Huntington's Disease and Dystonia Flashcards Preview

Neurology > Huntington's Disease and Dystonia > Flashcards

Flashcards in Huntington's Disease and Dystonia Deck (18)
Loading flashcards...
1
Q

What is Hyperkinetic movement?

A

Too much movement. (excessive)

2
Q

What is Hypokinetic movement?

A

Too little movement. (Paucity)

3
Q

If a parent has Huntington’s disease what child does their child have of inheriting it.

A

a 50/50 chance.

4
Q

What causes Huntington’s disease?

A

A defective gene on chromosome 4 - which produces a protein called Huntingtin- this protien becomes mutated and causes neurons to die.

5
Q

What number of years is the typical progression of HD?

A

10-25 years

6
Q

What is Juvenile HD?

A

onset before 20 years.

7
Q

What are symptoms of HD?

A
  • Language/speech changes: Dysarthria
  • Dysphagia
  • Motor control changes
  • Behavioural and mood changes
  • Cognitive changes
8
Q

What are 2 examples of speech and language difficulties experienced by someone with HD?

A
  • impaired breathing for speech production
  • hoarse, harsh, strained or strangled vocal quality
  • inappropriate rate, rhythm and pitch of speech
  • imprecise articulation.
9
Q

What are 2 examples of cognitive and language difficulties experienced by someone with HD?

A
  • difficulties beginning conversation
  • lack of spontaneity in communication
  • difficulty putting thoughts into words
  • reduced number of words available to the person
  • limited ability to respond within a conversation
  • specific word finding difficulties
  • difficulty understanding complex information
  • slow response time
10
Q

Medical managment of HD include?

A
  • Anti-psychotic medication

- Antidepressants

11
Q

Dystonia is caused by….

A

dysfunction in the Basal Ganglia, cerebellum, supplementary motor areas, sensorimotor cortex

12
Q

Symptoms of dystonia?

A

uncontrollable excessive muscle spasms and contractions (involuntary)
sometimes a tremor is present too

13
Q

Which of the following could be used to test for dystonia?

  • MRI scan
  • CAT scan
  • Blood tests
  • Urine tests
  • CSF test
  • X-ray
  • genetic testing
A
  1. MRI
  2. Blood & Urine tests
  3. Genetics testing
14
Q

What is secondary dystonia?

A

dystonia resulting from external factors, eg, another neurological condition, trauma or medication.

15
Q

Which type of dystonia affects the ‘trunk’ and at least two other parts of the body?

A

Generalised dystonia.

16
Q

Whats the difference between Segmental and Multifocal dystonia?

A

both affect two regions of the body:

in segmental dystonia it is two connected regions and in multifocal it is two unconnected regions.

17
Q

What is Hemidystonia?

A

When it affects one side of the body entirely.

18
Q

which of the following can be used to treat dystonia:

  • muscle stimulants
  • muscle relaxants
  • levopada
  • baclofen
  • aspirin
  • botox
  • deep brain stimulation
  • pallidotomy
  • thalamotomy
A
  • muscle relaxants
  • baclofen
  • botox
  • deep brain stimulation
  • pallidotomy
  • thalamotomy