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Block 5 Week 3 Dani > Parkinsons and Huntingtons - Farbman > Flashcards

Parkinsons and Huntingtons - Farbman Flashcards

1
Q

Will movement disorders of the basal ganglia result in involuntary movements directly caused by losses in strength or cerebellar function?

A

No

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2
Q

What is the one class of movement disorders that is bradykinetic?

A

Parkinsonism

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3
Q

Dysfunction of the substantia nigra affects production of what neurotransmitter?

A

dopamine

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4
Q

Besides Parkinson disease itself, what are three other causes of Parkinsonism?

A
  1. antipsychotic drugs–deplete dopamine availability
  2. Postencephalitis–damage to nigrostriatal system
  3. toxic agents–manganese, MPTP
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5
Q

Describe the following characteristics of Parkinson’s Disease:

  1. avg. age of onset
  2. Male:female ratio
  3. prevalence
  4. abnormal labs
A
  1. 63
  2. 3:2 male to female
  3. 160/100,000
  4. all labs normal
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6
Q

What are the four cardinal symptoms of PD?

A
  1. Resting tremor
  2. Rigidity
  3. Bradykinesia
  4. Postural instability
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7
Q

is the resting tremor in PD symmetric or asymmetric?

A

asymmetric

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8
Q

What are some of the specific traits of PD bradykinesia?

A

loss of automatic movements (arm wing with walk), masked facies, festinating gait, freezing

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9
Q

What clinically available imaging exam can diagnose PD? What research exam is available

A

SPECT, cuz you suSPECT someone as PD

PET for research cuz you use your PET rats to study!

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10
Q

What is the mechanism of Levodopa action?

A

goes to the brain and is converted to dopamine

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11
Q

What is the mechanism of MAOb inhibitors?

A

prevents MAO from working, stops dopamine breakdown

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12
Q

What is the mechanism of COMT inhibitors?

A

prevents COMT from working, stops dopamine breakdown

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13
Q

What is the mechanism of anti-cholinergic drugs?

A

restores dopamine:choline balance in the striatum by blocking acetylcholine

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14
Q

when treating PD, are we treating the disease or symptoms?

A

the symptoms

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15
Q

When is surgical deep brain stimulation an option for pt’s with PD?

A

Only after “maximal medical treatment”

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16
Q

What is the relation between DBS and dopamine sensitivity?

A

Pt must be dopamine sensitive for DBS to work

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17
Q

Will DBS work for postural instability?

A

No

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18
Q

What basal ganglia do they use in DBS?

A

subthalamic nucleus or GPi

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19
Q

What symptoms precede PD?

A

anosmia
constipation
REM sleep behavior disorder

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20
Q

What issues with blood pressure do Pt’s with PD suffer?

A

orthostatic hypotension

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21
Q

What two psychological symptoms do pts with PD suffer?

A

depression and dementia

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22
Q

In the Braak Hypothesis, at what Braak stage do PD patients become symptomatic?

A

Stage 3

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23
Q

What nuclei/centers are involved at stage 3? (3)

A

Substantia nigra
Locus ceruleus
Dorsal IX / X nucleus and olfactory bulb

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24
Q

What is the most common type of movement disorder?

A

Essential tremor

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25
Where does an essential tremor usually present?
Neck or arms
26
how is an essential tremor different than a parkinsonian tremor?
essential is faster, BILATERAL, action tremor, and may respond to alcohol!
27
Which tremor responds to alcohol?
Essential tremor
28
Descrebe athetosis?
movements that are slow, sinuous, and writhing in nature
29
Describe dystonia?
movements are sustained so they appear to be abnormal postures
30
Botulinum toxin is used for treatment of what movement disorder?
FOCAL dystonia
31
What is used to treat generalized dystonia?
DBS
32
What percent of children have tics?
20%
33
Is it possible to outgrow tic?
yes
34
tourrette's syndrome is the combination of what two things?
motor and vocal tics
35
Are tics suppressible?
For a short time only
36
What movement disorder is characterized by violent movements of the proximal muscles and looks like chorea?
hemiballismus
37
(blank) disorder is seen commonly secondary to a stroke in the contralateral subthalamic nucleus
hemiballismus
38
T/F: hemiballismus will resolve spontaneously
T
39
T/F: chorea may be drug induced
true
40
(blank) happens in children as complication of previous group A hemolytic streptococcal infection and may recur during pregnancy
syndenham's chorea
41
Is the progression of chorea proximal to distal?
No, it is random
42
What is the average age of onset of HD?
40
43
(blank) is characterized by chorea, speech disturbances, falls, and cognitive and psychiatric changes
huntington's
44
Can HD have an early onset?
Yes, with akinesia
45
HD causes atrophy of what part of the brain?
caudate
46
T/F: HD is fully penetrant
true :(
47
What is the genetic mechanism for the HD mutation?
CAG trinucleotide repeat
48
How many repeats are necessary for full penetrance?
>40
49
How many repeats are necessary to be considered normal?
<26
50
T/F: HD shows anticipation
T
51
Tetrabenazine is a drug used for chorea that works primarily by:
depleting dopamine
52
(blank) percent of patients with HD suffer depression
50
53
T/F: 80% of pts with HD meet criteria for major depressive episode
False, only 30%
54
T/F: Mania presents in 1/10 pts with HD
T
55
Apathy, impulsivity, and executive dysfunction are characteristics of PD or HD?
HD
56
What risk does tetrabenazine carry with it?
While helping chorea, it increases the risk of suicide and depression
57
Wilson Disease has a triad of (blank x3) findings:
hepatic, neurological, and psychiatric
58
Is Wilson disease auto. dominant or recessive?
Recessive
59
Wilson disease is involved with the abnormal metabolism (build up) of what heavy metal?/
Copper
60
What is the gold standard for diagnosing wilson disease?
24 hour urine copper content >100ug
61
Is ceruloplasmin low or high in WD? Is this more or less sensitive that urine copper?
LOW, less sensitive
62
T/F: a liver biopsy without any neurological or psychiatric symptoms would be positive for WD
True
63
Kayser-Fleisher rings are a buildup of what element around the iris and are indicative of what disease?
copper, WD
64
T/F: Of the triad of symptoms in WD, only hepatic findings are always present
T
65
What are the three most common hepatic issues in WD
jaundice, varices, and spider angiomas
66
(Aphasia/Dysarthria) is commonly present in WD
dysarthria
67
What movement disorder should you consider if you have a child with abnormal movements?
Wilson's disease
68
What surgical procedure is curative for wilson's disease?
liver transplant
69
does a pedigree for wilson's disease present in the same way as all other normal autosomal recessive diseases?
yes
70
In WD, an MRI will show hyperintensities of the bilateral (blank and blank) on (T1/T2) images
thalamus and putamen on T2
71
The FACE OF THE GIANT PANDA is seen on midbrain images of what disease?
WD
72
In MRI imaging, seeing high signal in the tegmentum with a normal red nucleus is known as the (blank) and is seen in (HD/PD/WD)
FACE OF THE GIANT PANDA, Wilson's disease
73
Treatment of WD is the removal/lowering of (copper/iron)
copper
74
What element is used to block copper absorption?
zinc
75
Pnicillamine and trientene are chelators used to:
remove copper
76
T/F: scans or bloodwork are the primary, sufficient, and necssary method of diagnosing a movement disorder
False
77
What are the criteria for diagnosing a movement disorder? (3)
1. Good history 2. Family history 3. Good physical exam

Block 5 Week 3 Dani (12 decks)

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