EpistemisCervical dystonia
Specialty: Neurology.
Why it occurs
- Primary idiopathic focal dystonia (most common cause, of idiopathic origin with functional alteration in the basal ganglia)
- Secondary effect to neuroleptics or other dopamine receptor blockers
- Previous cervical or head trauma (post-traumatic dystonia)
- Chiari malformation type I
- Hepatolenticular degeneration (Wilson's disease)
Initial workup
Clinical evaluation using the TWSTRS scale (Toronto Western Spasmodic Torticollis Rating Scale) to measure severity and direction of rotation (torticollis, laterocollis, anterocollis, retrocollis); brain and cervical spine MRI to rule out structural abnormalities of the craniocervical junction; Serum ceruloplasmin in people under 40 years of age.
red flags
Cervical dystonia of sudden onset after administration of an antiemetic or neuroleptic drug (acute dystonic reaction with risk of concomitant respiratory compromise due to pharyngeal spasm), or dystonia associated with progressive myelopathy and first motor neuron signs.
Standard management
- Injection of botulinum toxin type A or B into the corresponding hyperactive cervical muscles — p. e.g., contralateral sternocleidomastoid, ipsilateral splenius capitis) every 12 weeks (first-line treatment with high level of evidence
- Trihexyphenidyl — 2-15 mg/day
- Clonazepam — 0.5-2 mg/day
- Baclofen (10-40 mg/day).
Educational guidance for study. It is NOT a prescription recommendation. The actual choice depends on the cause, the patient, and current guidelines.
- Area
- Neurology
- Listed causes
- 5
- Treatment options
- 4