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Guess the disease!

Topic: Behavior

Created on Wednesday, February 21 2007 by jdmiles

Last modified on Wednesday, February 21 2007.

GUESS WHAT I'M THINKING!
I'm thinking of 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.
What disease am I thinking of?

A) Alpers' Disease

B) Alexander Disease

C) Korsakoff's Amnesic Syndrome

D) Sydenham Chorea

E) Dancing Eyes-Dancing Feet 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

A) Alpers' Disease

This answer is incorrect.

Alpers' Disease is a disorder which may be inherited or sporadic. It begins in early infancy with loss of smile, loss of interest in surroundings, episodes of sweating, seizures, and diffuse myoclonic jerks. As it progresses, there follows diffuse spasticity, blindness, delayed growth, and progressive microcephaly. In late stages, the patient is essentially decorticate. The etiology is not yet understood. (See References)


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B) 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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C) Korsakoff's Amnesic Syndrome

This answer is correct.

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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D) Sydenham Chorea

This answer is incorrect.

Sydenham Chorea is a choreiform disorder associated with streptococcal infection. (See References)


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E) Dancing Eyes-Dancing Feet Syndrome

This answer is incorrect.

Dancing Eyes-Dancing Feet Syndrome is a cerebellar ataxia with polymyoclonus which may be postinfectious. (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