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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
Q

Where does an essential tremor usually present?

A

Neck or arms

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26
Q

how is an essential tremor different than a parkinsonian tremor?

A

essential is faster, BILATERAL, action tremor, and may respond to alcohol!

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27
Q

Which tremor responds to alcohol?

A

Essential tremor

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28
Q

Descrebe athetosis?

A

movements that are slow, sinuous, and writhing in nature

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29
Q

Describe dystonia?

A

movements are sustained so they appear to be abnormal postures

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30
Q

Botulinum toxin is used for treatment of what movement disorder?

A

FOCAL dystonia

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31
Q

What is used to treat generalized dystonia?

A

DBS

32
Q

What percent of children have tics?

A

20%

33
Q

Is it possible to outgrow tic?

A

yes

34
Q

tourrette’s syndrome is the combination of what two things?

A

motor and vocal tics

35
Q

Are tics suppressible?

A

For a short time only

36
Q

What movement disorder is characterized by violent movements of the proximal muscles and looks like chorea?

A

hemiballismus

37
Q

(blank) disorder is seen commonly secondary to a stroke in the contralateral subthalamic nucleus

A

hemiballismus

38
Q

T/F: hemiballismus will resolve spontaneously

A

T

39
Q

T/F: chorea may be drug induced

A

true

40
Q

(blank) happens in children as complication of previous group A hemolytic streptococcal infection and may recur during pregnancy

A

syndenham’s chorea

41
Q

Is the progression of chorea proximal to distal?

A

No, it is random

42
Q

What is the average age of onset of HD?

A

40

43
Q

(blank) is characterized by chorea, speech disturbances, falls, and cognitive and psychiatric changes

A

huntington’s

44
Q

Can HD have an early onset?

A

Yes, with akinesia

45
Q

HD causes atrophy of what part of the brain?

A

caudate

46
Q

T/F: HD is fully penetrant

A

true :(

47
Q

What is the genetic mechanism for the HD mutation?

A

CAG trinucleotide repeat

48
Q

How many repeats are necessary for full penetrance?

A

> 40

49
Q

How many repeats are necessary to be considered normal?

A

<26

50
Q

T/F: HD shows anticipation

A

T

51
Q

Tetrabenazine is a drug used for chorea that works primarily by:

A

depleting dopamine

52
Q

(blank) percent of patients with HD suffer depression

A

50

53
Q

T/F: 80% of pts with HD meet criteria for major depressive episode

A

False, only 30%

54
Q

T/F: Mania presents in 1/10 pts with HD

A

T

55
Q

Apathy, impulsivity, and executive dysfunction are characteristics of PD or HD?

A

HD

56
Q

What risk does tetrabenazine carry with it?

A

While helping chorea, it increases the risk of suicide and depression

57
Q

Wilson Disease has a triad of (blank x3) findings:

A

hepatic, neurological, and psychiatric

58
Q

Is Wilson disease auto. dominant or recessive?

A

Recessive

59
Q

Wilson disease is involved with the abnormal metabolism (build up) of what heavy metal?/

A

Copper

60
Q

What is the gold standard for diagnosing wilson disease?

A

24 hour urine copper content >100ug

61
Q

Is ceruloplasmin low or high in WD? Is this more or less sensitive that urine copper?

A

LOW, less sensitive

62
Q

T/F: a liver biopsy without any neurological or psychiatric symptoms would be positive for WD

A

True

63
Q

Kayser-Fleisher rings are a buildup of what element around the iris and are indicative of what disease?

A

copper, WD

64
Q

T/F: Of the triad of symptoms in WD, only hepatic findings are always present

A

T

65
Q

What are the three most common hepatic issues in WD

A

jaundice, varices, and spider angiomas

66
Q

(Aphasia/Dysarthria) is commonly present in WD

A

dysarthria

67
Q

What movement disorder should you consider if you have a child with abnormal movements?

A

Wilson’s disease

68
Q

What surgical procedure is curative for wilson’s disease?

A

liver transplant

69
Q

does a pedigree for wilson’s disease present in the same way as all other normal autosomal recessive diseases?

A

yes

70
Q

In WD, an MRI will show hyperintensities of the bilateral (blank and blank) on (T1/T2) images

A

thalamus and putamen on T2

71
Q

The FACE OF THE GIANT PANDA is seen on midbrain images of what disease?

A

WD

72
Q

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)

A

FACE OF THE GIANT PANDA, Wilson’s disease

73
Q

Treatment of WD is the removal/lowering of (copper/iron)

A

copper

74
Q

What element is used to block copper absorption?

A

zinc

75
Q

Pnicillamine and trientene are chelators used to:

A

remove copper

76
Q

T/F: scans or bloodwork are the primary, sufficient, and necssary method of diagnosing a movement disorder

A

False

77
Q

What are the criteria for diagnosing a movement disorder? (3)

A
  1. Good history
  2. Family history
  3. Good physical exam

Block 5 Week 3 Dani (12 decks)

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