Guess the disease!

Topic: Behavior

Created on Wednesday, February 21 2007 by jdmiles

Last modified on Wednesday, February 21 2007.

 
        A) Rett Syndrome
 
        B) Korsakoff's Amnesic Syndrome
 
        C) Hemifacial Spasm
 
        D) Wilson's Disease
 
        E) Riley-Day 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

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A) Rett Syndrome

This answer is incorrect.

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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B) 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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C) Hemifacial Spasm

This answer is correct.

Hemifacial Spasm is a disorder characterised by painless twitching of the muscles on one side of the face (in 5%, bilateral, but asynchronous when it is). Usually caused by arterial compression of the facial nerve, which results in focal demyelination followed by ephaptic coupling of parallel axons. It can also result from Bell's palsy. Medcial treatments include Carbemazepine, baclofen, gabapentin and botulinum toxin. Surgical decompression of the nerve is a highly successful procedure, with some risks, including monaural deafness.  (See References)

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D) Wilson's Disease

This answer is incorrect.

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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E) 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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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