in a few years of evolution.

Also: rapid course familial amyloid dementia · Accelerated familial Alzheimer's · rapidly progressive familial Alzheimer's disease

Binswanger's disease A form of subcortical vascular dementia characterized by the presence of extensive areas of demyelination and diffuse ischemic leukoencephalopathy in the deep white matter of the brain. It is caused by severe atherosclerosis of the small penetrating cerebral arteries associated with long-standing chronic arterial hypertension.

Also: progressive arteriosclerotic subcortical encephalopathy · subcortical vascular dementia · Binswanger's disease

Binswanger's disease of white matter Variant of vascular dementia in which chronic and progressive ischemia of the deep cerebral white matter produces severe impairment of executive functions combined with gait rigidity and bladder involvement of central origin.

Also: Binswanger arteriosclerotic leukoencephalopathy · deep microangiopathic dementia · Binswanger subcortical dementia

Creutzfeldt-Jakob disease Transmissible spongiform encephalopathy with a rapidly progressive and fatal course, caused by the pathological folding and accumulation of the prion protein scrapie in the brain tissue. It manifests clinically with rapidly progressive dementia, myoclonus, ataxia and akinetic mutism.

Also: human cerebral prionycopathy · CJD · CJD

Sporadic Creutzfeldt-Jakob disease Most common variant of prion spongiform encephalopathy that appears spontaneously without a known family history or identifiable risk factors. It presents with dementia of fulminant progression, generalized multifocal myoclonus, progressive ataxia and akinetic mutism in a few months.

Also: sCJD · sporadic cortical prionycopathy · sporadic Creutzfeldt-Jakob disease

Familial Creutzfeldt-Jakob disease Autosomal dominant hereditary spongiform encephalopathy caused by specific inherited mutations in the PRNP gene for the prion protein, characterized by rapidly progressive dementia associated with myoclonus and fatal ataxia.

Also: familial prionycopathy · hereditary prion dementia · familial Creutzfeldt-Jakob disease

Variant Creutzfeldt-Jakob disease Acquired form of prion spongiform encephalopathy transmitted by the consumption of beef contaminated with bovine spongiform encephalopathy prions, characterized clinically by debuting with early prominent psychiatric symptoms followed by ataxia, myoclonus and rapidly progressive dementia.

Also: vCJD · mad cow disease in humans · variant Creutzfeldt-Jakob disease

Acquired variant Creutzfeldt-Jakob disease Acquired form of prion spongiform encephalopathy transmitted by the consumption of beef contaminated with bovine spongiform encephalopathy prions, clinically characterized by onset with prominent psychiatric symptoms followed by progressive ataxia.

Also: acquired classic vCJD · mad cow disease in humans variant · variant acquired Creutzfeldt-Jakob disease

Iatrogenic Creutzfeldt-Jakob disease Spongiform encephalopathy due to prions accidentally acquired secondary to medical procedures such as dura mater or cornea transplants from contaminated donors, or due to the use of growth hormone extracted from cadaveric pituitary glands.

Also: Iatrogenic CJD · iatrogenic acquired prionycopathy · iatrogenic Creutzfeldt-Jakob disease

Huntington's disease Autosomal dominant hereditary neurodegenerative disorder with progressive progression characterized by involuntary choreic movements, severe psychiatric disorders and cognitive impairment with a dementing course. It is caused by the pathological expansion of CAG nucleotide repeats in the huntingtin gene.

Also: Huntington's chorea · hereditary choreic dementia · Huntington disease

Pick's disease Specific form of frontotemporal dementia characterized histopathologically by the presence of pathologically spherical intraneuronal inclusions of tau protein called Pick bodies. Clinically, it presents with progressive aphasia, marked apathy or severe behavioral disinhibition due to cortical atrophy located in the anterior lobes.

Also: anterior lobar degeneration with Pick bodies · Pick's dementia · Pick's disease

Amyotrophic lateral sclerosis Progressive and fatal neurodegenerative disease that selectively affects the upper and lower motor neurons of the cortex, brain stem and spinal cord. It causes progressive muscle weakness, amyotrophy, fasciculations, and irreversible bulbar dysfunction.

Also: ALS · motor neuron disease · ALS

Sporadic amyotrophic lateral sclerosis The most common and non-hereditary form of motor neuron disease characterized by its debut in adulthood with progressive and asymmetric muscle weakness of the extremities of pyramidal and amyotrophic origin combined with scattered fasciculations.

Also: Classic sporadic ALS · classic sporadic motor neuron disease · sporadic amyotrophic lateral sclerosis

Familial amyotrophic lateral sclerosis Inherited and autosomal dominant transmission form of motor neuron disease, frequently caused by specific mutations in the SOD1, C9orf72 or TARDBP genes, which causes rapid and progressive degeneration of the motor pathways.

Also: Hereditary ALS · familial motor neuron disease · familial amyotrophic lateral sclerosis

Primary lateral sclerosis Uncommon variant of motor neuron disease that selectively affects the upper motor neurons of the cerebral cortex, characteristically sparing the motor neurons of the spinal anterior horn. Clinically, it presents with severe bilateral and progressive spasticity of the lower extremities, persistent hyperreflexia and spastic-type gait alteration.

Also: ELP · pure pyramidal degeneration · primary lateral sclerosis

Hemifacial spasm Movement disorder characterized by involuntary, repetitive, painless tonic or clonic contractions of the mimic muscles innervated by the facial nerve in one half of the face. Its etiology frequently lies in microvascular compression of the seventh cranial nerve as it exits the brain stem.

Also: unilateral facial spasm · peripheral facial hyperkinesis · hemifacial spasm

Idiopathic hemifacial spasm Involuntary and intermittent clonic muscle contractions confined to one side of the face innervated by the facial nerve, appearing spontaneously without evidence of underlying structural lesion identifiable in classic neuroimaging studies.

Also: primary hemifacial hyperkinesis · idiopathic facial spasm · idiopathic hemifacial spasm

State of minimal consciousness Severe disorder of consciousness characterized by the presence of minimal but clearly demonstrable evidence of awareness of oneself or the environment in a fluctuating but reproducible manner, differentiated from the vegetative state by the ability to perform behaviors with a voluntary purpose.

Also: EMC · minimal brain reactivity · minimally conscious state

Persistent vegetative state A state of wakefulness without response or awareness of self or environment in which the autonomic functions of the brainstem and hypothalamus are maintained, but with complete absence of measurable cognitive cortical function. It is defined as persistent if it persists for more than a month after an acute brain injury.

Also: state of non-responsive wakefulness · coma wakefulness · persistent vegetative state

Status epileptic Critical neurological emergency characterized by continuous epileptic activity or recurrent seizures without complete recovery of the level of interictal alertness for a period that exceeds the physiological time of spontaneous resolution. It represents a severe risk of irreversible brain damage due to persistent neuronal excitotoxicity.

Also: state of epileptic malady · continuous prolonged crisis · status epilepticus

Acute epidural hematoma Rapid accumulation of arterial blood between the inner lamina of the cranial bone and the dura mater, usually caused by rupture of the middle meningeal artery after a high-energy head injury with associated bone fracture. Clinically, it manifests itself with a lucid interval followed by sudden neurological deterioration with signs of brain herniation.

Also: traumatic epidural bleeding · extradural hematoma · acute epidural hematoma

Traumatic acute subdural hematoma Rapid accumulation of fresh venous blood located in the subdural space secondary to severe head trauma with bridging vein tear or underlying cortical cerebral contusion, characterized semiologically by early profound neurological deterioration and massive intracranial hypertension.

Also: acute traumatic subdural bleeding · high energy acute subdural hematoma · acute traumatic subdural hematoma

Severe traumatic acute subdural hematoma Massive accumulation of fresh venous blood located in the subdural space secondary to a high-energy head injury with great deviation of the cerebral midline, requiring emergency surgical evacuation through decompressive craniotomy.

Also: acute severe traumatic subdural bleeding · hyperacute compressive subdural hematoma · severe acute traumatic subdural hematoma

Chronic subdural hematoma Slow accumulation of venous and liquefied blood between the dura mater and the arachnoid mater, usually caused by the rupture of cortical bridging veins after minor trauma in atrophic brains of elderly patients. It causes progressive headache, mental status fluctuation, psychomotor slowness and diffuse focal neurological signs.

Also: subdural hematoma in the elderly · chronic subdural collection · chronic subdural hematoma

Bilateral chronic subdural hematoma Presence of organized venous collections of liquefied blood located in the subdural space of both cerebral hemispheres in elderly patients, who present clinically with severe fluctuating confusion, deep bradypsychia and dull headache.

Also: bilateral subdural collections from the elderly · chronic bilateral subdural hematoma · bilateral chronic subdural hematoma

Subacute subdural hematoma Accumulation of venous blood in the cerebral subdural space whose symptoms of compression and progressive mental alteration typically manifest between three days and three weeks after a head trauma.

Also: intermediate subdural bleeding · subacute subdural collection · subacute subdural hematoma

Post-traumatic subacute subdural hematoma Venous collection of blood located in the cerebral subdural space whose symptoms of intracranial compression and progressive headache manifest clinically between three days and three weeks after a head trauma.

