Loading....
Coupon Accepted Successfully!

Medicine

Open Flashcards

Neurology

Question
16 out of 23
 

All are true about Huntington's disease except: (DNB Dec 2010)



A Chorea

B Behavioral disturbance

C Early onset of memory loss

D Cog-wheel rigidity.

Ans. C

Early onset of memory loss

Huntington’s Disease (Ref. Hari-18th ed., pg- 3330)

1). Autosomal dominant, degenerative brain disorder Q

2). The HD gene called IT IS is located on chromosome 4

3). It contain CAG triple repeat expansion

4). Onset is 4 –5th decade. May be childhood to >75year

5). Clinical hallmarks are progressive chorea and emotional disturbance, dementia and death Q

6). Gait is poorly coordinated and has a dancing quality. Emotional disturbance manifest as irritability in behavior

7). With advancing disease, there may be a reduction in chorea and emergence of dystonia, rigidity, bradykinesia, myoclonus, and spasticity.

8). Attention, Judgment, awareness and executive functions are seriously deficient at an early stage.

9). Depression, social withdrawal, irritability and disinhibitions positive

10). Seizure are common which Juvenile HD & rare which Adult HD.

11). Pathologically there is atrophy of caudate nucleus

12). Neuro chemically there is marked decrease at GABA anergic and cholinergic neurons in corpus striatum.

13). Treatment – Phenothiazine, haloperidol, benzodiazepines, olanzapine.

Extra Edge: Other triple repeat genetic abnormality are dystrophica myotonia (CTG),fragile X syndrome(CGG), Freidrich’s ataxia(GAA)}

Neurology Flashcard List

23 flashcards
1)
2)
3)
4)
5)
6)
7)
8)
9)
10)
11)
12)
13)
14)
15)
16)
17)
18)
19)
20)
21)
22)
23)