Sydenham’s Chorea Brain Disease

Written by on June 13, 2021

The disease, known also as sydenham’s chorea (not to be confused with huntington’s disease, a hereditary disease of adults that is sometimes called huntington’s chorea), is usually, but not always, a complication of rheumatic fever.

This condition is a very rare genetic disorder.

It’s characterised by misshapen red blood cells.

It causes neurological abnormalities and affects brain functioning.

Chorea for this condition commonly involves abnormal arm and leg movements, shoulder shrugs, and pelvic thrusts.

It can also involve rapid, purposeless movements of the face.

People with this form of chorea can also exhibit dystonia.

This is characterised by involuntary muscle contractions of the mouth and face, such as:

  • teeth grinding
  • involuntary belching
  • drooling or spitting
  • lip and tongue biting
  • difficulty with speech or communication
  • vocal tics, such as grunting, involuntary speaking, or slurred speech

Definition

Chorea is an abnormal involuntary movement disorder, one of a group of neurological disorders called dyskinesias, which are caused by overactivity of the neurotransmitter dopamine in the areas of the brain that control movement.

Chorea is characterised by brief, irregular contractions that are not repetitive or rhythmic, but appear to flow from one muscle to the next. Chorea often occurs with athetosis, which adds twisting and writhing movements. Chorea is a primary feature of Huntington’s disease, a progressive, hereditary movement disorder that appears in adults, but it may also occur in a variety of other conditions. Sydenham’s chorea occurs in a small percentage (20 percent) of children and adolescents as a complication of rheumatic fever. Chorea can also be induced by drugs (levodopa, anti-convulsants, and anti-psychotics) metabolic and endocrine disorders, and vascular incidents.

Treatment

There is no standard course of treatment for chorea. Treatment depends on the type of chorea and the associated disease. Treatment for Huntington’s disease is supportive, while treatment for Sydenham’s chorea usually involves antibiotic drugs to treat the infection, followed by drug therapy to prevent recurrence. Adjusting medication dosages can treat drug-induced chorea. Metabolic and endocrine-related choreas are treated according to the cause(s) of symptoms.

Prognosis

The prognosis for individuals with chorea varies depending on the type of chorea and the associated disease. Huntington’s disease is a progressive, and ultimately, fatal disease. Sydenham’s chorea is treatable and curable.

Patient Organisations

 

Publication

Sydenham Chorea (St. Vitus’ Dance) information sheet compiled by NINDS, the National Institute of Neurological Disorders and Stroke.

An information booklet on Huntington’s disease compiled by the National Institute of Neurological Disorders and Stroke (NINDS).

Huntington’s Disease information sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS).

https://www.ninds.nih.gov/Disorders/All-Disorders/Chorea-Information-Page

 


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