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Movement Disorders Flashcards

1
Q

What is the most common movement disorder?

A

Parkinsons Disease

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2
Q

What percent of the population is affected by PD?

A

1-2%

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3
Q

Which gender is more commonly affected by PD?

A

Males

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4
Q

What is the second most common neurodegenerative disorder?

A

Alzheimers

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5
Q

What is the second most common neurodegenerative disorder?

A

Parkinsons Disease

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6
Q

Most common age group for the diagnosis of Parkinsons?

A

50-70

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7
Q

What genes have an association to Parkinsons?

A

LLRK2, PARK2, PARK7, PINK1 and SNCA

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8
Q

What are risk factors for Parkinsons Disease?

A
  1. Age
  2. Male
  3. Race (non-Asian)
  4. Family Hx (genes)
  5. Environmental
  6. Trauma
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9
Q

What are environmental risk factors to developing Parkinsons Disease?

A
  1. Herbicides, pesticides
  2. Metals (Mg, Fe) exposure
  3. Well Water, Farming and Rural Residence
  4. Wood Pulp Mills
  5. Steel and Alloy workers
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10
Q

Is smoking a risk or protective of PD?

A

Protective (possibly)

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11
Q

Describe the pathophysiology of PD

A
  1. Loss of dopaminergic cells within the substantial nigra

2. Accumulation of Lewy Bodies and Lewy Neurites (not specific to PD)

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12
Q

Where is the loss of dopaminergic neurons most prominent?

A

Ventral Lateral Substantia Nigra

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13
Q

What percent of dopaminergic neurons must be lost before motor signs of PD?

A

60-80%

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14
Q

What are Lewy Bodies?

A

Eosinophilic, round cytoplasmic inclusions

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15
Q

Where does the substantia nigra project?

A

Striatum

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16
Q

What is the major function of the striatum?

A

Regulation of posture and muscle tone

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17
Q

What are the motor features of PD?

A

Tremor
Rigidity
Akinesia (bradykinesia)
Postural instability

+ stooped posture, shuffling gait, enbloc turning and poor arm swing

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18
Q

What are the features of the tremor associated with PD?

A
  1. Asymmetric onset
  2. Rill rolling or supination/pronation
  3. Affects all limbs (late)
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19
Q

What are the non-motor features of PD?

A
  1. Mood
  2. Memory
  3. Autonomics
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20
Q

What are the mood symptoms of PD?

A

Depression, Anxeity and Hallucinations (late stage, well formed visual hallucinations)

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21
Q

What are the autonomic symptoms of PD?

A
  1. Constipation
  2. Sialhorhea
  3. Urinary frequency
  4. Sweating
  5. Orthostatic Hypotension
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22
Q

What disease may be associated with REM sleep disorder?

A

PD

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23
Q

What are the medications used to treat PD?

A
  1. Sinemet (Levadopa/Carbidopa)
  2. Dopamine Agonists (Ropiranole)
  3. MAOB Inhibitors (rasaGELINE ad seleGILINE)
  4. COMT Inhibitors (entaCapone)
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24
Q

Which Parkinsons medications have been shown to slow the disease progression?

A

MAO-B Inhibitors (rasaGILINE and seleGILINE)