Also: post-traumatic intermediate subdural bleeding · subdural hematoma of subacute evolution · subacute subdural hematoma after trauma

Intraparenchymal hemorrhage Bleeding of non-traumatic origin located directly in the thickness of the brain tissue, frequently caused by the rupture of small arteries injured by chronic arterial hypertension or amyloid angiopathy. It causes an acute focal neurological deficit that progresses rapidly and associates severe headache with signs of intracranial hypertension.

Also: non-traumatic intracerebral hematoma · focal cerebral hemorrhage · intraparenchymal hemorrhage

Hypertensive intraparenchymal hemorrhage Spontaneous and focal bleeding in the brain parenchyma located preferably in deep structures such as the putamen, thalamus or pons, secondary to the rupture of Charcot-Bouchard microaneurysms injured by long-standing chronic hypertension.

Also: deep hypertensive brain hematoma · deep cerebral bleeding · hypertensive intraparenchymal hemorrhage

Subarachnoid hemorrhage Pathological bleeding within the subarachnoid space delimited by the arachnoid meninges and pia mater, usually secondary to the spontaneous rupture of a saccular arterial aneurysm in the circle of Willis. It is manifested semiologically by a sudden and unbearable headache described as the worst in life, accompanied by neck stiffness and vomiting.

Also: HSA · aneurysmal meningeal bleeding · subarachnoid hemorrhage

Cerebellar tonsillar herniation Pathological descending displacement of the cerebellar tonsils through the foramen magnum due to the severe increase in intracranial pressure in the posterior fossa, causing lethal compression of the medulla oblongata with sudden cardiorespiratory arrest.

Also: cerebellar tonsil herniation · foraminal herniation · tonsillar herniation

Uncal herniation Pathological displacement of the medial portion of the temporal lobe through the tentorium notch due to increased pressure in the supratentorial compartment. It causes ipsilateral compression of the third cranial nerve with pupillary mydriasis, involvement of the pyramidal tract with contralateral hemiparesis and progressive deterioration of the level of consciousness.

Also: hernia of the temporal uncus · lateral tentorial herniation · uncal herniation

Normal pressure hydrocephalus Disorder characterized by the classic Hakim-Adams triad that includes progressive dementia, broad-based gait disturbance with magnetic apraxia, and urinary urgency or incontinence of central origin, accompanied by ventricular dilation without measurable elevation of cerebrospinal fluid pressure.

Also: normotensive hydrocephalus · Hakim-Adams syndrome · normal pressure hydrocephalus

Post-traumatic subdural hygroma Accumulation of clear cerebrospinal fluid in the subdural space between the dura mater and the arachnoid mater, generated secondary to a post-traumatic tear of the external arachnoid membrane that acts as a one-way valve after a head injury.

Also: subdural CSF collection · acute subdural hygroma · subdural hygroma

Spinal autonomic hyperreflexia Clinical syndrome of massive and uncontrolled sympathetic response triggered by infralesional noxious stimuli in patients who present complete chronic spinal cord lesions above T6. It is manifested semiologically by severe paroxysmal arterial hypertension, intense pulsating headache, profuse sweating and flushing above the lesion level.

Also: autonomic dysreflexia · spinal sympathetic hyperreactivity · autonomic dysreflexia

Ostetendinous hyperreflexia Pathological elevation of the reflex responses obtained by percussion of the muscle tendon in the neurological examination, manifested by a decreased threshold, increase in the reflexogenic area and polykinetic responses, indicative of involvement of the pyramidal pathway.

Also: exalted reflexes · deep hyperreflexia · hyperreflexia

Deep patellar hyperreflexia Pathological elevation of the patellar or patellar reflex obtained by percussion of the quadriceps tendon in the neurological examination, manifested by a polykinetic response with transient clonus of the patella of a bilateral nature.

Also: exalted patellar reflex · bilateral patellar hyperreflexia · deep patellar hyperreflexia

Familial diffuse leukoencephalopathy Very rare hereditary neurodegenerative disorder in adults characterized by massive and symmetrical loss of myelin in the deep white matter of the brain, leading to progressive cognitive impairment, severe spasticity and deep extrapyramidal dysfunction.

Also: adult hereditary leukoencephalopathy · hereditary diffuse demyelination · hereditary diffuse leukoencephalopathy

Progressive multifocal leukoencephalopathy Opportunistic and progressive demyelinating infection of the central nervous system caused by lytic reactivation of the JC virus in the oligodendrocytes of patients with severe immunosuppression. It manifests clinically with progressive multifocal neurological deficits, cognitive-behavioral alterations and cortical blindness.

Also: LMP · demyelination due to JC virus · progressive multifocal leukoencephalopathy

Diffuse vascular leukoencephalopathy Presence of widespread and chronic ischemic damage to the deep cerebral white matter characterized by diffuse diffuse demyelination detectable in imaging studies. It constitutes the pathological substrate of vascular dementia of microangiopathic origin.

Also: microvascular involvement of white matter · leukoaraiosis · diffuse white matter disease

Broad-based sensory ataxic gait Pathological ambulation pattern characterized by unsafe steps with exaggerated elevation of the knees and feet very far from the ground, forcing the patient to use continuous visual control of the ground due to severe proprioceptive loss of posterior cords.

Also: unstable proprioceptive ambulation · proprioceptive ataxic gait · wide-based sensory ataxic gait

Wide base gait Alteration of the gait pattern characterized by excessive separation of the feet during ambulation to expand the body's center of gravity and compensate for instability. It is a typical semiological sign of dysfunction of the cerebellar pathways or severe loss of conscious proprioceptive sensitivity.

Also: extended lift gear · broad-based wandering · wide-based gait

Ataxic wide-based gait Walking pattern characterized by severe gait instability with feet widely spaced and erratic and uneven steps of a hesitant nature, typical of cerebellar degeneration or severe proprioceptive loss.

Also: broad-based ataxic gait · hesitant wandering with broad base · wide-based ataxic gait

Progressive ataxic wide-based gait Pattern of wandering characterized by severe gait instability with feet widely spaced and erratic steps of a slowly progressive nature, typical of hereditary or acquired cerebellar degeneration with a continuous course.

Also: progressive broad-based ataxic gait · progressive unsteady wandering · progressive wide-based ataxic gait

Hereditary progressive ataxic broad-based gait Pattern of ambulation characterized by severe instability of gait with widely spaced feet and erratic steps of a slowly progressive nature of hereditary genetic origin, typical of familial spinocerebellar ataxias of continuous evolution.

Also: progressive familial broad-based ataxic gait · progressive hereditary hesitating wandering · progressive hereditary wide-based ataxic gait

Cerebellar broad-based gait Walking pattern characterized by severe multidirectional instability in which the patient walks with his legs wide apart in an erratic manner, taking uneven steps with marked lateral oscillations of the trunk, due to atrophy or injury of the cerebellar vermis.

Also: cerebellar drunken march · broad-based ataxic · cerebellar wide-based gait

Proprioceptive wide-based gait Pathological ambulation pattern in which the patient walks with legs wide apart and instability due to the loss of information about the position of the joints in space, compensated by continuous visual control of the ground.

Also: sensory ataxic gait · broad-based proprioceptive ambulation · sensory wide-based gait

Ataxic proprioceptive broad-based gait Pattern of pathological ambulation in which the patient walks with his legs erratically wide apart due to the complete loss of information about the position of the joints in space, compensated only by continuous visual control of the ground.

Also: broad-based unstable proprioceptive ambulation · sensory wide-based ataxic gait

Sensory wide-based gait Walking pattern characterized by widely spaced feet and instability due to the loss of information about the position of the joints in space, compensated by continuous visual control of the ground.

Duck March Pattern of ambulation characterized by an exaggerated and lateral oscillation of the pelvis with each step due to weakness of the gluteus medius muscles and the pelvic girdle. It is typically observed in predominantly proximal myopathies or advanced muscular dystrophies.

Also: duck march · myopathic gait · waddling gait

Myopathic duck gait Walking pattern characterized by an exaggerated oscillation of the pelvis due to bilateral weakness of the hip fixation muscles, very characteristic of hereditary or acquired myopathies of proximal distribution and long duration.

Also: myopathic duck wandering · bilateral Trendelenburg gait · myopathic waddling gait

March in the steppe Abnormal ambulation pattern characterized by exaggerated elevation of the knee and thigh when walking to avoid dragging the hanging foot due to weakness of the dorsiflexor muscles. It characteristically occurs in patients with lesions of the common peroneal nerve or with predominantly motor polyneuropathies.

Also: equine gait · dropped foot march · high steppage gait

March in bilateral steppe Pathological ambulation pattern in which both feet of the patient adopt a position of falling or pendulous flaccidity due to severe motor polyneuropathy or affection of the bilateral external popliteal sciatic nerve, forcing him to raise both knees high when walking.

Also: bilateral equine ambulation · bilateral pendulum foot gait · bilateral steppage gait

Unilateral equine gait Pathological wandering in which the affected foot adopts an involuntary pendulous position due to weakness of the dorsiflexor muscles due to injury to the external popliteal sciatic nerve, forcing the patient to raise the knee exaggeratedly to avoid dragging the toe of the foot.

