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Guess the disease!

Topic: Behavior

Created on Wednesday, February 21 2007 by jdmiles

Last modified on Wednesday, February 21 2007.

GUESS WHAT I'M THINKING!

I'm thinking of a disease wherein decreased cerluloplasmin leads to deposition of copper first in the liver and later in the brain (especially putamen and globus pallidus). Common symptoms include tremor and bradykinesia. Treatment is with d-penicillamine.
What disease am I thinking of?

 
        A) Zellweger Syndrome
 
        B) Alpers-Hutttenlocher syndrome
 
        C) Korsakoff's Amnesic Syndrome
 
        D) Alexander Disease
 
        E) Wilson's Disease
 

 


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This question was created on February 21, 2007 by jdmiles.
This question was last modified on February 21, 2007.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) Zellweger Syndrome

This answer is incorrect.


Zellweger Syndrome is an autosomal recessive disorder that results in a lack of liver peroxisomes. It results in accumulation of very long chain fatty acids, and is terminal at a few months of age.  (See References)

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B) Alpers-Hutttenlocher syndrome

This answer is incorrect.


Alpers-Hutttenlocher syndrome is a subtype of progressive cerebral poliodystrophy in which there are also hepatic symptoms: jaundice, fatty degeneration, and cirrhosis. Other symptoms include anemia, thrombocytopenia, and trichorrhexis.  (See References)

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C) Korsakoff's Amnesic Syndrome

This answer is incorrect.


Korsakoff's Amnesic Syndrome is a syndrome of severe anterograde and retrograde amnesia. It is often associated with polyenuropathy. It is frequently associated with a thiamine deficiency secondary to alcoholism or malnutrition, with lesions of the mammillary bodies visible on pathology or MRI. It can have other etiologies, including ischemic lesions in various parts of the brain.   (See References)

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D) Alexander Disease

This answer is incorrect.


Alexander Disease is a rare, congenital disease which does not appear to be inherited. No metabolic cause has been found. Clinical features include seizures, psychomotor retardation, and failure to thrive, followed by progressive macrocephaly. The frontal lobes show white matter degeneration, and pathology shows Rosenthal fibers found near the pia and blood vessels, these are thought to be detritus from glial destruction.  (See References)

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E) Wilson's Disease

This answer is correct.


Wilson's Disease is a disease wherein decreased cerluloplasmin leads to deposition of copper first in the liver and later in the brain (especially putamen and globus pallidus). Common symptoms include tremor and bradykinesia. Treatment is with d-penicillamine.  (See References)

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References:

1. Victor, M., and Ropper, A.H. (2001). Adams and Victor's Principles of Neurology, 7th Edition. McGraw-Hill, New York.
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behavior
Guess the disease!
Question ID: 02210701
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 02/21/2007
Modified: 02/21/2007
Estimated Permutations: 0

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