Loading....
Coupon Accepted Successfully!

Medicine

Open Flashcards

Neurology

Question
18 out of 27
 

True about CADASIL (PNQ)



A Monogenic stroke syndrome

B White matter changes

C Onset is usually in the fourth or fifth decade of life

D All of the above

Ans. D

All of the above

Locked-in syndrome, is a condition in which a patient is aware and awake but cannot move or communicate verbally due to complete paralysis of nearly all voluntary muscles in the body except for the eyes. Total locked-in syndrome is a version of locked-in syndrome where the eyes are paralyzed, as well.

Locked-in syndrome is also known as cerebromedullospinal disconnection pseudocoma and ventral pontine syndrome.

Causes

Unlike persistent vegetative state, in which the upper portions of the brain are damaged and the lower portions are spared, locked-in syndrome is caused by damage to specific portions of the lower brain and brainstem (Ventral Pons), with no damage to the upper brain. Locked-in syndrome can be caused by stroke at the level of the basilar artery denying blood to the pons.

CADASIL (cerebral Autosomal dominant arteriopathy with subcortical infarcts & leukoencephalopathy) (Ref. Hari-18th ed., pg-3279)

1). It is a monogenic stroke syndrome.

2). It is an inherited disorder that presents as small-vessel strokes, progressive dementia, and extensive symmetric white matter changes visualized by MRI.

3). Onset is in the fourth or fifth decade of life.

4). This autosomal dominant condition is caused by one of several mutations in Notch-3.

5). Other monogenic ischemic stroke syndromes are

a. Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL)

b. Hereditary endotheliopathy, retinopathy, nephropathy, and stroke ( Ref. HariERNS).

c. Fabry's disease also produces both large-vessel arteriopathy and small-vessel infarcts by an unknown

d. mechanism.

Neurology Flashcard List

27 flashcards
1)
2)
3)
4)
5)
6)
7)
8)
9)
10)
11)
12)
13)
14)
15)
16)
17)
18)
19)
20)
21)
22)
23)
24)
25)
26)
27)