Chorea

Question 1

What laboratory testings are done for choreatic conditions?

Answer 1

• huntington: genetic testing
• wilson: increased copper excretion
• syndenham: documentation of previous streptococcal infections
• neuroacanthocytosis: acanthocytes, increased creatinkinase levels

Question 2

What does the MRI show in huntington disease?

Answer 2

• decreased signals in neostriatum (increased iron deposition), caudate nucleus and putamen
• atrophy of neostriatum
• enlargement of frontal horns

Question 3

Treatment of chorea

Answer 3

only symptomatic treatment:

• neuroleptics: (block DA receptors)
  typical: haloperidol, fluphenazine
  atypical: risperidone, olanzapine, clozapine, quetiapine
• DA depleting agents: reserpine, tetrabenazine
• GABAnergic agents: clonazepam, gabapentin, valporate
• Co-Enzyme Q10, minocycline
• IV Ig, plasmapheresis
• SSRI and tricyclic antidepressants, antipsychotics
• deep brain stimulation and cell Transplantationen

Question 4

Clinical features of syndenham chorea

Answer 4

= rheumatic chorea
most common cause of aquired chorea in young people

major sign: acute rheumatic fever (might be sufficient for the diagnosis of rheumatic fever)
milkmaids grip, pronator sign, psychiatric symptoms

main feature: involuntary movements, hypotonia, muscle weakness

can be general or unilateral

signs show at rest, get worse if stressed, absent while sleeping
resolves after 3 - 6 month

Question 5

Treatment of syndenham chorea

Answer 5

infectious: penicillin, cephalosporin
cardiologic: prednisone, IV-Ig, plasma exchange
neurologic: anticonvulsants (valporic acid, carbamezapine), DA-blockers, neuroleptics

Question 6

Clinical features of huntington chorea

Answer 6

• movement disorders, cognitive disorders, behavioral disorders
• spasticity, clonus, extensor plantar responses
• dysarthria, dysphagia
• tics, myoclonus
• cognitive decline, dementia
• in illness progression: slowly replaced with dystonia and parkinsonain feat.
• in advanced stage: akinetic-rigid syndrome

Question 7

what is the clincial features in wilsons disease?

Answer 7

• hepatic dysfunction (fulminant hep. failure, cirrhosis, chr. active hepatitis)
• neurospychiatric signs (most common: asym. tremor)
• ophtalmolog. symptoms (kayser fleischer rings)

Question 8

diagnosis criteria for wilsons disease

Answer 8

kayer fleischer rings

ceruloplasmin

Question 9

wilsons disease treatment

Answer 9

• chelating agents: penicillamine, trientine, zinc
• anticholingerics, GABA antagonist, levodopa
• antiepileptics
• neuroleptics
• protein restriction
• avoid diet high in copper
  liver transplant