EpistemisKorea
Specialty: Neurology.
Why it occurs
- Huntington's disease (autosomal dominant neurodegenerative disorder)
- Sydenham chorea (late manifestation of rheumatic fever due to group A streptococcus infection)
- Korea gravidarum (associated with pregnancy in women with a history or vulnerability)
- Systemic lupus erythematosus and antiphospholipid syndrome (autoimmune cause due to vasculitis or direct antibodies against basal ganglia)
- Pharmacological adverse effect (levodopa-induced dyskinesia, oral contraceptives, neuroleptics)
Initial workup
Genetic study for the expansion of CAG triplets in the HTT gene (Huntington confirmation); throat swab, antistreptolysin O (ASLO) titers and immunoglobulins; autoantibody profile (ANA, anti-DNA, antiphospholipid antibodies); capillary and plasma glycemia; MRI of the skull with emphasis on basal nuclei (characteristic caudate atrophy in Huntington).
red flags
Unilateral acute onset chorea (hemibalism-hemichorea) associated with cardiovascular risk factors or uncontrolled diabetes (suggests ischemic/hemorrhagic stroke in the subthalamic nucleus or non-ketotic hyperglycemic hemichorea-hemibalism syndrome), or chorea accompanied by severe dysphagia leading to repeated aspiration.
Standard management
- Tetrabenazine — 12.5-100 mg/day, presynaptic dopamine depletor, monitoring the appearance of depression or parkinsonism
- Deutetrabenazine or Valbenazine
- Haloperidol in low doses — 0.5-2 mg/day, dopaminergic antagonist to suppress hyperkinetic movements
- Corticosteroids (in autoimmune or severe Sydenham's chorea).
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