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) Reye's Syndrome
 
        C) Williams Syndrome
 
        D) Hemifacial Spasm
 
        E) Alexander Disease
 

 

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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 incorrect.

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) 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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C) Williams Syndrome

This answer is correct.

Williams Syndrome is a disease, also called elfin facies syndrome, which is a congenital form of mental retardation related to an abnormality on chromosome 7. Patients are mildly developmentally delayed, but retain musical ability and social skills. Motor skills are impaired. Patients tend also to have supravalvular aortic stenosis.  (See References)

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D) Hemifacial Spasm

This answer is incorrect.

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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E) 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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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