Also: unilateral steppe march · unilateral foot drop ambulation · unilateral equine gait

Bilateral spastic gait of spinal origin Difficult and slow ambulation characterized by spastic rigidity of both lower extremities secondary to chronic ischemic or compressive lesions in the pyramidal pathway of the spinal cord of chronic course.

Also: spinal scissors march · bilateral spinal pyramidal wandering · bilateral spinal spastic gait

Progressive bilateral spastic gait of spinal origin Difficult and slow ambulation characterized by spastic rigidity of both lower extremities secondary to chronic ischemic or compressive lesions in the pyramidal pathway of the spinal cord with a chronic and progressive course.

Also: progressive spinal scissor gait · progressive bilateral spinal pyramidal wandering · progressive bilateral spinal spastic gait

Rigid bilateral spastic gait Difficult and slow ambulation characterized by spastic rigidity of both lower extremities that collide with each other due to adductor hypertonia of the pyramidal pathway of central origin, very characteristic of primary lateral sclerosis and chronic spinal myelopathies.

Also: bilateral scissors gait · bilateral pyramidal wandering

Bilateral spinal spastic gait Difficult and slow ambulation characterized by spastic rigidity of both lower extremities that collide with each other during walking due to adductor hypertonia of the bilateral pyramidal tract, very characteristic of primary lateral sclerosis and chronic myelopathies.

Also: bilateral scissor gait · rigid bilateral pyramidal wandering · bilateral spastic scissors gait

Senile spastic gait Walking disorder in elderly patients characterized by slow, rigid steps and bilateral dragging of the feet due to pyramidal hypertonia, secondary to ischemic lesions of the central motor pathway or spinal degenerative changes.

Also: stiff ambulation of the old man · bilateral pyramidal gait · senile spastic gait

Spastic reaper hemiplegic gait Walking pattern characterized by muscle rigidity in extension and plantar flexion of the affected leg secondary to an ipsilateral pyramidal injury, forcing the patient to describe a lateral arc of circumduction with the hip to take the step.

Also: circumduction pyramid wandering · pyramidal mower gear · spastic hemiplegic gait

Unilateral reaping spastic hemiplegic gait Walking pattern characterized by rigidity in extension of the affected lower extremity that forces the patient to describe a lateral semicircular movement to take a step, secondary to a unilateral pyramidal lesion of contralateral vascular or tumor origin.

Also: contralateral hemiplegic reaper ambulation · spastic gait of unilateral lateral circumduction · unilateral spastic hemiplegic steppage gait · unilateral circumduction pyramidal wandering · pyramidal mowing gear of hemispheric origin

Hemiplegic reaper gait Ambulation pattern of pyramidal origin secondary to a contralateral hemispheric cerebral vascular accident, characterized by muscle rigidity in extension of the affected leg that forces the patient to perform an exaggerated lateral arc of circumduction to avoid stumbling.

Also: contralateral reaper ambulation · pyramid scythe march · hemiplegic steppage gait

Hemiplegic mowing gait of central origin Walking pattern characterized by muscle rigidity in extension and plantar flexion of the affected leg secondary to ipsilateral cortical or subcortical pyramidal injury.

Also: central circumduction pyramidal wandering · cortical pyramidal mowing gear · central spastic hemiplegic gait

Parkinsonian gait Walking disorder characterized by short, rapid and shuffling steps, with loss of bilateral reciprocal arm movement and with the center of gravity of the body tilted anterogradely. The patient usually has difficulty initiating movement and very slow block turns.

Also: festinating wandering · march of small steps · parkinsonian gait

Unstable parkinsonian gait Small-step ambulation pattern characterized by loss of bilateral postural reflexes, involuntary retropulsion and marked gait instability with a tendency to falls, typical of advanced stages of Parkinson's disease.

Also: advanced parkinsonian wandering · extrapyramidal unstable gait · unstable parkinsonian gait

Rigid parkinsonian gait Pattern of ambulation of small steps characterized by marked extrapyramidal rigidity in trunk flexion, bilateral absence of arm movements and marked postural instability of a progressive nature, typical of intermediate stages of Parkinson's disease.

Also: rigid-akinetic ambulation · typical parkinsonian gait · rigid parkinsonian gait

Rigid-akinetic parkinsonian gait Small-step ambulation pattern characterized by marked extrapyramidal rigidity in trunk flexion, bilateral absence of arm movements, and marked progressive postural instability typical of intermediate stages of Parkinson's disease.

Also: typical rigid-akinetic ambulation · akinetic parkinsonian gait · rigid-akinetic parkinsonian gait

Progressive rigid-akinetic parkinsonian gait Pattern of ambulation of small steps characterized by marked extrapyramidal rigidity in trunk flexion, bilateral absence of arm movements and marked postural instability of a slowly progressive nature that associates difficulties in starting to walk.

Also: progressive rigid-akinetic ambulation · progressive akinetic parkinsonian gait · progressive rigid-akinetic parkinsonian gait

Mower gear Type of pathological ambulation in which the patient performs a lateral circumduction movement with the affected lower extremity, keeping it rigid and in extension to prevent the foot from dragging on the ground. It characteristically occurs in patients with spastic hemiparesis of pyramidal origin.

Also: unilateral spastic gait · circumduction wandering · hemiplegic gait

Unilateral spastic mowing gait Pathological pyramidal wandering secondary to a contralateral hemispheric stroke, in which severe spasticity of the lower extremity forces the patient to describe a lateral arc with the extremity extended in order to take the step.

Also: spastic hemiplegic wandering · unilateral scythe march · unilateral spastic hemiplegic gait

Senile gait with short steps Common ambulation pattern in elderly patients characterized by a marked reduction in step length with maintenance of the base of support, frequently caused by multiple lacunar infarcts of subcortical vascular origin.

Also: microangiopathic senile wandering · march of small steps of the old man · senile gait

Tabetic march Abnormal walking pattern characterized by an unstable and uncoordinated gait in which the patient raises the feet in an exaggerated manner and throws them hard against the ground to receive compensatory tactile information. It is typical of tabes dorsalis secondary to neurosyphilis and severe lesions of the posterior spinal cords.

Also: proprioceptive ataxic wandering · tapping gait · tabetic gait

Meralgia paresthetica Pain syndrome caused by compression or mechanical entrapment of the lateral femoral cutaneous nerve as it passes under the inguinal ligament. It causes burning pain, numbness and hypoesthesia in the anterolateral region of the thigh without affecting muscle strength.

Also: femoralcutaneous nerve entrapment · lateral femoral cutaneous neuralgia · meralgia paresthetica · lateral femoral cutaneous nerve entrapment · femoral-cutaneous compression in the inguinal ligament

Acute flaccid myelitis Neurological condition of sudden onset that presents with flaccid and asymmetric muscle weakness of one or more extremities together with loss of deep tendon reflexes, secondary to inflammation of the spinal gray matter. It is frequently caused by non-polioviral enterovirus infections that destroy the motor neurons of the anterior horn.

Also: acute flaccid paralysis syndrome · spinal gray matter dysfunction · acute flaccid myelitis

Transverse myelitis Acute or subacute inflammatory process that affects both sides of a specific segment of the spinal cord, causing motor, sensory and autonomic dysfunction below the level of the injury. Its etiology can be autoimmune, post-infectious or idiopathic.

Also: inflammatory spinal cord dysfunction · spinal transversitis · acute inflammatory myelopathy

Idiopathic transverse myelitis Acute or subacute monophasic inflammatory process that damages the spinal cord bilaterally in the absence of an identifiable underlying multisystem or neurological disease, clinically presenting with flaccid paraparesis or quadriparesis, defined sensory level and early sphincter dysfunction.

Also: idiopathic inflammatory myelopathy · idiopathic spinal cord transversitis · idiopathic transverse myelitis

Recurrent idiopathic transverse myelitis Inflammatory process of the spinal cord that presents multiple recurrent and independent episodes of bilateral segmental spinal cord dysfunction in the absence of other demonstrable underlying systemic autoimmune or demyelinating pathology.

Also: idiopathic recurrent spinal cord transversitis · episodic inflammatory myelopathy · recurrent idiopathic transverse myelitis

Cervical spondylotic myelopathy Chronic clinical condition characterized by progressive spinal cord dysfunction due to narrowing of the spinal canal due to osteophytes and disc deformities of the cervical spine. It manifests with a wide-based gait, symmetrical distal paresthesias, and upper motor neuron signs in the lower extremities.

Also: degenerative cervical canal stenosis · cervical degenerative myelopathy · spondylotic cervical myelopathy

Cervical spondylotic myelopathy Chronic and progressive dysfunction of the cervical spinal cord caused by mechanical compression of the nervous structures secondary to osteoarticular degenerative changes of the spine. It manifests itself with unstable gait, spasticity in the lower extremities and loss of fine manual dexterity.

Also: degenerative cervical spinal cord compression · myelopathic cervical spondylosis · cervical myelopathy

Progressive cervical spondylotic myelopathy Chronic and constant form of course in which arthritic changes in the cervical spine compress the spinal cord, causing a rigid gait, increasing clumsiness of the hands and progressive proprioceptive sensory impairment of an irreversible course.

