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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) Dancing Eyes-Dancing Feet Syndrome

B) Williams Syndrome

C) Alpers' Disease

D) Wernicke-Korsakoff Syndrome

E) Causalgia

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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) 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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B) 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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C) 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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D) Wernicke-Korsakoff Syndrome

This answer is incorrect.

Wernicke-Korsakoff Syndrome is a syndrome of ophthalmoparesis, nystagmus, ataxia, confusion, and inability to learn or form new memories. Other findings often include peripheral neuropathy, postural hypotension, and hypothermia. It is associated with thiamine deficiency, often secondary to malnutrition or alcoholism. (See References)


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E) Causalgia

This answer is incorrect.

Causalgia is a type of peripheral neuralgia in which there is persistent burning pain in an extremity, along with abnormal sympathetic innervation in the affected nerve. It is most often seen after trauma. (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