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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 X-linked dominant form of mental retardation primarily affecting girls. These girls develop normally until 6 to 18 months of age, after which they lose mental abilities and motor skills.
What disease am I thinking of?

 
        A) Sydenham Chorea
 
        B) Alexander Disease
 
        C) Wernicke's Encephalopathy
 
        D) Causalgia
 
        E) Rett 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) Sydenham Chorea

This answer is incorrect.


Sydenham Chorea is a choreiform disorder associated with streptococcal infection.   (See References)

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B) 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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C) Wernicke's Encephalopathy

This answer is incorrect.


Wernicke's Encephalopathy is a syndrome of ophthalmoparesis, nystagmus, ataxia, and confusion. Other findings often include postural hypotension and hypothermia. It is associated with thiamine deficiency, often secondary to malnutrition or alcoholism.   (See References)

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D) Causalgia

This answer is incorrect.


Causalgia is a type of peripheral neuralgia in which there is persistent burning pain in an extremity, along with abnormal sympathetic innervation in the affected nerve. It is most often seen after trauma.  (See References)

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E) Rett Syndrome

This answer is correct.


Rett Syndrome is an X-linked dominant form of mental retardation primarily affecting girls. These girls develop normally until 6 to 18 months of age, after which they lose mental abilities and motor skills.  (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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