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Block 5 Week 3 Meg > Dr. Farbman: Movement Disorders > Flashcards

Dr. Farbman: Movement Disorders Flashcards

1
Q

Movement disorders impair (blank) without affecting strength or cerebellar function.

A

voluntary movement

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2
Q

What are movement disorders a result of?

A

dysfunction of the basal ganglia

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3
Q

Are movements increased or decreased in movement disorders?

A

increased (hyperkinesia) or decreased (hypokinesia)

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4
Q

What are types of hypokinesia?

A

parkinsonism

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5
Q

What are types of hyperkinesia?

A 
tremor
chorea
athetosis
dystonia
tics 
ballismus
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6
Q

What are the motor features of parkinsonism?

A

tremor
rigidity
bradykinesia
postural instability

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7
Q

What does a dysfunction of the substantia nigra affect?

A

production of dopamine

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8
Q

Causese of parkinsonism?

A

antipsychotic drugs *influence dopamine
postencephalitis
toxic agents *manganese, MPTP
Parkinson disease **most common

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9
Q

Idiopathic degeneration of neurons in substantia nigra causing loss of dopamine

A

Parkinson’s disease

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10
Q

When is the mean onset of Parkinson’s? Are males more affected or females?

A

63; 3 to 2 male to female

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11
Q

What is the prevalence of Parkinson’s?

A

160/100,000

*50,000 new cases annually

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12
Q

Cardinal symptoms of Parkinson’s disease?

A

resting tremor *asymmetric
rigidity *increased resistance to passive movement
hypokinesia *loss of automatic movements (armswing)
postural instability *difficulty rising from a chair

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13
Q

How do you diagnose Parkinson’s disease?

A

*clinical diagnosis
nuclear imagining
response to medication

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14
Q

What is the gold standard medication for Parkinson’s disease?

A

Levodopa

  • *treats the symptoms, not the underlying disease
  • *goes to brain, gets converted into dopamine (dopamine can’t cross BBB)
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15
Q

When is surgery an option in Parkinson’s?

A

only after maximal medical treatment

in dopa-responsive patients

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16
Q

What is the operation of choice for Parkinson’s disease?

A

deep brain stimulation at substantia nigra or GPi

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17
Q

What symptoms often precede Parkinson’s disease?

A

anosmia
constipation
REM sleep behavior disorder

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18
Q

What are other problems associated w Parkinson’s disease?

A

depression
dementia
orthostatic hypotension

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19
Q

Most common movement disorder
Usually in arms or neck
Different tremor than Parkinson’s

A

essential tremor

20
Q

How does essential tremor differ from Parkinsons?

A

faster
BILATERAL
action tremor
may respond to alcohol

21
Q

movements that are slow, sinuous and writhing in nature

A

athetosis

22
Q

movements are sustained so they appear to be abnormal postures

A

dystonia

23
Q

What is the dystonia treatment of choice?

A

botox for focal dystonia

DBS surgery for general dystonia

24
Q 
Very common
Affect up to 20% of children
Can “outgrow” them
Rapid, purposeless movement
Repetitive
Often suppressible, at least for a time
A

tics

25
Q

What defines tourette’s syndrome?

A

motor + vocal tics

26
Q

Violent movements
Proximal muscles are involved
May look like chorea
Often resolves spontaneously

A

Hemiballismus

27
Q

When is hemiballismus most commonly seen?

A

secondary to stroke in contralateral subthalamic nucleus

28
Q

Irregular, unpredictable jerky movement

Can spread from one part of the body to another in random sequence

A

chorea

29
Q

Causes of chorea

A

Syndenham’s *occurs in children after strep
Drug induced
Huntington’s disease *most common

30
Q

What are three “marked peculiarities” of Huntington disease?

A
  1. hereditary
  2. tendency to insanity/suicide
  3. grave disease only in adult life
31
Q

When is the mean onset of Huntington?

A

age 40

32
Q

What are the symptoms of Huntingtons?

A 
chorea
speech disturbances
falls
cognitive
psychiatric
caudate atrophy
*young onset variant w rigidity and akinesia
33
Q

Huntington Disease is a fully penetrant (blank) disorder; the gene is on chromosome 4; the trinucleotide repeat is (blank); it demonstrates (blank)

A

autosomal dominant; CAG; anticipation

34
Q

How many repeats of CAG will classify a person as normal? How many repeats will classify them as fully penetrant?

A

40

35
Q

What percentage of Huntington pts are affected by depression?

A

50%

  • 30% meet criteria for major depressive episode
  • suicide more common than in other depressed patients
36
Q

What percentage of Huntington pts experience mania?

A

10%

37
Q

Why is Huntington difficult to treat?

A

progressive, neurogenerative

38
Q

Cognitive problems with Huntington?

A

apathy
impulsivity
executive dysfunction

39
Q

Triad of hepatic, neurological, and psychiatric
Autosomal recessive gene, which, when present, causes abnormal metabolism of copper
Build-up of free copper is toxic

A

Wilson disease

40
Q

What is the gold standard test for Wilson disease?

A

24 degree urinary screen of copper - will be elevated if WD

*liver biopsy could be diagnostic

41
Q

Symptoms of Wilson disease?

A 
tremors
parkinsonism
chorea
dystonia
dysathria
*hepatic symptoms always present 
jaundice
varices
spider veins
psychiatric symptoms
42
Q

What type of genetic disease is Wilson’s disease? What is the mutated gene?

A

autosomal recessive; ATP7B

43
Q

How do you diagnose Wilson’s disease?

A

MRI - big bilateral thalamus, putamen

Midbrain shows face of the giant panda - high signal in tegmentum with normal red nucleus

44
Q

Treatment of Wilson Disease

A

removal/lowering of copper

45
Q

What do you need to diagnose a movement disorder?

A

good history
family history
good exam
**treat based on phenomenology of movements

Block 5 Week 3 Meg (10 decks)

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