CHOREIC DISORDERS
DEFINITION:
A group of disorders characterized by brief, involuntary, random,
and purposeless movements of various body parts.
EPIDEMIOLOGY:
- incidence: ?
- age of onset:
- risk factors:
- see differential diagnosis
DIFFERENTIAL DIAGNOSIS:
CLINICAL FEATURES:
- brief, involuntary, random, and purposeless movements of
various body parts:
- facial muscles
- different facial expressions
- grimaces, chewing movements, tongue protrusion
- neck and trunk
- swaying and jerking of the head and body
- extremities
- restless appearance of limbs
- gait disturbances - halting or lurching
- motor impersistence
- unable to maintain any posture for more than a few
seconds ("milkmaid grasp")
- variable from mild to severe
- exacerbated by action, emotion, intense concentration,
stress
- absent during sleep
- may be associated with athetosis - choreoathetosis
2. Chorea Caused by Cardiac Surgery
- related to intraoperative impaired cerebral perfusion
affecting the putamen and globus pallidus
- develop within 4 days postoperatively
- types of chorea observed:
- facial muscles, trunk, and extremities
- tend to resolve completely but may be left with a very mild
or persistent chorea
- other associated neurologic manifestations:
- generalized hypotonia, developmental delay
3. Lupus-Associated Chorea
- chorea may be the first manifestation of SLE
- can begin anywhere from 7 yrs before to 3 yrs after the
onset of systemic manifestations
- indistinguishable from Sydenham chorea
- lasts about 12 wks with recurrence in 25% of patients
- other neurological manifestations (ataxia, seizures,
psychosis) may occur with chorea but only after onset of
systemic features
INVESTIGATIONS:
- electrolytes, glucose, calcium, phosphorus
- thyroid function tests, parathyroid hormone
- renal and liver function tests
- serum lactate, pyruvate, amino acids
- blood cultures - bacteria - group A beta-hemolytic strep
- spirochete - Borrelia burgdorferi (Lyme)
- serology - HIV, other viruses
- monospot
2. Urine
- screen for amino acids, organic acids, and oligosaccharides
3. Imaging Studies
MANAGEMENT:
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