Resident level quizzes are in crimson, student level quizzes are in blue.

Quiz Set: N-009

Kar-Ming Fung, M.D., Ph.D.

1.    It has been proved that Canavan's disease is more frequently seen in which of the following racial ethnic group?  Answer

A.  Ashkenazi Jews

B.  Japanese, Koreans, and Chinese from the northeastern part of China

C.  African American

D.  American Indians

E.  Asians living in the Indian subcontinent

2.    In addition to Canavan's disease, which of the following diseases are also characterized by progressive enlargement of the head?  Answer

A.  Mitochondrial myopathy, encephalopathy, lactic-acidosis, and stroke like episodes (MELAS)

B.  Cerebral amyloid angiopathy (CAA)

C.  Moyamoya disease

D.  Alexander's disease   

E.  Ophthalmoplegic retinitis pigmentosa with polyneuropathy

3.    Electron microscopy will not be helpful in identifying abnormal metabolites in which of the following diseases?  Answer

A.  Krabbe's disease

B.  Tay Sachs Disease

C.  X-linked adrenocortical leukodystrophy

D.  Mucopolysaccharides

E.  McArdle's disease

4.    Abnormal accumulation of substances will be detected by electron microscopy in which of the following diseases?  Answer

A.  Canavan's disease

B.  Glutaric acidemia type I

C.  Methanol intoxication

D.  Hartnup disease

E.  Gangliosidosis (GM1 and GM2)

5.    Polysaccharide that cannot be digested by diastase is the major constituent of the abnormal accumulation of which of the following diseases?  Answer

A.  Adult polyglucosan body disease

B.  McArdle's disease

C.  Farber's disease

D.  Gauche's disease

E.  Dementia with Lewy bodies

6.    Which of the following disease is not a lysosomal storage disease?  Answer

A.  Pompe's disease

B.  McArdle's disease

C.  Farber's disease

D.  Gauche's disease

E.  Nieman-Pick disease

Last updated: October, 20, 2011. Contributed by Kar-Ming Fung, M.D., Ph.D.

Dept. of Pathology, University of Oklahoma Health Sciences Center, Oklahoma City, OK, U.S.A.