Clinical Approach to Movement Disorder Flashcards

1
Q

What is the most common type of bradykinetic disorder (akinetic rigid syndrome)?

A

Parkinsonism

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2
Q

What is the pathogenesis of PD?

What are the 3 major clinical features?

A

Depletion of DA in the nigrostriatal system, disrupting the balance of DA and ACh.

Tremor (pill-rolling)
Rigidity
Bradykinesia

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3
Q

What are the main features of Progressive Supranuclear Palsy?

A

Bradykinesia and rigidity.

Loss of voluntary control of the eyes (especially vertical gaze).

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4
Q

What are features of MSA (Shy-Drager syndrome)?

A

Bradykinesia and rigidity.

Pronounced ANS dysfunction.

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5
Q

What are features of Cortical Basal (Ganglionic) Degeneration (CBD)?

A

Bradykinesia and rigidity.

May also see signs of cortical loss, apraxia, myoclonus or aphasia.

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6
Q

Are anti-parkinsonian medicines helpful in treatment of other akinetic rigid syndromes (PSP, MSA, CBD)?

A

Generally, no, but no other useful therapy exists.

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7
Q

What is the presentation of HD?

When do symptoms onset?

What chromosome is the CAG repeat found?

A

Gradual onset of chorea and dementia.

Between ages 30-50, with average lifespan being about 15 years after onset.

Chr. 4

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8
Q

What triggers the onset of Sydenham’s Chorea?

What ages are most common?

What is the major symptom?

What is the treatment?

A

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.

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9
Q

What is the presentation of Idiopathic Torsion Dystonia?

What is the inheritance?

What is the onset and progression?

A

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.

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10
Q

What is the presentation of Focal Torsion Dystonia?

What is an example of this?

What is the treatment?

A

Dystonia confined to a focal area. Same symptoms as ITD.

Writer’s cramp

Botox to weaken the associated muscles

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11
Q

When does Wilson’s disease onset?

What are the neurological clinical features?

What is the treatment?

What are the 2 major organs involved?

Prognosis?

A

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

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12
Q

What is required for a diagnosis of Gilles De La Tourette’s syndrome?

What cases are more common: familial or sporadic?

A

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

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13
Q

What can be used as treatment for Tourette’s syndrome?

A

Clonidine
Haldol
Phenothiazides

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14
Q

What usually will improve an essential tremor?

What can be used as treatment?

A

EtOH

Beta blockers
Primidone
Benzos
Topiramate
Deep brain stimulation (DBS)
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15
Q

What are the bradykinetic and ridigity disorders?

A
PD - tremor
LBD - psychosis
PSP - no voluntary vertical eye movement
MSA - ANS dysfunction
CBD - cortical sensory loss, apraxia, myoclonus, aphasia
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16
Q

What is the exception to the rule that diseases are only bradykinetic or hyperkinetic?

A

Wilson disease