Movement Disorders

Question 0

Opsoclonus-Myoclonus

Answer 0

Paraneoplastic syndrome
Sometimes associated w/neuroblastoma
Opsoclonus: disorganized chaotic eye movements

Question 1

Acute Cerebellar Ataxia

Answer 1

usually post- infectious (viral, especially VZV) or post-vaccine
self-limited, resolves in weeks to months
low recurrence, unclear if steroids help

Question 2

Sydenham chorea

Answer 2

Complication of rheumatic fever
“Milk-maid’s grip” and “darting tongue”
can’t maintain motor command d/t intrusions/relaxations
Tx: haldol (block dopamine)
usu self-limited
other options: fluphenazine, risperdal, tetrabenazine
* PCN for secondary prevention
* trihexyphenidyl can worsen S chorea (helps dystonia)

Question 3

Spinocerebellar Ataxia

Answer 3

Autosomal Dominant
Multiple overlapping forms
Progressive neuro sx: hypotonia&raquo_space; spasticity, ataxia, nystagmus
Wide variety of neuro sx: retinopathy, cognitive impairment, epilepsy, (extra)pyramidal signs

Question 4

Friedreich Ataxia

Answer 4

Autosomal recessive
GAA repeat expansion in Frataxin gene
Sx develop 5-25yo
Ataxia, dysarthria, loss of proprioception/vibration sense, areflexia
Systemic sx: HOCM, DM, scoliosis

Question 5

Ataxia-Telangiectasia

Answer 5

Autosomal recessive
Defect in DNA repair
Sx develop 1-4yo
Choreathetosis, ataxia, dysarthria, areflexia
Oculocutaneous (develops after neuro sx)
Sinopulmonary infections, malignancy (leukemia/lymphoma), DM