Basal Ganglia and Movement Disorders Flashcards
Describe the tremor seen in Parkinson’s disease.
resting tremor of 4-5 per second in the lips, head, and fingers (pill rolling)
Where is the mutation in primary dystonia?
DYT1 TOR1A gene
What is the other name for Wilson’s Disease?
hepatolenticular degeneration
What tests are indicative of Wilson’s disease?
LFTs, slit lampe test (Kayser-Fleischer rings), decreased ceruloplasmin, elevated urine copper
What defines Tourette’s syndrome?
multiple motor tics with at least one vocal tic
Name two COMT inhibitors.
entacapone, tolcapone
Name two Monoamine oxidase B inhibitors.
selegiline, rasagiline
What is olivopontocerebellar degeneration?
multiple system atrophy associated with ataxia
What is Striatonigral degneration?
form of MSA in which Parkinsonian features
predominate although autonomic and cerebellar symptoms can be seen
What is Shy-Drager syndrome?
MSA with autonomic dysfunction predominating; Orthostatic hypotension is the key finding
What is Progressive Supranuclear Palsy?
triad of :
- progressive supranuclear ophthalmoplegia (impaired voluntary vertical gaze, but preserved doll’s eyes),
- pseudobulbar palsy
- axial rigidity
What is the inheritance of Huntington’s disease?
AD
What is the genetic abnormality seen in Huntington’s disease?
great than forty tri-nucleotide CAG repeat on Ch 4
Where is degeneration seen in Huntington’s disease?
Frontal lobe and caudate