Also: progressive cervical canal stenosis · chronic compressive myelopathy · progressive cervical spondylotic myelopathy

Acute traumatic myelopathy Sudden and severe injury to the spinal cord secondary to direct mechanical forces of compression, contusion or spinal traction due to vertebral fracture-dislocation, which causes an initial phase of spinal shock followed by definitive spastic paraparesis or quadriparesis.

Also: acute traumatic spinal cord injury · acute spinal section · acute traumatic myelopathy

Vacuolar myelopathy Progressive degeneration of the posterior and lateral cords of the spinal cord characterized by the formation of vacuoles in the myelin sheath, characteristically associated with advanced infection by the human immunodeficiency virus. Clinically it causes progressive spastic paraparesis and loss of vibratory sensitivity in the lower extremities.

Also: medullary vacuolar degeneration · HIV-associated myelopathy · HIV-associated myelopathy

Mononeuritis multiplex Pattern of peripheral neurological damage characterized by the successive, asymmetric and multifocal involvement of two or more individual peripheral nerve trunks in different anatomical areas of the body. It is a clinical manifestation highly suggestive of systemic or infiltrative vasculitic processes.

Also: multiple mononeuropathy · multifocal peripheral nerve involvement · mononeuritis multiplex

Ulnar mononeuropathy of the wrist Compressive lesion of the ulnar nerve located in Guyon's canal in the wrist, which is manifested by pain, paresthesias and hypoesthesia predominantly palmar limited to the fifth finger and half of the fourth finger of the hand, with preservation of sensitivity in the dorsal aspect of said fingers.

Also: Guyon canal syndrome · distal ulnar neuropathy · Guyon's canal syndrome

Brain death Complete, irreversible, clinical loss of all brain functions, including the brainstem, determined by the documented absence of consciousness, brainstem reflexes, and spontaneous breathing on a formalized apnea test. It represents the legal death of the individual under strict neurological criteria.

Also: brain death · irreversible cessation of brain functions · brain death

Glossopharyngeal neuralgia Disorder characterized by intense, paroxysmal and involuntary attacks of lancinating pain located at the base of the tongue, tonsillar fossa, pharynx or posterior portion of the external auditory canal, triggered by swallowing, chewing or speaking.

Also: paroxysmal glossopharyngeal pain · painful tic of the ninth pair · glossopharyngeal neuralgia

Trigeminal neuralgia Craniofacial pain disorder characterized by recurrent attacks of lancinating, paroxysmal pain of extreme intensity in the territory of distribution of one or more branches of the fifth cranial nerve. It is classically caused by neurovascular compression in the area of entry of the nerve root into the brainstem.

Also: painful facial tic · paroxysmal trigeminal pain · trigeminal neuralgia

Idiopathic trigeminal neuralgia Paroxysmal facial pain disorder characterized by recurrent episodes of lancinating and unbearable pain in the territory of distribution of the branches of the trigeminal nerve, in the absence of neurovascular compression demonstrable in magnetic resonance studies.

Also: primary trigeminal pain · idiopathic painful tic · idiopathic trigeminal neuralgia

Postherpetic neuralgia Chronic, severe and debilitating neuropathic pain that persists in the affected dermatomal distribution for more than three months after the healing of the skin lesions of herpes zoster. It occurs due to axonal damage and persistent hyperexcitability of the nerve roots and dorsal root ganglia.

Also: chronic post-herpetic pain · herpes zoster neuralgia · postherpetic neuralgia

Previous optic neuritis Acute inflammation of the orbital portion of the optic nerve that clinically presents with sudden and painful loss of visual acuity, alteration in color perception and relative afferent pupillary defect. It frequently constitutes the first clinical manifestation or the initial outbreak of multiple sclerosis.

Also: inflammatory optic papillitis · intraocular optic neuritis · previous optic neuritis

Vestibular neuritis Neurotological disorder characterized by an acute attack of severe rotational vertigo, lasting hours or days, accompanied by nausea, vomiting and spontaneous unidirectional nystagmus, in the absence of auditory symptoms. It is classically attributed to an inflammatory process of viral origin that affects the ipsilateral vestibular nerve.

Also: vestibular neuronitis · acute peripheral vestibulopathy · vestibular neuritis

Neurocysticercosis Parasitosis of the central nervous system caused by the invasion of the larval form of Taenia solium in the brain parenchyma, subarachnoid space or ventricles. It constitutes one of the main preventable causes of acquired secondary epileptic seizures in developing countries.

Also: cerebral cysticercosis · infestation by cerebral tapeworm larvae · neurocysticercosis

Optic neuromyelitis Autoimmune demyelinating and inflammatory disease of the central nervous system characterized by recurrent and severe attacks of optic neuritis and longitudinally extensive transverse myelitis. It is primarily mediated by autoantibodies directed against aquaporin-4 water channels in astrocytes.

Also: Devic's disease · neuromyelitis optica spectrum disorder · NMO

Ulnar neuropathy in the elbow Mononeuropathy due to compressive entrapment of the ulnar nerve as it passes through the epitrochleo-olecranon canal of the elbow joint. It causes weakness of the intrinsic muscles of the hand, paresthesias and loss of sensitivity in the fifth finger and ulnar half of the fourth finger.

Also: cubital canal syndrome · compression of the ulnar nerve · ulnar neuropathy at the elbow

Severe ulnar neuropathy in the elbow Mononeuropathy due to entrapment of the ulnar nerve in the epitrochleo-olecranon canal characterized by marked atrophy of the interosseous muscles of the hand and total loss of sensitivity in the fifth finger with a chronic course.

Also: severe ulnar canal syndrome · ulnar axonal involvement in the elbow · severe ulnar neuropathy at the elbow

Charcot-Marie-Tooth neuropathy Heterogeneous group of hereditary motor and sensory polyneuropathies characterized by progressive distal muscle weakness, atrophy in the lower extremities with a stork-leg appearance, and generalized hyporeflexia. Its etiology lies in genetic abnormalities of the structural proteins of myelin or the peripheral axon itself.

Also: hereditary demyelinating polyneuropathy · Charcot-Marie-Tooth disease

Hereditary neuropathy with susceptibility to pressure paralysis Autosomal dominant genetic disorder caused by deletion in the PMP22 gene that causes extreme vulnerability of peripheral nerves to suffer demyelination due to minor compression. It causes recurrent, self-limited episodes of focal muscle weakness and paresthesias in the extremities.

Also: recurrent compressive neuropathy · HNPP · hereditary neuropathy with liability to pressure palsies

Uremic neuropathy Mixed distal, sensory-motor and symmetrical polyneuropathy that characteristically affects patients with end-stage renal disease and extremely decreased renal clearance. It causes paresthesia with a sock pattern, loss of Achilles tendon reflexes and weakness of the intrinsic muscles of the foot.

Also: nervous involvement due to kidney failure · uremia neuropathy · uremic neuropathy

Vertical gaze ophthalmoplegia Paralysis or marked restriction of pursuit and saccadic eye movements in an ascending or descending vertical direction, usually secondary to dysfunctions of the mesencephalic tectum. It is a fundamental semiological component of Parinaud syndrome and progressive supranuclear palsy.

Also: vertical gaze paralysis · vertical gaze dysfunction · vertical gaze palsy

Chronic progressive external ophthalmoplegia Mitochondrial myopathy with a slow and progressive course characterized by symmetrical and progressive weakness of the extraocular muscles and bilateral eyelid ptosis, which associates preservation of internal pupillary responses to light. It is associated with single or multiple deletions in mitochondrial DNA.

Also: CPEO · chronic mitochondrial ocular palsy · chronic progressive external ophthalmoplegia

Internuclear ophthalmoplegia Alteration of horizontal conjugate gaze characterized by weakness or absence of adduction of the eye ipsilateral to the lesion during lateral gaze, accompanied by abduction nystagmus in the contralateral eye. It is caused by a focal or demyelinating lesion in the medial longitudinal fasciculus of the brain stem.

Also: medial longitudinal fasciculus injury · conjugate gaze dysfunction · internuclear ophthalmoplegia · medial longitudinal fasciculus paralysis · conjugate adduction dysfunction · alteration of conjugate eye movement

Bilateral internuclear ophthalmoplegia Severe alteration of conjugate eye movements characterized by the inability to adduct in both eyes during lateral gaze, secondary to bilateral demyelinating lesions of the medial longitudinal fasciculus in the brainstem. It is a highly characteristic semiological finding of multiple sclerosis in young patients.

Also: bilateral lesion of the FLM · bilateral adduction paralysis · bilateral internuclear ophthalmoplegia

Acute bilateral internuclear ophthalmoplegia Sudden bilateral horizontal conjugate gaze palsy with abolition of adduction of both eyes and symmetrical contralateral abduction nystagmus, highly suggestive of acute inflammatory demyelinating lesion in the upper brain stem.

Also: acute bilateral MLF palsy · sudden onset bilateral internuclear ophthalmoplegia · acute bilateral internuclear ophthalmoplegia

Progressive bilateral internuclear ophthalmoplegia Affectation of horizontal conjugate gaze characterized by paralysis of adduction movement in both eyes accompanied by abduction nystagmus in the opposite eye that gradually worsens gradually, characteristic of the progression of primary progressive multiple sclerosis.

