Huntingdon's Flashcards
huntingdon’s is caused due to
degeneration of basal ganglia
- specifically indirect pathway
what is the pathology of huntingdon’s
degeneration of striatum and cortex
lack of inhibition in the globus palladis externus
globus palladis externus is abnormally active and unable to inhibit the thalamus
how does huntingdon’s affect the relationship between the thalamus and globus palladis externus
GPE is not able to inhibit the thalamus
thalamus will send excessive excitatory signals back to the cortex
intentional movement in those with huntingdon’s will be
hyperkinetic
ballistic
choreiform like
explain etiology of huntingdon’s disease? what is the likelihood?
genetic - child of parent with HD has 50% chance of inheritance
typical age of onset, time with disease, and age of death
30-40s
death typically occurs 15-20 years after onset of symptoms
how is mortality in those with HD different from other neurodegenerative disease
HD - disease will be the thing that kills person
other = systematic failure of organ system causes death (ie respiratory failure with ALS)
clinical manifestation of HD in movement
dyskinesias
chorea
athetosis
what are dyskinesias
abnormal involuntary movements
what is chorea
jerky, arrhythmic movements
what is athetosis
rhythmic and wormlike movements
what gait dysfunctions can be seen in HD
ataxic / choeriform like movement
decreased stride length
decreased stride velocity
widened BOS / lateral sway
hyperkinetic movements in the face can cause
swallowing difficulties
speech difficulties
eye movement difficulties
cognitive impairments noticed in HD
dementia
lack of judgement
memory loss
personality changes / depression
what auxillary clinical manifestations can be seen in those with HD
abnormal posture reactions
reduced trunk rotation
abnormal tone
what are the most common initial symptoms of HD
any one or combination of:
incoordination
clumsiness
jerkiness
what is the medical management of HD
ain’t one
- meds are given to control extra movements
what do medications typically result in for those with HD
parkinsonian-like symptoms
what should be focused on during examination in HD patients
movement analysis
cognition screening
what outcome measures are used in HD populations
6MWT
TUG
Berg
SF-36
goals of treatment in those with DH
maintaining QOL
prevention of secondary impairments
education on preventions, AE, safety
fall prevention
when thinking about interventions, we should encourage
external cueing for purposeful movement
– access cortical pathways more than basal ganglia’s automatic pathways
what is the SF-36
Short-Form 36 item survey that assesses functional status and well being
what are the domains assessed in the SF-36
physical function
limitations
vitality
social function
emotional based limitations
mental health
health transition
strong recommendations for exercise in patients with HD include
aerobic ex at mod intensity
strengthening (3x week for 12 wks)
gait training
what % HRmax is associated with aerobic exercise in those with HD
55-90% HRmax
what are the goals of gait training in HD populations
improving spatiotemporal measures
speed / step length
what is weakly recommended for those with HD
balance / breathing exercises
