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Wilson's!

Topic: Adult

Created on Thursday, November 13 2008 by rednucleus

Last modified on Thursday, November 13 2008.

A 6-year-old male has been referred to you for further evaluation of a possible Wilson's disease because of dystonic gait and limbs. History taking and thorough physical examination are carried out by you and you think that Wilson's disease is not the correct diagnosis in this child. Why?

 
        A) Cataract
 
        B) Dysphagia
 
        C) Wing-beating tremor
 
        D) Favorable response to low dose L-dopa
 
        E) Flexor planters
 

 


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This question was created on November 13, 2008 by rednucleus.
This question was last modified on November 13, 2008.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) Cataract

This answer is incorrect.


Sun-flower cataract is seen with Wilson's.  (See References)

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B) Dysphagia

This answer is incorrect.


Dysphagia and dysarthria are one of the prominent features of Wilson's.  (See References)

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C) Wing-beating tremor

This answer is incorrect.


Highly characteristic of Wilson's. Note that Wilson's can result in resting, postural, and cerebellar types of tremor.  (See References)

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D) Favorable response to low dose L-dopa

This answer is correct.


One of the most important differential diagnosis of Wilson's is dopa responsive dystonia (DRD). All cases of childhood dystonia should receive a trial of a low dose L-dopa; if they respond, the diagnosis of DRD can be done.  (See References)

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E) Flexor planters

This answer is incorrect.


Solid upper motor neuron signs are against the diagnosis of Wilson's. So, this boy should have flexor planters if he has Wilson's.  (See References)

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References:

1. Victor, M., and Ropper, A.H. (2005). Adams and Victor's Principles of Neurology, 8th Edition. McGraw-Hill, New York. (ISBN:007141620X)Advertising:
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adult
Wilson's!
Question ID: 111308119
Question written by rednucleus. (C) FrontalCortex.com 2006-2009, all rights reserved. Created: 11/13/2008
Modified: 11/13/2008
Estimated Permutations: 120

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