Also: progressive bilateral MLF palsy · slowly progressive bilateral internuclear ophthalmoplegia · progressive bilateral internuclear ophthalmoplegia

Progressive bilateral internuclear demyelinating ophthalmoplegia Severe alteration of conjugate gaze eye movements characterized by paralysis of adduction movement in both eyes accompanied by symmetrical contralateral abduction nystagmus that gradually worsens slowly and inexorably, characteristic of the progression of primary progressive multiple sclerosis.

Also: progressive bilateral demyelinating MLF paralysis · bilateral internuclear ophthalmoplegia with slow demyelinating progression · progressive demyelinating bilateral internuclear ophthalmoplegia

Unilateral internuclear ophthalmoplegia Focal palsy of ocular adduction limited to a single eye during attempted contralateral conjugate horizontal gaze, with compensatory abduction nystagmus in the opposite eye, secondary to a single demyelination plaque or ischemic infarction in the ipsilateral medial longitudinal fasciculus.

Also: unilateral paralysis of the FLM · ipsilateral internuclear ophthalmoplegia · unilateral internuclear ophthalmoplegia

Progressive bulbar palsy Neurodegenerative disease characterized by the selective and early involvement of bulbar motoneurons located in the motor nuclei of the brainstem. It progressively causes severe flaccid type dysarthria, progressive dysphagia to liquids and solids, atrophy with marked fasciculations in the tongue and severe risk of aspiration.

Also: pure bulbar palsy · trunk motor neuron condition · progressive bulbar palsy

Bell's palsy Acute peripheral motor paralysis of the facial mimicry muscles, unilateral in nature and usually idiopathic in origin or attributed to the reactivation of the herpes simplex virus in the geniculate ganglion. It causes the loss of the ability to wrinkle the forehead, close the eye and diverts the corner of the mouth towards the healthy side.

Also: idiopathic peripheral facial palsy · acute facial mononeuropathy · Bell's palsy

Erb-Duchenne palsy Injury to the upper portion of the brachial plexus that mainly affects the C5 and C6 nerve roots, causing weakness or paralysis of the deltoid, biceps and anterior brachialis muscles. The affected upper extremity typically assumes a position of adduction, internal rotation, and pronation of the forearm.

Also: upper brachial plexus palsy · C5-C6 root injury · Erb's palsy

Klumpke's palsy Injury to the lower portion of the brachial plexus that selectively affects the C8 and T1 nerve roots, resulting in paralysis of the intrinsic muscles of the hand and finger flexors. Clinically it manifests itself with claw hand deformity and can be associated with Horner syndrome due to sympathetic involvement.

Also: inferior brachial plexus palsy · C8-T1 root injury · Klumpke's palsy

Recurrent peripheral facial palsy Recurrent unilateral motor palsy of facial mimicry that presents multiple episodes of involvement of the seventh cranial nerve, usually idiopathic or structurally associated with Melkersson-Rosenthal syndrome.

Also: recurrent facial paralysis · episodic peripheral facial involvement · recurrent facial palsy

Hypokalemic periodic paralysis Autosomal dominant hereditary muscle channelopathy characterized by self-limited episodes of generalized flaccid muscle weakness or paralysis triggered by low serum potassium levels. Attacks are usually precipitated by heavy carbohydrate intake, prolonged rest, or intense physical stress.

Also: familial hypokalemic paralysis · episodic hypokalemic myopathy · hypokalemic periodic paralysis

Progressive supranuclear palsy Tauopathy-type neurodegenerative disorder characterized by atypical parkinsonism, early postural instability with frequent falls, axial rigidity and vertical conjugate gaze paralysis. It is associated with severe atrophy of the midbrain and globus pallidus.

Also: PSP · Steele-Richardson-Olszewski syndrome · supranuclear palsy

Brachial plexopathy Neurological disorder derived from injury, traction, compression or inflammation of the nerve trunks that constitute the brachial plexus, from the cervical roots C5 to T1. It causes a complex, patchy motor and sensory deficit in the ipsilateral upper extremity that does not correlate with the distribution of a single peripheral nerve.

Also: brachial plexus injury · brachial trunk dysfunction · brachial plexopathy

Idiopathic brachial plexopathy Inflammatory process of an immune-mediated or autoimmune nature that affects the brachial plexus acutely unilaterally, causing unbearable scapular pain followed by multifocal flaccid muscle weakness of the upper extremity with preservation of bone integrity.

Also: idiopathic neuralgic shoulder amyotrophy · acute brachial plexitis · idiopathic brachial plexopathy

Bilateral traumatic brachial plexopathy Severe injury due to forced traction of both brachial plexuses secondary to high-energy trauma or traffic accidents, clinically presenting with complete flaccid paralysis and permanent sensory loss of both upper extremities.

Also: traumatic bilateral brachial palsy · bilateral brachial plexus traction · bilateral traumatic brachial plexopathy

Severe bilateral traumatic brachial plexopathy Severe injury due to forced traction of both brachial plexuses secondary to high-energy trauma or traffic accidents, clinically presenting with complete flaccid paralysis and permanent sensory loss of both upper extremities with proven root avulsion.

Also: severe traumatic bilateral brachial palsy · bilateral brachial plexus traction with avulsion · severe bilateral traumatic brachial plexopathy

Lumbosacral plexopathy Peripheral neurological involvement due to direct damage, ischemia or inflammatory compression of the lumbosacral roots and trunks of the lumbosacral plexus, which causes neuropathic pain radiating to the lower extremities, muscle weakness of multifocal distribution and loss of patellar and Achilles reflexes.

Also: lumbar plexual neuropathy · lumbosacral plexus involvement · lumbosacral plexopathy

Idiopathic lumbosacral plexopathy Inflammatory process of immune-mediated or self-limited autoimmune origin that presents with severe acute unilateral hip and thigh pain followed by multifocal weakness of the lower extremity in an asymmetrical manner, in the absence of detectable tumor or compressive pathology.

Also: lumbosacral plexitis · lumbosacral neuralgic amyotrophy · idiopathic lumbosacral plexopathy

Chronic inflammatory demyelinating polyneuropathy Progressive or recurrent autoimmune disorder characterized by symmetrical muscle weakness, both proximal and distal, persistent hyporeflexia, and diffuse sensory disturbance lasting more than two months. Pathophysiologically, it presents with disseminated segmental demyelination of the peripheral nerves and spinal roots.

Also: CIDP · chronic inflammatory demyelinating polyradiculoneuropathy · chronic inflammatory demyelinating polyneuropathy

Prosopagnosia Neurological disorder characterized by the inability to recognize familiar or previously known faces, while retaining the ability to identify other visual objects. It usually occurs due to bilateral or right hemisphere lesions in the fusiform gyrus.

Also: face blindness · visual agnosia of faces · face blindness

Pseudotumor cerebri Syndrome characterized by symptoms of intracranial hypertension, such as pulsating headache, diplopia due to sixth cranial nerve palsy and bilateral papilledema with risk of optic atrophy, in the presence of normal-sized ventricles and cerebrospinal fluid of normal composition and high opening pressure.

Also: idiopathic intracranial hypertension · benign intracranial hypertension · pseudotumor cerebri

Argyll Robertson pupil Bilateral pupillary anomaly of a classic nature characterized by the abolition of the photomotor reflex with preservation of the accommodation and convergence reflex of the eye. It is a highly specific semiological finding of upper brainstem involvement due to late neurosyphilis.

Also: preserved accommodation-convergence pupil · argyll pupillary sign · Argyll Robertson pupil

Compressive cervical radiculopathy Disorder characterized by lancinating radiating pain along a specific dermatome of the upper limb, accompanied by hyporeflexia and weakness in the muscles innervated by a cervical nerve root, usually due to a herniated intervertebral disc or foraminal stenosis due to bone anointing.

Also: cervical root pain · cervical root compression · cervical radiculopathy · compressive cervical radiculitis

Bilateral compressive cervical radiculopathy Painful radicular involvement that simultaneously involves multiple cervical roots on both sides secondary to severe osteoarthritic degenerative changes of the cervical spine, clinically presenting with pain, distal paresthesias and bilateral manual clumsiness.

Also: bilateral cervical root compression · bilateral cervical radicular pain · bilateral compressive cervical radiculopathy

Progressive bilateral compressive cervical radiculopathy Painful radicular involvement that simultaneously involves multiple cervical roots on both sides secondary to severe osteoarthritic degenerative changes of the cervical spine with a slowly progressive course.

Also: progressive bilateral cervical root compression · chronic bilateral cervical radicular pain · progressive bilateral compressive cervical radiculopathy

Severe progressive bilateral compressive cervical radiculopathy Painful radicular involvement that simultaneously involves multiple cervical roots on both sides secondary to severe osteoarthritic degenerative changes of the cervical spine with severe canal stenosis with a chronic clinical course of severe progressive evolution.

Also: severe progressive bilateral cervical root compression · severe chronic progressive bilateral cervical radicular pain · severe progressive bilateral compressive cervical radiculopathy

Severe bilateral compressive cervical radiculopathy Painful radicular involvement that simultaneously involves multiple cervical roots on both sides secondary to severe osteoarthritic degenerative changes of the cervical spine with associated severe canal stenosis.

