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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 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.
What disease am I thinking of?

 
        A) Wernicke's Encephalopathy
 
        B) Dancing Eyes-Dancing Feet Syndrome
 
        C) Asperger Syndrome
 
        D) Alpers' Disease
 
        E) Korsakoff's Amnesic 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) 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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B) Dancing Eyes-Dancing Feet Syndrome

This answer is incorrect.


Dancing Eyes-Dancing Feet Syndrome is a cerebellar ataxia with polymyoclonus which may be postinfectious.  (See References)

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C) Asperger Syndrome

This answer is incorrect.


Asperger Syndrome is a disorder thought to typify a high-functioning variant of Autism. These patients may be adept at various cognitive skills, but have difficulty relating socially or emotionally with others.  (See References)

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D) Alpers' Disease

This answer is correct.


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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E) 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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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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