Guess the disease!

Topic: Behavior

Created on Wednesday, February 21 2007 by jdmiles

Last modified on Wednesday, February 21 2007.

 
        A) Alpers-Hutttenlocher syndrome
 
        B) Korsakoff's Amnesic Syndrome
 
        C) Brown-Sequard Syndrome
 
        D) Riley-Day Syndrome
 
        E) Devic's 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) 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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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) 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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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) Devic's Syndrome

This answer is incorrect.

Devic's Syndrome is a variant of multiple sclerosis (MS), in which optic neuritis and transverse myelitis occur together, or at least within weeks of each other. The transverse myelitis frequently leads to necrosis, and is thus more permanent than the usual transverse myelitis associated with MS. It also differs from MS in that the lesions are often limited to those described above, and the rest of the CNS is spared. Serologic finidngs are also frequently different from those of MS, e.g., no oligoclonal bands.  (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