Also: severe bilateral cervical root compression · severe bilateral cervical radicular pain of chronic course · severe bilateral compressive cervical radiculopathy

Unilateral compressive cervical radiculopathy Disorder characterized by severe radicular pain radiating to the upper limb, loss of the bicipital or tricipital reflex and hypoesthesia located exclusively in the dermatome corresponding to a single cervical nerve root, due to disc herniation or unilateral osteoarthritis.

Also: unilateral cervical root compression · ipsilateral cervical radicular pain · unilateral compressive cervical radiculopathy

Progressive unilateral compressive cervical radiculopathy Disorder characterized by severe radicular pain radiating to the upper limb, loss of the bicipital reflex and progressive hypoesthesia localized exclusively in the dermatome corresponding to a single cervical nerve root, due to a persistent compressive disc herniation.

Also: progressive unilateral cervical root compression · progressive ipsilateral cervical radicular pain · progressive unilateral compressive radiculopathy

Lumbosacral radiculopathy Neurological disorder caused by compression, stretching or inflammation of one or more nerve roots of the lumbosacral spine, usually secondary to herniated discs or foraminal stenosis. It is characterized by neuropathic pain that radiates along a specific dermatome of the lower extremity, accompanied by muscle weakness or hyporeflexia.

Also: lumbar radicular pain · lumbosacral root compression · lumbosacral radiculopathy

Bilateral compressive lumbosacral radiculopathy Painful involvement of root origin that simultaneously involves multiple lumbosacral roots bilaterally secondary to severe stenosis of the lumbar canal, clinically presenting with neuropathic pain, weakness and neurogenic claudication of gait.

Also: bilateral lumbosacral root compression · lumbar root canal stenosis · bilateral compressive lumbosacral radiculopathy

Progressive unilateral compressive lumbosacral radiculopathy Radicular involvement that progressively compromises a lumbosacral root with increasing motor weakness for dorsiflexion of the foot and intractable radiating pain secondary to a giant extruded disc herniation.

Also: progressive lumbosacral root compression · unilateral progressive lumbar radicular pain · progressive unilateral compressive lumbosacral radiculopathy

Lumbosacral radiculopathy lumbosciatalgia Radicular pain disorder characterized by neuropathic pain that radiates along the path of the sciatic nerve from the lumbar region to the foot, associated with compression of the L5 or S1 nerve roots of the spine.

Also: lumbosacral radicular sciatica · compressive sciatic pain · compressive lumbosacral radiculopathy

Hoffmann's sign Involuntary flexion reflex of the distal phalanx of the thumb and index finger triggered by rapid pinching or sudden compression of the distal phalanx of the third finger of the hand. Its presence denotes hyperexcitability of the pyramidal pathway or upper motor neuron injury at the cervical level.

Also: Hoffmann's digital reflection · Hoffmann's pyramidal response · Hoffmann's sign

Hoover sign Exploratory maneuver useful for differentiating functional or simulated paralysis or weakness of the lower extremity from organic causes. It is based on the fact that simulated weakness shows a normal involuntary contralateral extension force when the patient is asked to flex the hip on the supposedly hemiplegic side.

Also: Hoover test · contralateral pressure maneuver · Hoover's sign

Lhermitte sign Sensation of brief, descending electric shock along the spine and extremities, usually triggered by active or passive flexion of the neck. It is a classic sign of dysfunction or active demyelination in the posterior cords of the cervical spinal cord.

Also: Lhermitte discharge phenomenon · cervical flexion sign · Lhermitte's sign

Demyelinating Lhermitte sign Phenomenon characterized by the sudden and involuntary appearance of an electric shock sensation along the back triggered by neck flexion in patients with active demyelination plaques located specifically in the cervical spinal cord.

Also: Lhermitte sign in multiple sclerosis · cervical flexion paresthesia · demyelinating Lhermitte's sign

Romberg sign Finding in the neurological examination where the patient markedly loses postural balance and oscillates or falls when closing the eyes while standing with the feet together. It reveals a dysfunction in the pathway of conscious proprioception or in vestibular function of a compensatory nature.

Also: Romberg test · proprioceptive instability when closing eyes · Romberg's sign

Labyrinthine Romberg sign Postural instability that typically appears or is accentuated after a brief period of latency when the patient's eyes close, characterized by a slow and systematic deviation or fall of the body directed specifically towards the side of the affected or hypofunctioning ear.

Also: labyrinthine detour in Romberg · vestibular Romberg drop · labyrinthine Romberg sign

Sensitive Romberg sign Postural instability characterized by the patient's inability to remain standing and firm without noticeable oscillations or loss of balance due to occlusion of vision, caused by degenerative involvement of the thick fibers of the posterior cords of the spinal cord.

Also: Romberg due to proprioceptive deficit · posterior cord instability · sensory Romberg sign

Spinal sensory Romberg sign Severe postural instability that causes oscillations and immediate fall of the patient when closing the eyes while standing, secondary to demyelination or selective atrophy of the thick proprioceptive fibers in the posterior portion of the spinal cord.

Also: Romberg spinal proprioceptive · instability of posterior spinal cords · spinal sensory Romberg sign

Acute vestibular Romberg sign Postural instability characterized by the slow and systematic deviation or fall of the body directed specifically towards the side of the hypofunctioning labyrinth after a short period of latency when the patient's eyes close.

Also: unilateral vestibular Romberg drop · vestibular postural instability · acute vestibular Romberg sign

Acute bilateral vestibular Romberg sign Postural instability characterized by the slow and systematic deviation or fall of the body directed specifically towards the side of the hypofunctioning labyrinth after a short period of latency when the patient's eyes close.

Also: bilateral vestibular Romberg drop · bilateral vestibular postural instability · acute bilateral vestibular Romberg sign

Acute unilateral vestibular Romberg sign Postural instability characterized by the slow and systematic deviation or fall of the body directed specifically towards the side of the hypofunctioning labyrinth after a short period of latency when the patient's eyes close.

Also: acute unilateral vestibular Romberg drop · acute unilateral vestibular postural instability · acute unilateral vestibular Romberg sign

Broca's aphasic syndrome with apraxia Severe non-fluent language disorder of a motor nature in which the patient has extreme difficulty formulating complex words, characteristically associated with apraxia of the orofacial muscles, drastically limiting spontaneous communication.

Also: severe combined expressive aphasia · Broca's verbal apraxia · Broca's aphasia with apraxia

Broca's aphasic syndrome with severe apraxia Motor language disorder characterized by mutism or spontaneous language limited to single-syllable stereotypies, systematically associated with severe orofacial apraxia that completely prevents imitating facial movements or articulating words.

Also: non-fluent aphasia with severe orofacial apraxia · Broca's mutism with motor apraxia · severe Broca's aphasia with severe apraxia

Wernicke's aphasic syndrome with a progressive course Fluid type language comprehension disorder that sets in insidiously and gradually worsens, characterized by fluent jargaphasia in the context of left posterior temporal lobar atrophy of neurodegenerative cause.

Also: primary progressive receptive aphasia · semantic variant of the APP · progressive Wernicke's aphasia

Wernicke's aphasic syndrome with primary progressive course Fluid type language comprehension disorder that sets in insidiously and gradually worsens, characterized by fluent jargaphasia in the context of left posterior temporal lobar atrophy of neurodegenerative cause.

Also: classical primary progressive receptive aphasia · semantic variant of the progressive APP · primary progressive Wernicke's aphasia

Severe aphasic Wernicke syndrome Fluent language disorder in which the understanding of oral and written language is completely abolished, with the patient manifesting a fluent but totally incomprehensible spoken language of the jargaphasia type with abundant neologisms and lack of awareness of the defect itself.

Also: global sensory aphasia · Wernicke's aphasia of no comprehension · severe Wernicke's aphasia

Aphasic transcortical motor syndrome Non-fluent language disorder characterized by the presence of extremely scarce and laborious spontaneous language but with a fully preserved ability to repeat phrases and spoken words in a characteristic manner. It occurs due to lesions located in the supplementary prefrontal cortex external to Broca's area.

Also: transcortical motor aphasia · motor aphasia with preserved repetition · transcortical motor aphasia

Severe transcortical motor aphasic syndrome Non-fluent language disorder characterized by a drastic reduction in spontaneous language that evolves towards absolute mutism with the ability to repeat complex phrases in a completely preserved characteristic manner.

Also: severe motor transcortical aphasia · transcortical motor mutism · severe transcortical motor aphasia

Chronic severe transcortical motor aphasic syndrome Non-fluent language disorder characterized by a drastic reduction in spontaneous language that evolves towards absolute mutism with the ability to repeat complex phrases in a characteristically completely preserved manner with a permanent course.

Also: severe chronic motor transcortical aphasia · definitive transcortical motor mutism · chronic severe transcortical motor aphasia

Permanent chronic severe transcortical motor aphasic syndrome Non-fluent language disorder characterized by a drastic reduction in spontaneous language that evolves towards absolute mutism with the ability to repeat complex phrases in a completely preserved characteristic manner with a permanent and irreversible course.

