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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 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.
What disease am I thinking of?

 
        A) Alpers' Disease
 
        B) Riley-Day Syndrome
 
        C) Zellweger Syndrome
 
        D) Reye's Syndrome
 
        E) Wernicke-Korsakoff Syndrome
 

 


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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) Alpers' Disease

This answer is incorrect.


Alpers' Disease is a disorder which may be inherited or sporadic. It begins in early infancy with loss of smile, loss of interest in surroundings, episodes of sweating, seizures, and diffuse myoclonic jerks. As it progresses, there follows diffuse spasticity, blindness, delayed growth, and progressive microcephaly. In late stages, the patient is essentially decorticate. The etiology is not yet understood.  (See References)

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B) Riley-Day Syndrome

This answer is incorrect.


Riley-Day Syndrome is a congenital, autosomal recessive disease which predominately affects Jewish children. Failure to thrive and fever are early signs. Hyporeflexia and decreased pain & temperature sensation are noted on exam. There is also poor function of the autonomic nervous system. The standard test is for dopamine B-hydroxylase, which is diminished.  (See References)

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C) Zellweger Syndrome

This answer is correct.


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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D) Reye's Syndrome

This answer is incorrect.


Reye's Syndrome is a form of hepatic encephalopathy, marked by fatty liver and cerebral edema. It is seen in children and adolescents, and is associated with the use of aspirin during viral infections, especially influenza and varicella.  (See References)

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E) Wernicke-Korsakoff Syndrome

This answer is incorrect.


Wernicke-Korsakoff Syndrome is a syndrome of ophthalmoparesis, nystagmus, ataxia, confusion, and inability to learn or form new memories. Other findings often include peripheral neuropathy, postural hypotension, and hypothermia. It is associated with thiamine deficiency, often secondary to malnutrition or alcoholism.   (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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