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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 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.
What disease am I thinking of?

A) Williams Syndrome

B) Alpers' Disease

C) Brown-Sequard Syndrome

D) Wilson's Disease

E) Zellweger 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) 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) Alpers' Disease

This answer is correct.

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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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) 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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E) Zellweger Syndrome

This answer is incorrect.

Zellweger Syndrome is an autosomal recessive disorder that results in a lack of liver peroxisomes. It results in accumulation of very long chain fatty acids, and is terminal at a few months of age. (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