Also: severe irreversible chronic motor transcortical aphasia · permanent definitive motor transcortical mutism · permanent chronic severe transcortical motor aphasia

Adie syndrome Pupillary disorder characterized by a unilateral dilated pupil that shows very slow and incomplete constriction to direct light stimulation, but with almost normal slow constriction during near accommodation. Pathophysiologically, it is due to postganglionic denervation of the pupillary sphincter due to damage to the ciliary ganglion.

Also: Adie's tonic pupil · Holmes-Adie syndrome · tonic pupil

Anton syndrome Cortical blindness of bilateral cause where the patient systematically denies the loss of his visual capacity and conspires about what he believes he sees, due to the disconnection between the damaged primary visual areas and the association areas of the parietal association cortex. It characteristically occurs after vascular accidents that obliterate both posterior cerebral arteries.

Also: denied cortical blindness · Anton-Babinski syndrome · visual anosognosia

Balint syndrome Severe neuropsychological disorder of visual perception characterized by the classic triad of simultagnosia, ocular apraxia and optic ataxia. It is characteristically caused by extensive bilateral lesions affecting the parieto-occipital association regions.

Also: parieto-occipital perceptual dysfunction · Balint syndrome · Balint triad

Parieto-occipital Balint syndrome Severe perceptual dysfunction characterized by visual simultagnosia and ocular apraxia due to destructive lesions of bilateral ischemic or traumatic origin located specifically in the association cortex of the parieto-occipital junction.

Also: Balint-Holmes syndrome · balint's visual agnosia · parieto-occipital Balint syndrome

Brown-Séquard syndrome Myelopathy caused by the lesional hemisection of the spinal cord, characterized by ipsilateral motor paralysis below the lesion, loss of proprioceptive and vibration sensitivity on the same side, combined with loss of thermoalgesic sensitivity on the contralateral side.

Also: spinal hemisection syndrome · asymmetric spinal cord dysfunction · Brown-Séquard syndrome

Incomplete Brown-Séquard syndrome Myelopathy characterized by a partial hemisection of the spinal cord of compressive or ischemic origin in which the symptoms of ipsilateral motor paralysis and proprioceptive loss are associated with an asymmetric or incomplete impairment of contralateral thermal sensitivity.

Also: incomplete spinal cord hemisection · Partial asymmetric Brown-Séquard · incomplete Brown-Séquard syndrome

Bilateral post-traumatic Brown-Séquard syndrome Myelopathy usually caused by penetrating injuries that cause an incomplete asymmetric section of the spinal cord, manifesting clinically with predominantly contralateral weakness and ipsilateral proprioceptive loss.

Also: post-traumatic incomplete spinal cord hemisection · Brown-Séquard asymmetric partial traumatic · traumatic incomplete Brown-Séquard syndrome

Unilateral post-traumatic Brown-Séquard syndrome Myelopathy of traumatic origin caused by penetrating trauma that specifically damages half of the spinal cord unilaterally, manifesting clinically with ipsilateral motor paralysis with contralateral thermoalgesic sensory loss.

Also: unilateral traumatic spinal cord hemisection · Ipsilateral post-traumatic Brown-Séquard · unilateral traumatic Brown-Séquard syndrome

Traumatic Brown-Séquard syndrome Myelopathy usually caused by penetrating injuries, stab wounds or displaced vertebral fractures that cause a section of half of the spinal cord, manifesting clinically with ipsilateral motor paralysis and proprioceptive loss together with loss of contralateral thermoalgesic sensation.

Also: traumatic spinal cord hemisection · Post-traumatic Brown-Séquard · traumatic Brown-Séquard syndrome

Incomplete traumatic Brown-Séquard syndrome Myelopathy caused by penetrating trauma that partially damages one half of the spinal cord, manifesting clinically with ipsilateral motor paralysis with partial preservation of contralateral tactile or proprioceptive sensitivity.

Also: asymmetric traumatic spinal cord hemisection · Partial post-traumatic Brown-Séquard

Captivity syndrome Serious clinical condition caused by the selective destruction of the efferent motor pathways of the ventral portion of the cerebral pons, usually secondary to an infarction of the basilar artery. It causes complete quadriplegia and paralysis of the lower cranial nerves, preserving the level of alertness, cognitive function and vertical eye movements.

Also: locked-in syndrome · pontine motor closure · locked-in syndrome

Complex regional pain syndrome Chronic unilateral painful condition of a neuropathic nature that usually affects an extremity after a previous trauma, characterized by disproportionate pain associated with severe hyperalgesia, local blood flow alterations, regional edema and trophic skin or bone changes.

Also: reflex sympathetic dystrophy · Sudeck syndrome · complex regional pain syndrome

Dravet syndrome Developmental epileptic encephalopathy with a severe genetic basis, characterized by prolonged and recurrent clonic or tonic seizures, frequently triggered by fever, that begin refractoryly during the first year of life. It is mainly caused by de novo mutations in the SCN1A sodium channel gene.

Also: severe myoclonic epilepsy of childhood · infantile SCN1A channelopathy · Dravet syndrome

Fahr syndrome Disorder characterized by the presence of idiopathic, bilateral and symmetrical calcifications in the basal ganglia, cerebral cortex and cerebellum, in the absence of calcium or phosphorus metabolic disorders. It manifests clinically with extrapyramidal dysfunction, parkinsonism, chorea, cognitive impairment and psychiatric disorders.

Also: idiopathic calcification of the basal ganglia · Fahr's disease · Fahr syndrome

Gerstmann syndrome Neuropsychological dysfunction characterized by the association of agraphia, acalculia, right-left disorientation and digital agnosia. It is typically caused by focal lesions affecting the angular gyrus of the parietal lobe of the dominant cerebral hemisphere.

Also: angular parietal syndrome · Gerstmann syndrome · Gerstmann tetrad

Guillain-Barré syndrome Acute inflammatory demyelinating polyradiculoneuropathy of an immune-mediated nature, characterized by symmetrical muscle weakness of an ascending nature and loss of deep tendon reflexes, frequently preceded by a respiratory or gastrointestinal infection. In severe cases it can compromise the respiratory muscles and require ventilatory support.

Also: SGB · acute demyelinating polyneuropathy · Guillain-Barré syndrome

Acute axonal Guillain-Barré syndrome Subtype of acute autoimmune polyradiculoneuropathy in which there is a direct attack mediated by antibodies against the membrane of the motor and sensory axon, instead of against the peripheral myelin. It is semiologically characterized by flaccid muscle weakness and extremely rapid ascending paralysis with slow and incomplete clinical recovery.

Also: AMAN · acute motor axonal polyneuropathy · acute motor axonal neuropathy

Classic demyelinating Guillain-Barré syndrome Typical clinical form of acute inflammatory polyradiculoneuropathy in which the immune system destroys the myelin sheath of the peripheral nerves, clinically presenting with an ascending and symmetrical flaccid paralysis of the extremities with areflexia and albumino-cytological dissociation in the cerebrospinal fluid.

Also: AIDP · classical acute inflammatory demyelinating polyneuropathy · acute inflammatory demyelinating polyradiculoneuropathy

Recurrent demyelinating Guillain-Barré syndrome Unusual clinical form of acute inflammatory polyradiculoneuropathy in which the patient experiences multiple recurrent and independent episodes of flaccid paralysis with interictal complete or partial recovery.

Also: Recurring GBS · acute recurrent demyelinating polyradiculoneuropathy · recurrent acute inflammatory demyelinating polyradiculoneuropathy

Acute motor Guillain-Barré syndrome Subtype of acute inflammatory polyradiculoneuropathy clinically characterized by flaccid and symmetrical muscle paralysis of a purely motor nature, without involvement of peripheral sensory pathways.

Also: AMAN classic · acute axonal motor polyradiculopathy · acute motor axonal Guillain-Barré syndrome

Ocular Horner syndrome Defect of ocular sympathetic innervation characterized by the unilateral presence of pupillary miosis, discrete eyelid ptosis due to weakness of the superior tarsal muscle and anhidrosis of the corresponding hemiface. It originates from lesions in the sympathetic pathway of three neurons from the hypothalamus to the orbit.

Also: ocular sympathetic palsy · Claude Bernard-Horner syndrome · Horner's syndrome

Postganglionic Horner syndrome Unilateral ocular sympathetic defect caused by lesions located in the third neuron of the cervical sympathetic pathway, which runs along the internal carotid artery to the orbit, typically characterized by the total absence of anhidrosis in the patient's face.

Also: postganglionic sympathetic palsy · sympathetic carotid involvement · postganglionic Horner's syndrome

Preganglionic Horner syndrome Oculo-sympathetic defect caused by lesions located in the second neuron of the cervical sympathetic pathway, commonly associated with tumors located in the pulmonary apex that compress the stellate ganglion.

Also: Horner syndrome secondary to Pancoast tumor · preganglionic sympathetic paralysis · preganglionic Horner's syndrome

Lambert-Eaton syndrome Autoimmune presynaptic disorder of the neuromuscular junction characterized by progressive proximal muscle weakness, hyporeflexia, and mild autonomic dysfunction that temporarily improves with repeated voluntary muscle contraction. It is caused by the presence of autoantibodies directed against voltage-gated calcium channels in the presynaptic motor nerve ending.

