Table 1e.

Characteristics X-linked hereditary choreas.

Disease; Gene	Region; Age	Mov Disord	Key Findings	Other Sx	NPsych	CI/Dem	I and L
McLeod syndrome; XK	Before 40 yrs.	Chorea, dystonia, parkinsonism	Areflexia Sensorimotor axonopathy	Cardiomyopathy Arrhythmias MyopathySeizures	Yes	No	L: Elevated serum liver enzymes and creatine kinase I: caudate nucleus and putamen atrophy
Pelizaeus-Merzbacher disease; PLP1	Neonatal to 5 yrs.	Choreoathetosis Dystonia	Pendular nystagmus Spasticity Head tremor Generalized hypotonia	Ataxia Mental retardation	No	Yes	I: Hypomyelination of corona radiata, optical radiations, internal capsule

Mov Dis: Movement Disorders; Sx: Symptoms; NPsych: Neuropsychiatric features; CI/Dem: Cognitive impairment or dementia; I: Imaging; L: Laboratory