Huntington's Disease and Dystonia Flashcards
(20 cards)
what does hyperkinetic mean?
excessive movement
what does hypokinetic mean?
too little (paucity) movement
what is chorea?
jerky movements that are involuntary and rapid
what causes HD?
defective gene, therefore HD is inherited and is a dominant gene
because of the autosomal dominant inheritance pattern, what chance does a child have of inheriting HD if 1 parent is unaffected and 1 parent is?
50/50
when is the typical onset age?
35-55
what are the communication changes in HD?
dysarthria
language and cognitive
what other symptoms of HD are there?
dysphagia
motor control changes
mood changes
cognitive
what happens to speech as the disease progresses?
- early on it becomes unintelligible
- often non-verbal in advanced stages
what are the speech difficulties a patient with HD faces?
impaired breathing
hoarseness
inappropriate rate
imprecise articulation
what are the language difficulties facing a HD patient?
- limited ability to -respond within a convo
- specific word finding difficulties
- slow response time
- difficulty understanding complex info
which gene is affected?
tis on chromosome 4
what does the huntingtin protein do to the brain?
causes neurones to die - the cortex and basal ganglia are particularly sensitive
which areas of the brain are affected with Dystonia?
dysfunction in basal ganglia, cerebellum, motor cortexes + sensorimotor cortexes
how do people get dystonia?
it can be inherited or acquired
what symptoms are associated with dystonia?
uncontrollable and excessive muscle spasms and contractions,
tremor
how many dystonia sub groups are there?
primary (only thing)
secondary acquired (concomitant)
plus (resembles another disorder e.g. PD)
what kind of surgery would help manage symptoms?
thalamotomy
pallidotomy
what is laryngeal dystonia?
when the laryngeal muscles spasm
what kind of dystonia affects lower facial muscles, tongue and jaw, and may result in swallowing difficulties?
oromandibular dystonia