Chorea and Choreoathetosis (Pediatric) Etiology
Static (fixed) injury:
Cerebral palsy (often with delayed onset or with symptoms only on awakening); encephalitis/post-encephalitis; trauma; tumors; kernicterus (jaundice or high bilirubin at birth)
Hereditary/Degenerative:
Ataxia-telangiectasia (AT); basal ganglia calcification (Fahr's disease); pantothenate kinase 2 deficiency ("Hallervorden-Spatz disease"); Huntington's disease (but the dystonic-parkinsonian Westphal variant is more common in children); Rett syndrome; neuroacanthocytosis; HARP syndrome (hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration); benign hereditary chorea
Chemical/Metabolic:
Acyl-CoA dehydrogenase deficiency; mitochondrial disorders (e.g., Leigh's syndrome, etc.); Wilson's disease; GM1 gangliosidosis; metachromatic leukodystrophy; Lesch-Nyhan disease; Niemann-Pick disease type C; methylmalonic aciduria; nonketotic hyperglycinemia; Pelizaeus-Merzbacher disease; vitamin E deficiency or malabsorption (Bassen-Kornzweig disease); hypoparathyroidism; hyperthyroidism; propionic acidemia; hypernatremia; hypomagnesemia; hypocalcemia; hypoglycemia; hyperglycemia; post-cardiac bypass
Immune-mediated:
Lupus erythematosis; Henoch-Schonlein purpura; anticardiolipin or antiphospholipid antibody syndrome; Sydenham's chorea (associated with previous [one to six months] streptococcal infection; chorea gravidarum (during or immediately after pregnancy, particularly if there is a prior history of Sydenham's chorea)
Vascular:
Basal ganglia stroke; cerebral vasculitis; Moya-Moya disease; complex migraine; alternating hemiplegia
Malformations:
Holoprosencephaly; Joubert syndrome; agenesis of the corpus callosum
Drug-induced:
Neuroleptic medications including anti-emetics (e.g., haloperidol, thorazine, clomipramine, pimozide, pemoline, prochlorperazine, metoclopramide, etc.); calcium channel blockers (e.g., flunarizine, cinarizine, etc.); amphetamines (e.g., Ritalin™, Dexedrine™, etc.); anti-seizure medications (e.g., phenytoin, carbamazepine, valproate, phenobarbital, etc.); anti-cholinergic medications (e.g., trihexyphenydil, benztropine, Benadryl™, etc.); anti-histamines; tricyclic antidepressants; benzodiazepines; stimulants (e.g., bronchodilators); clonidine; L-dopa; amantadine; cocaine; bismuth; lithium; manganese toxicity; ethanol; carbon monoxide; oral contraceptives; general anesthesia
Acute/Paroxysmal:
PKC: paroxysmal kinesogenic choreoathetosis (triggered by movement and responds to treatment with carbamazepine); PNKC: paroxysmal nonkinesiogenic choreoathetosis (also known as paroxysmal dystonic choreoathetosis [PDC or Mount & Reback disease] often worsened by stress, caffeine, or alcohol, improved by clonazepam or acetazolamide, localized to a gene near 2q33-q35, also called DYT8); PEC: paroxysmal exercise-induced choreoathetosis (occurs with fatigue, rather than at the onset of movement)
Disorders that mimic chorea:
Spasmus nutans, tics, dystonia, shaking/shuddering spells, proprioceptive loss (e.g., spinal cord injury, peripheral neuropathy, etc.), self-stimulation, psychogenic disorders, anxiety, normal development (less than 1 year of age)
