Huntington + Tourette Flashcards

1
Q

what is the etiology of huntington disease?

A

autosomal dominant inheritance

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2
Q

what is the age of onset of huntington disease?

A

30-50 years old

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3
Q

what is the pathogenesis of huntington disease?

A

degeneration of GABA neurons, which are the inhibitors of movement

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4
Q

what is observed in autopsy of a patient with huntington disease? (2)

A

severe striatal atrophy + ventricle enlargement

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5
Q

what are the 2 main S&S of huntington disease?

A

dementia
chorea

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6
Q

what does progression of dementia look like in huntington disease? (4)

A

irritability to…
moodiness to …
psychiatric disturbance to …
dementia

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7
Q

what is the progression of chorea in huntington disease? (3)

A

restlessness to …
fidgetiness to …
chorea/dystonia

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8
Q

what are 3 imaging to obtain to diagnose huntington disease?

A

CT/MRI
PET scan

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9
Q

what will be seen in a CT/MRI of a patient with huntington disease? (2)

A

atrophy

cerebral atrophy
atrophy of caudate nucleus

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10
Q

what will be seen in a PET scan of a patient with huntington disease?

A

reduced striatal metabolic rate

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11
Q

how is a diagnosis of huntington disease made?

A

genetic testing

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12
Q

what is the treatment goal for huntington disease?

A

symptomatic

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13
Q

what medication can be used for huntington to reduce movement and improve dyskinesia?

A

tetrabenazine for chorea

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14
Q

what medication can be used for huntington disease to control dyskinesia and behavioral disturbances?

A

haloperidol (antipsychotic)

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15
Q

what medication can be used for huntington disease for psychosis, disruptive behavioral symptoms without chorea?

A

“quiet for psychosis”

quetiapine

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16
Q

what should be offered to family members of patients who have huntington disease?

A

genetic testing

17
Q

what is the etiology of tourette syndrome?

A

bilinear transmission
inherited from both parents in most cases

18
Q

where does the disturbance occur in tourette syndrome?

A

mesolimbic spinal system

19
Q

when is onset of Tourette syndrome?

A

before 18 yo

20
Q

what is the majority of initial presentations of tourette syndrome?

A

motor tics

21
Q

what can tourette syndrome be associated with?

A

obsessive-compulsive disorders

22
Q

what is the 3 DSM-5 criteria for tourette syndrome?

A

multiple motor + at least 1 vocal tic at some point

present for over 1 year

onset before 18 yo

23
Q

what is the treatment for mild, non-disabling tics? (2)

A

education
cognitive behavioral therapy

24
Q

what is the treatment for bothersome tics?

A

tetrabenazine

25
Q

what is the treatment for focal motor (motor tic in specific muscle group) or vocal tics?

A

botulinum toxin injections into affected muscles

26
Q

what is the treatment for bothersome tics + prefer non-pharmacologic treatment or who have failed pharmacologic treatment? (2)

A

CBT + habit reversal training