Thursday 8 June 2017

Niemann picks disease | DNB Based MCQ

NIME Next Batch PGI Quest in Delhi from 10 August to 20 August


In Niemann picks disease, the main phospholipids abnormally deposited in brain, spleen and liver is: 

A. Lecithin 
B. Plasmalogen 
C. Cardiolipin 
D. Sphingomyelin

Ans. D. Sphingomyelin
• Accumulation of galactocerebroside occurs in Krabbe disease, which is due to galactocerebrocide deficiency.
• Accumulation of glucocerebroside occurs in Gaucher disease, which is due to defects in 13 glucocerebrosidase. The reticuloendothelial cells and CNS are affected.
• Accumulation of GM2 ganglioside occurs in Tay-Sachs disease because of hexosaminidase A deficiency. The swollen ganglion cells of the retina contribute to a classic sign of Tay-Sachs---the macular cherry-red spot.
• Accumulation of sphingomyelin in a variety of organs occurs in Niemann-Pick disease, which is due to a defect in sphingomyelinase.
• Accumulation of sulfatide occurs in Metachromatic leukodystrophy, caused by arylsulfatase A deficiency.