Guess the disease!

Topic: Behavior

Created on Wednesday, February 21 2007 by jdmiles

Last modified on Wednesday, February 21 2007.

 
        A) Brown-Sequard Syndrome
 
        B) Wilson's Disease
 
        C) Alexander Disease
 
        D) Tropical Spastic Paraparesis
 
        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) Brown-Sequard Syndrome

This answer is correct.

Brown-Sequard Syndrome is a homolateral paralysis which spares the face, with ipsilateral loss of position and vibratory sense, and contralateral loss of temperature and pain. Results from a unilateral spinal cord lesion.  (See References)

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B) 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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C) 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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D) Tropical Spastic Paraparesis

This answer is incorrect.

Tropical Spastic Paraparesis is a myelopathy related to infection with the HTLV-I virus. It is endemic to Martinique, Jamaica, Colombia and Japan. Key clinical signs include slowly progressing paraparesis with increased DTRs and positive Babinski.  (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