Sydenham Chorea

Aka: Sydenham Chorea, Sydenham's Chorea, Rheumatic Chorea, Saint Vitus Dance

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II. Pathophysiology

  1. Childhood Movement Disorder
  2. Occurs within 8 months of Streptococcal infection
    1. Specifically Group A Beta Hemolytic Streptococcus
  3. Late manifestation of Rheumatic Fever

III. Epidemiology

  1. Incidence: <5% of Rheumatic Fever cases
  2. Ages: 5 to 15 years
  3. Girls affected more often than boys

IV. Symptoms: Gradual onset of neurological symptoms

  1. Atypical behavior
    1. Irritability and crying
    2. Anxiety and restlessness
    3. Transient acute Psychosis
  2. Poor memory retention
  3. Motor weakness
  4. Abrupt onset of choreoform movements (purposeless, nonrhythmic, involuntary) only present while awake
    1. Irregular and aimless
    2. Affects extremities, face and trunk

V. Signs

  1. Choreiform movements
    1. Abrupt, purposeless, nonrhythmic involuntary movement
  2. Hyperextended joints
  3. Motor weakness
  4. Inaccurate voluntary motions
  5. Clumsiness
  6. Hypotonia
  7. Diminished Deep Tendon Reflexes
  8. Speech Impairment (Dysarthria)
  9. Asymmetric (one side usually more affected than other)

VI. Labs: Cerebrospinal fluid

  1. Intracerebral pressure (opening pressure) increased
  2. CSF Glucose increased
  3. CSF Leukocyte count increased
  4. Late manifestation results in negative markers
    1. ASO Titer negative
    2. Acute phase reactants negative

VII. Prognosis

  1. Self limited
  2. Expect improvement within 3 to 6 weeks (complete resolution by 2-3 years)

VIII. Management

  1. Benzodiazepines have been used in the past

IX. Reference

  1. Swedo (1993) Pediatrics 91(4): 706-13 [PubMed]

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Related Studies

Ontology: Rheumatic Chorea (C0152113)

Definition (CSP) acute, usually self-limited disorder of early life, usually between ages 5 and 15, or during pregnancy, and closely linked with rheumatic fever; characterized by involuntary movements that gradually become severe, affecting all motor activities including gait, arm movements, and speech; called also Sydenham's, acute, juvenile, or simple chorea, or St. Vitus dance.
Concepts Disease or Syndrome (T047)
MSH D002819 , D020822
ICD9 392
ICD10 I02 , I02.9
SnomedCT 194716004, 155271001, 46826000
English SYDENHAMS CHOREA, chorea minor, St. Vitus's Dances, Sydenham Chorea, Chorea, Rheumatic, Choreas, Rheumatic, Choreas, Sydenham, Sydenham Choreas, St. Vitus's Dance, St. Vitus Dance, St. Vituss Dance, Rheumatic Choreas, Chorea, Sydenham, acute chorea, Rheumatic Chorea, Sydenham's chorea (diagnosis), Sydenhams Chorea, Chorea, Sydenham's, Sydenham's Chorea, Rheumatic chorea NOS, saint vitus dance, st. vitus' dance, Chorea;rheumatic, Chorea;Sydenhams, sydenhams chorea, vitus dance saint, dance st vitus, dance st vitus's, chorea sydenhams, sydenham chorea, sydenham's chorea, st. vitus's dance, Rheumatic chorea NOS (disorder), Rheumatic chorea, Sydenham's chorea, Rheumatic chorea (disorder), chorea; Sydenham, chorea; minor, chorea; rheumatic, Sydenham; chorea, minor; chorea, rheumatic; chorea, rheumatic chorea, Sydenhams chorea
Dutch reumatische chorea, Sydenham; chorea, chorea; Sydenham, chorea; minor, chorea; reumatisch, minor; chorea, reumatisch; chorea, Reumatische chorea, Chorea, Sydenham-, Chorea, reumatische, Sydenham-chorea
German rheumatische Chorea, Chorea, rheumatische, Chorea rheumatica, Sydenham-Chorea, Chorea Sydenham, Rheumatische Chorea, Sydenham Chorea
Portuguese Coreia reumática, Coreia de Sydenham
Spanish Corea reumático, corea reumática, SAI (trastorno), corea reumática, SAI, corea de Sydenham, corea reumática (trastorno), corea reumática, Corea de Sydenham
Japanese リウマチ性舞踏病, シデナム舞踏病, ｼﾃﾞﾅﾑﾌﾞﾄｳﾋﾞｮｳ, ﾘｳﾏﾁｾｲﾌﾞﾄｳﾋﾞｮｳ
Italian Corea di Sydenham, Ballo di San Vito, Corea reumatica
Czech chorea Sydenhamova, Revmatická chorea, revmatická chorea, Sydenhamova chorea, chorea revmatická, tanec svatého Víta, tanec sv. Víta, chorea rheumatica, chorea st. Viti, chorea minor
Korean 류마티스 무도병
Hungarian Rheumás chorea, Sydenham-chorea
Norwegian Chorea rheumatica, Sydenhams chorea, Sanktveitsdans, Sydenhams korea, St. Veits’ dans
French Danse de Saint-Guy, Chorée de Sydenham, Chorée rhumatismale, Danse de Saint-Vitus
Derived from the NIH UMLS (Unified Medical Language System)

Related Topics in Motor Disorders

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