Huntington's Disease

Question 1

what are the three CNS pathologies we are covering in this course?

Answer 1

PD, MS, and HD

Question 2

what is the only CNS & PNS pathology we are covering in this course

Answer 2

ALS and related pathologies

Question 3

what are the four PNS pathologies we are covering in this course

Answer 3

Polio/PPD, GBS, CMT, MG

Question 4

what is the etiology of HD

Answer 4

autosomal dominant

Question 5

what is the pathophysiology of HD

Answer 5

neuronal loss particularly in the caudate nucleus and putamen (corpus striatum)

Question 6

When is onset of sx in patients with HD

Answer 6

between ages 30-40

Question 7

what is the sx triad for HD

Answer 7

cognitive, psych, and movement disorders

Question 8

how does a HD brain appear on autopsy

Answer 8

like an Alzhiemers brain - enlarged ventricles and shrunken lobes

Question 9

what are the cognitive symptoms of HD

Answer 9

1. the earliest sign of the disease
2. slowed thinking/problem solving
3. memory deficits

Question 10

what are the pyschiatric sxs of HD

Answer 10

all of them - psych dx are integrated with the disorder and not a separate dx

Question 11

what is the classic movement finding for HD

Answer 11

involuntary choreaform or hemiballistic movement combined with hand dystonia

Question 12

how are voluntary movements in HD patients

Answer 12

uncoordinated and hypokinetic with poor postural control

Question 13

lifespan after HD dx

Answer 13

10-20 years - no cure

Question 14

describe pharm tx for HD

Answer 14

geared towards psych disorder management: antidepressants, antipsychotics, and mood-stabilizers

Question 15

what can be a medication Rx’d for movement disorders assoc with HD

Answer 15

tetrabenazine (xenazine) to suppress involuntary jerking and writhing (chorea)

Question 16

what does the literature say about exercise and HD patients

Answer 16

in favor - benefits to motor function, gait speed, balance, and CV function