Clinical Approach to Movement Disorder Flashcards
What is the most common type of bradykinetic disorder (akinetic rigid syndrome)?
Parkinsonism
What is the pathogenesis of PD?
What are the 3 major clinical features?
Depletion of DA in the nigrostriatal system, disrupting the balance of DA and ACh.
Tremor (pill-rolling)
Rigidity
Bradykinesia
What are the main features of Progressive Supranuclear Palsy?
Bradykinesia and rigidity.
Loss of voluntary control of the eyes (especially vertical gaze).
What are features of MSA (Shy-Drager syndrome)?
Bradykinesia and rigidity.
Pronounced ANS dysfunction.
What are features of Cortical Basal (Ganglionic) Degeneration (CBD)?
Bradykinesia and rigidity.
May also see signs of cortical loss, apraxia, myoclonus or aphasia.
Are anti-parkinsonian medicines helpful in treatment of other akinetic rigid syndromes (PSP, MSA, CBD)?
Generally, no, but no other useful therapy exists.
What is the presentation of HD?
When do symptoms onset?
What chromosome is the CAG repeat found?
Gradual onset of chorea and dementia.
Between ages 30-50, with average lifespan being about 15 years after onset.
Chr. 4
What triggers the onset of Sydenham’s Chorea?
What ages are most common?
What is the major symptom?
What is the treatment?
Prior group A hemolytic strep. infection.
Children and adolescents.
Unilateral choreiform movements which, when mild, can be confused for restlessness or fidgeting. May also see behavioral changes.
Bed-rest and antibiotics.
What is the presentation of Idiopathic Torsion Dystonia?
What is the inheritance?
What is the onset and progression?
Dystonic movements and postures without other signs.
- torticollis - neck twisted in one direction
- blepharospasm - involuntary/forced closure of eyelids
- oromandibular dystonia - spasms of muscles aroud mouth
- arms and legs help in extension and pronation
- multiple areas!
Can be AD, AR or XLR.
Onset can be at any time, but it remains throughout life.
What is the presentation of Focal Torsion Dystonia?
What is an example of this?
What is the treatment?
Dystonia confined to a focal area. Same symptoms as ITD.
Writer’s cramp
Botox to weaken the associated muscles
When does Wilson’s disease onset?
What are the neurological clinical features?
What is the treatment?
What are the 2 major organs involved?
Prognosis?
Childhood or young adult life.
Both brady- and hyperkinetic features.
Penicillamine (Cu chelating agent)
Cu restriction in diet
Brain and liver
History/exam
Increased Cu in urine
Low ceruloplasmin
K-F rings
Fatal if untreated, but there are good therapies
What is required for a diagnosis of Gilles De La Tourette’s syndrome?
What cases are more common: familial or sporadic?
Multiple chronic tics (motor + vocal) with onset before age of 21.
Coprolalia - obscene/vulgar speech
Echolalia - parroting other’s speech
Echopraxia - imitating others’ movements
Palillalia - repetition of words/phrases
What can be used as treatment for Tourette’s syndrome?
Clonidine
Haldol
Phenothiazides
What usually will improve an essential tremor?
What can be used as treatment?
EtOH
Beta blockers Primidone Benzos Topiramate Deep brain stimulation (DBS)
What are the bradykinetic and ridigity disorders?
PD - tremor LBD - psychosis PSP - no voluntary vertical eye movement MSA - ANS dysfunction CBD - cortical sensory loss, apraxia, myoclonus, aphasia