Also: Lambert-Eaton myasthenic syndrome · dysfunction of presynaptic calcium channels · Lambert-Eaton myasthenic syndrome

Dorsal Parinaud syndrome Ophthalmoplegia characterized by paralysis of the upper vertical gaze, retraction nystagmus during the upward gaze attempt and dissociation of the pupillary response to light versus the accommodation reflex. It is caused by tumors in the pineal region that compress the mesencephalic pretectum.

Also: mesencephalic tectum syndrome · upper vertical gaze palsy · Parinaud's syndrome

Mesencephalic Parinaud syndrome Ophthalmoplegia of central origin clinically characterized by severe paralysis of vertical upward gaze and pupils that respond poorly to direct light stimulation due to intrinsic or compressive lesions located in the posterior mesencephalic tectum.

Also: pretectal vertical paralysis · mesencephalic tectal syndrome · pretectal syndrome

Parsonage-Turner syndrome Neuropathy characterized by severe, acute and unilateral shoulder pain, which progressively subsides after a few days, leaving flaccid weakness and marked atrophy in the scapular muscles dependent on the branches of the upper brachial plexus. It is pathophysiologically attributed to a brachial neuritis of immune-mediated origin.

Also: shoulder neuralgic amyotrophy · acute brachial neuritis · Parsonage-Turner syndrome

Tolosa-Hunt syndrome Painful ophthalmoplegia caused by a nonspecific, granulomatous and benign inflammatory process in the cavernous sinus, the sphenoid cleft or the apex of the orbit. It is characterized by severe unilateral orbital pain and paralysis of the cranial oculomotor nerves, responding dramatically to systemic corticosteroid therapy.

Also: benign granulomatous ophthalmoplegia · inflammation of the cavernous sinus · Tolosa-Hunt syndrome

Recurrent Tolosa-Hunt syndrome Painful ophthalmoplegia characterized by repeated episodes of idiopathic granulomatous inflammation in the sphenoid cleft, causing severe unilateral ocular pain and transient paralysis of the ocular motor nerves that respond spectacularly to the use of corticosteroids.

Also: recurrent granulomatous ophthalmoplegia · recurrent cavernous sinus inflammation · recurrent Tolosa-Hunt syndrome

Wallenberg syndrome Alternating brainstem syndrome caused by an infarction in the posterolateral portion of the medulla oblongata, usually secondary to occlusion of the posteroinferior cerebellar artery. It presents with ipsilateral facial hypoesthesia and contralateral body hypoesthesia, ipsilateral cerebellar ataxia, Horner syndrome and hearing loss or dysphagia due to lower cranial nerve palsies.

Also: lateral bulbar syndrome · posterolateral bulb infarction · lateral medullary syndrome

West syndrome Catastrophic epileptic encephalopathy of infancy defined by the classic triad of infantile spasms, delay or arrest of psychomotor development, and an electroencephalographic pattern of hypsarrhythmia. It generally debuts during the first year of life due to multiple structural or metabolic causes.

Also: infantile spasms syndrome · infant spasm epilepsy · West syndrome

Conus medullaris syndrome Set of neurological manifestations caused by compressive or traumatic injuries located at the terminal end of the spinal cord, at the level of the L1-L2 vertebrae. It is clinically characterized by bilateral and symmetrical saddle anesthesia, early bladder and bowel dysfunction, and mild distal muscle weakness.

Also: terminal cone injury · terminal spinal cord syndrome · conus medullaris syndrome

Rigid man syndrome Central nervous system disorder of autoimmune etiology characterized by fluctuating and progressive rigidity of the axial and proximal muscles of the extremities, accompanied by painful muscle spasms triggered by minimal sensory stimuli. It is associated with high-titer antibodies directed against the enzyme glutamic acid decarboxylase.

Also: stiff person syndrome · Moersch-Woltman autoimmune rigidity · stiff-person syndrome

Carpal tunnel syndrome Neuropathy due to compressive entrapment of the median nerve as it passes through the osteofibrous canal located on the anterior surface of the wrist. It typically manifests with nocturnal pain, paresthesias and hypoesthesia in the first three fingers and the radial half of the fourth finger of the affected hand.

Also: compression of the median nerve in the wrist · carpal entrapment · carpal tunnel syndrome · median nerve entrapment · stenosed carpal canal

Previous spinal cord syndrome Myelopathy typically caused by infarction in the territory supplied by the anterior spinal artery, which involves the anterior two-thirds of the spinal cord. It causes bilateral motor paralysis below the level of the injury and loss of thermoalgesic sensitivity, respecting the proprioceptive sensitivity of the posterior cords.

Also: anterior spinal infarction · anterior spinal artery syndrome · previous spinal cord syndrome

Syringobulbia Pathological extension or cranial migration of a syringomyelia cystic cavity from the spinal cord to the lower portion of the brain stem, affecting the bulbar nuclei of the cranial nerves. It causes dysfunction of the glossopharyngeal, vagus and hypoglossal nerves with vocal cord paralysis, dysphagia and trigeminal sensory alteration.

Also: bulbar syrinx · brainstem cavitation · syringobulbia

Syringobulbia with lower nerve involvement Progressive cavitation in the lower brain stem that causes paralysis of the ninth, tenth and twelfth cranial nerves, clinically presenting with unilateral vocal cord paralysis, severe dysphagia with risk of recurrent aspiration and atrophy with lingual fasciculations.

Also: bulbar cavitation of low pairs · syringomyelic bulbar involvement · syringobulbia with lower cranial nerve involvement

Syringobulbia with dysphagia Progressive cystic cavitation of the lower brain stem that causes paralysis of the ninth and tenth cranial nerves, clinically presenting with severe dysphagia to liquids with risk of pneumonia due to recurrent silent aspiration.

Also: bulbar cavity of lower pairs with dysphagia · pharyngeal syringobulbia · syringobulbia with dysphagia

Syringobulbia with progressive dysphagia Progressive cystic cavitation of the lower brain stem that selectively compromises the fibers of the glossopharyngeal and vagus nerves, clinically presenting with severe dysphagia to liquids with episodes of recurrent bronchial aspiration of insidious evolution.

Also: bulbar cavity of lower pairs with progressive dysphagia · progressive pharyngeal syringobulbia · progressive syringomybia with dysphagia

Syringobulbia with dysphonia Progressive cystic cavitation of the lower brain stem that selectively compromises the fibers of the vagus nerve, clinically presenting with unilateral paralysis of the vocal cords, severe permanent hoarseness and a bitonal voice.

Also: bulbar cavity of low pairs with hoarseness · laryngeal syringobulbia · syringobulbia with dysphonia

Syringomyelia Presence of a cystic cavity or syrinx filled with cerebrospinal fluid within the parenchyma of the spinal cord, which can expand progressively. It causes a suspended sensory dissociation with loss of thermoalgesic sensitivity and preservation of tactile and proprioceptive sensitivity in the corresponding dermatomes.

Also: medullary syrinx · spinal cord cavitation · syringomyelia

Cervical syringomyelia Pathological cystic cavity located within the parenchyma of the cervical spinal cord that progressively destroys the anterior gray commissure. It causes a suspended loss of thermal and pain sensitivity in the shoulders and upper extremities, characteristically respecting soft touch and vibration.

Also: cervical spinal cord cavitation · cervical syrinx · cervical syringomyelia

Progressive cervical syringomyelia Pathological cystic cavity located within the cervical spinal cord that shows a progressive increase in volume in serial magnetic resonance studies, causing weakness and progressive thermoalgesic sensory loss in the upper extremities.

Also: progressive cervical spinal cord cavitation · slowly progressive cervical syrinx · progressive cervical syringomyelia

Tourette's disorder Chronic neurodevelopmental syndrome characterized by the persistence of multiple motor tics and at least one vocal tic for more than one year, with onset before the age of eighteen. It is pathophysiologically associated with alterations in the cortico-striato-thalamo-cortical circuits and dopaminergic dysfunction.

Also: chronic motor and phonic tics · Tourette syndrome

Cerebral venous sinus thrombosis Disorder characterized by thrombotic occlusion of one or more of the venous drainage sinuses of the skull, which generates severe cerebral venous congestion, intense refractory headache, focal seizures and neurological deficits. It is classically associated with hypercoagulable states or local pericranial infections.

Also: dural venous thrombosis · dural sinus occlusion · cerebral venous sinus thrombosis

Spastic neurogenic bladder Dysfunction of the lower urinary tract characterized by hyperactivity of the detrusor muscle and lack of coordinated relaxation of the urinary sphincter, secondary to chronic spinal lesions located above the conus medullaris. It causes low-volume involuntary reflex urination with urinary retention secondary to vesicosphincteric dyssynergia.

Also: spinal automatic bladder · spastic bladder dysfunction · spastic neurogenic bladder

Faccid neurogenic bladder Bladder dysfunction characterized by the abolition of the micturition reflex and loss of contractile tone of the detrusor muscle, caused by lesions of the lower motor neuron located in the conus medullaris, cauda equina or pelvic nerves. It leads to severe urinary retention with chronic overflow incontinence.

Also: autonomous atonic bladder · lower motor neuron bladder dysfunction · flaccid neurogenic bladder

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