Dyskinetic Movement Disorders Flashcards

1
Q

What is an essential tremor?

A

Tremor not associated with neuro disease

Enhanced on maintaining postures of holding arms out

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What exacerbates essential tremor?

A

Anxiety

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What may improve essential tremor?

A

Alcohol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

How is essential tremor investigated?

A

CT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How is disabling essential tremor managed?

A

Beta-blockers
Topiramate
Gabapentin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

How is Huntingtons disease inherited?

A

AD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Where is the Hungtingtin gene located?

A

Chromosome 4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

How many CAG repeats indicate Huntington’s?

A

> 36

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

How does Huntington’s disease present?

A

Chorea

Subtle personality change - irritable, aggressive, loss of interest

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

How is Huntington’s disease investigated?

A

MRI (showing atrophy of head of caudate)

Genetic testing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

How is Huntington’s treated?

A

Tetrabenazine for hyerkinesias

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is Wilson’s disease?

A

Build up of copper leading to hepatocellular degeneration and other organ damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

How is Wilson’s disease inherited?

A

AR

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Where is the mutation in Wilson’s disease?

A

ATP7B gene on chromosome 13

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What enzyme is affected in Wilson’s disease?

A

Caeruloplasmin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

How does Wilson’s disease present?

A
Dystonia
Parkinsonism
Tremor
Seizure
KF rings
17
Q

What do investigations show in Wilson’s disease?

A
LFTs abnormal
Serum copper increase
Caeruloplasmin conc decrease
Increased urinary copper excretion
Copper on liver biospy
18
Q

What does MRI show in Wilson’s disease?

A

Hyperintense putamen

19
Q

How is Wilson’s disease managed?

A

D-penicillamine
Zinc
Liver transplant

20
Q

How does D-penicillamine work in Wilson’s disease?

A

Copper chelating agent

21
Q

How does Zinc work in the treatment of Wilson’s disease?

A

Competes with copper for absorption

22
Q

What is Syndenham’s chorea?

A

Unilaterla, post-infective chorea

23
Q

How is Sydenham’s chorea treated?

A

It should settle spontaneously

24
Q

What is Gilles de la Tourette’s syndrome?

A

Syndrome causing multiple involuntary motor and vocal tics

25
Q

How is Gilles de la Tourette’s syndrome treated?

A

Clonidine or haloperidol

26
Q

What is Gilles de la Tourette’s associated with?

A

OCD

ADHD