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25
Which PD drug inhibits dopa-decarboxylase?
Carbidopa
26
What is the function of dopa-decarboxylase?
Converts L-Dopa to Dopamine
27
Can Carbidopa cross the BBB?
No
28
Where does Entacapone work?
In the periphery to prevent the conversion of L-Dopa to 3OM-dopa
29
Which Parkinsons drug smoothes the on/off phenomena of the disease?
Entacapone
30
Where do rasagiline and seligiline work?
In the brain the prevent the breakdown of Dopamine
31
Where is a DBS placed in PD to treat dyskinesia and tremor?
Subthalamic Nucleus
32
Where is a DBS placed in PD to treat dystonia and rigidity?
Globus Pallidus Externus
33
Where is a DBS placed in PD to treat just tremor?
VIM
34
What can be used to treat the postural instability of PD?
PT and Occupational Therapy
35
What can be used to treat the constipation of PD?
Miralax and herbal remedies
36
What can be used to treat the orthostatic hypotension seen in PD?
1. Fluids | 2. Increased salt intake
37
What can be used for the depression in PD?
SSRIs
38
What can be used to treat the REM sleep disturbances in PD?
Melatonin or Clonazepam
39
What can be used to treat the dystonia and pain in PD?
Botulinum toxin
40
What is used for the dementia in PD?
Donepezil, Memantine and Rivastigmine
41
What is the triad seen in Normal Pressure Hydrocephalus?
1. Abnormal Gait 2. Urinary Incontinence 3. Dementia
42
Which feature of NPH is earliest to present?
Abnormal Gait
43
Which feature of NPH is most responsive to treatment?
Abnormal Gait
44
Describe the gait in NPH
Bradykinetic, broad-based, magnetic and shuffling gait
45
How is NPH diagnosed?
CT or MRI; LP with the goal of removing large volumes of CSF
46
What will be seen on neuroimaging of NPH?
Dilation of the ventricles out of proportion to sulcal atrophy ; will see transependymal flow
47
What are the treatments for NPH?
1. Ventriculoperitoneal or Lumboperitoneal CSF shunting
48
What are the symptoms of Lewy Body Dementia?
1. Visual Hallucinations 2. Fluctuations in Cognitive Function 3. Parkinsonian motor features preceding cognitive features by less than 1 year 4. Anterograde memory loss 5. Prominent executive function
49
Describe the cognitive changes associated with Lewy Body Dementia
1. Fluctuations in alertness/attention- Daytime drowsiness 2. Staring into space 3. Disorganized speech
50
When is the peak age of incidence for PSP?
6th decade
51
What are the clinical manifestations of PSP?
1. Supranuclear Ophthalmoplegia (downward gaze first) 2. Pseudobulbar Palsy 3. Prominent Neck Dystonia 4. Parkinsonism 5. Behavioral and Cognitive disturbances 6. Frequent backwards falls
52
Describe the parkinsonism in PSP
More bradykinesia and less tremor or rigidity symptoms compared to PD
53
What is the genetics of Huntington's Disease?
Autosomal Dominant Chromosome 4
54
What trinucleotide repeat is associated with Huntington's Disease?
CAG
55
What genetic feature is associated with HD?
Genetic Anticipation
56
Describe the Westphall Variant of Huntington's Disease?
A variant of HD seen in younger children that has symptoms more similar to PD
57
What are the early symptoms of HD?
1. Chorea 2. Dystonia 3. Motor Impersistance 4. Gait Instability
58
What is the age of onset for HD?
40
59
What are the later cognate and psychiatric symptoms of HD?
1. Depression 2. Anxiety 3. Apathy 4. Impulsivity 5. Dementia
60
How long do patients survive after being diagnosed with HD?
10-15 years
61
What region of the brain is most affected by HD?
Caudate Nucleus with "box car appearance"
62
What is the microscopic finding of HD?
Loss of the spiny striatal neurons with gliosis
63
What are the medical treatments for HD?
1. Tetrabenazine (depleted dopamine) 2. SSRIs 3. Antipsychotics
64
What are the side effects of Tetrabenazine?
Depression and orthostatic hypotension
65
What is the most common cause of postural or action tremor?
Essential Tremor
66
What percent of the population has an essential tremor?
5%
67
Is Essential tremor symmetric?
No, but usually bilaterally
68
When is essential tremor most severe?
When arms are outstretched during goal-directed movements
69
How is Essential Tremor affected by Alcohol?
It improves
70
What are the 3 components of Essential Tremor?
1. Bilaterally action tremor 2. No other neuro signs (except cog wheeling) 3. May have head tremor with no signs of dystonia
71
What are the secondary criteria for Essential Tremor?
1. >3 years 2. Positive Family History 3. Benefit from alcohol
72
What is the age of onset of Essential Tremor?
Bimodal (2nd Decade and 6th Decade)
73
Which has a higher correlation with family history, PD or ET?
Essential Tremor
74
Which type of tremor is usually asymmetric?
Parkinsons Disease
75
What are the frequencies of PD and ET?
PD- 4-6Hz | ET- 4-10Hz
76
What is the type of tremor in PD or ET?
PD- supination + pronation | ET- flexion + extension
77
What are associated features of ET?
Deafness, dystonia and parkinsonism
78
What is the treatment for ET?
1. Propranolol 2. Topiramate 3. Primidone (Barbituate)
79
What is the treatment for ET when medical therapy fails?
DBP with electrodes to the VIM of the Thalamus

NEURO CLERKSHIP (10 decks)

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