Chorea and Ataxia Flashcards
Define chorea.
Chorea is a movement disorder characterized by the rapid, jerky motion of proximal muscle groups of the arms, legs, and face. The movements are present at rest, and become much more exaggerated with emotion or with intentionally trying to control the movements. The movements disappear with sleep.
What is the most common cause of chorea in children?
Sydenham chorea, which is a clinical manifestation of rheumatic fever.
List three potential causes of chorea in a child.
Sydenham chorea (rheumatic fever), SLE, and drugs/toxins.
What lab tests are useful in determining if chorea is due to a recent streptococcal infection?
Anti-DNAse B and ASO titers
What lab tests are useful in determining if chorea is due to an underlying autoimmune disorder?
ANA and antiphospholipid antibodies
How is chorea treated?
Secondary chorea usually resolves with treatment of the underlying cause, whereas there is no effective treatment for primary chorea. Regardless of the cause, dopamine receptor blockers can control the severity of the choreiform movements.
What is the inheritance pattern for Friedreich Ataxia?
It is an autosomal recessive disorder that maps to chromosome 9.
What are the (6) manifestations of Friedreich Ataxia?
Hpoactive or absent deep tendon reflexes, ataxia, corticospinal tract dysfunction (bilateral Babinski sign), impaired vibratory and proprioceptive function, hypertrophic cardiomyopathy, and diabetes mellitus.
What is the typical progression of disease in Friedreich Ataxia?
Progression is variable, but most patients require a wheelchair within 10-20 years of symptom onset, and death usually occurs between 30 and 40 years of age. Death is typically caused by the cardiomyopathy.
What is acute cerebellar ataxia of childhood?
It is the second most common cause of childhood ataxia and occurs most commonly in children 2-6 years of age. Its onset is abrupt, and about 50% of those affected have a history of prior URI or viral GI illness.
What is the most common cause of ataxia in childhood?
Drug ingestion
What physical exam, laboratory, and imaging findings would be expected in a child with acute cerebellar ataxia?
The child is irritable and has nausea/vomiting. They have ataxia but sensory exam is normal, as are the deep tendon reflexes. CSF is normal, except for occasional increased WBC to 30 lymphocytes/µL. CT and MRI of the head are normal.
List four groups of drugs that can cause ataxia.
Anticonvulsants (phenytoin and carbamazepine), benzodiazepines, antihypertensives, and antihistamines.
What is the recommended management for acute cerebellar ataxia of childhood?
Supportive care. Around 90% of patients recover in 6-8 weeks without specific therapy. Steroids are not indicated.
