Guess the disease!

Topic: Behavior

Created on Wednesday, February 21 2007 by jdmiles

Last modified on Wednesday, February 21 2007.

 
        A) Williams Syndrome
 
        B) Wernicke's Encephalopathy
 
        C) Tropical Spastic Paraparesis
 
        D) Riley-Day Syndrome
 
        E) Alpers-Hutttenlocher 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) Williams Syndrome

This answer is incorrect.

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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B) 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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C) 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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D) Riley-Day Syndrome

This answer is correct.

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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E) Alpers-Hutttenlocher syndrome

This answer is incorrect.

Alpers-Hutttenlocher syndrome is a subtype of progressive cerebral poliodystrophy in which there are also hepatic symptoms: jaundice, fatty degeneration, and cirrhosis. Other symptoms include anemia, thrombocytopenia, and trichorrhexis.  (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