II. Pathophysiology
- Childhood Movement Disorder
- Occurs within 8 months of Streptococcal infection
- Specifically Group A Beta Hemolytic Streptococcus
- Late manifestation of Rheumatic Fever
III. Epidemiology
- Incidence: <5% of Rheumatic Fever cases
- Ages: 5 to 15 years
- Girls affected more often than boys
IV. Symptoms: Gradual onset of neurological symptoms
- Atypical behavior
- Irritability and crying
- Anxiety and restlessness
- Transient acute Psychosis
- Poor memory retention
- Motor weakness
- Abrupt onset of choreoform movements (purposeless, nonrhythmic, involuntary) only present while awake
- Irregular and aimless
- Affects extremities, face and trunk
V. Signs
-
Choreiform movements
- Abrupt, purposeless, nonrhythmic involuntary movement
- Hyperextended joints
- Motor weakness
- Inaccurate voluntary motions
- Clumsiness
- Hypotonia
- Diminished Deep Tendon Reflexes
- Speech Impairment (Dysarthria)
- Asymmetric (one side usually more affected than other)
VI. Labs: Cerebrospinal fluid
- Intracerebral pressure (opening pressure) increased
- CSF Glucose increased
- CSF Leukocyte count increased
- Late manifestation results in negative markers
- ASO Titer negative
- Acute phase reactants negative
VII. Prognosis
- Self limited
- Expect improvement within 3 to 6 weeks (complete resolution by 2-3 years)
VIII. Management
- Benzodiazepines have been used in the past