Huntington + Tourette Flashcards
what is the etiology of huntington disease?
autosomal dominant inheritance
what is the age of onset of huntington disease?
30-50 years old
what is the pathogenesis of huntington disease?
degeneration of GABA neurons, which are the inhibitors of movement
what is observed in autopsy of a patient with huntington disease? (2)
severe striatal atrophy + ventricle enlargement
what are the 2 main S&S of huntington disease?
dementia
chorea
what does progression of dementia look like in huntington disease? (4)
irritability to…
moodiness to …
psychiatric disturbance to …
dementia
what is the progression of chorea in huntington disease? (3)
restlessness to …
fidgetiness to …
chorea/dystonia
what are 3 imaging to obtain to diagnose huntington disease?
CT/MRI
PET scan
what will be seen in a CT/MRI of a patient with huntington disease? (2)
atrophy
cerebral atrophy
atrophy of caudate nucleus
what will be seen in a PET scan of a patient with huntington disease?
reduced striatal metabolic rate
how is a diagnosis of huntington disease made?
genetic testing
what is the treatment goal for huntington disease?
symptomatic
what medication can be used for huntington to reduce movement and improve dyskinesia?
tetrabenazine for chorea
what medication can be used for huntington disease to control dyskinesia and behavioral disturbances?
haloperidol (antipsychotic)
what medication can be used for huntington disease for psychosis, disruptive behavioral symptoms without chorea?
“quiet for psychosis”
quetiapine
what should be offered to family members of patients who have huntington disease?
genetic testing
what is the etiology of tourette syndrome?
bilinear transmission
inherited from both parents in most cases
where does the disturbance occur in tourette syndrome?
mesolimbic spinal system
when is onset of Tourette syndrome?
before 18 yo
what is the majority of initial presentations of tourette syndrome?
motor tics
what can tourette syndrome be associated with?
obsessive-compulsive disorders
what is the 3 DSM-5 criteria for tourette syndrome?
multiple motor + at least 1 vocal tic at some point
present for over 1 year
onset before 18 yo
what is the treatment for mild, non-disabling tics? (2)
education
cognitive behavioral therapy
what is the treatment for bothersome tics?
tetrabenazine
what is the treatment for focal motor (motor tic in specific muscle group) or vocal tics?
botulinum toxin injections into affected muscles
what is the treatment for bothersome tics + prefer non-pharmacologic treatment or who have failed pharmacologic treatment? (2)
CBT + habit reversal training
