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Werdnig-Hoffman Disease

Topic: Pediatric

Created on Sunday, September 28 2008 by jdmiles

Last modified on Sunday, September 28 2008.

Which of the following statements about Werdnig-Hoffman disease is most accurate?

 
        A) Onset is most frequently after the first decade
 
        B) It is related to a defect on the APP gene
 
        C) The pathology involves the loss of motor and sensory neurons
 
        D) It is related to a defect in the SMN gene
 
        E) The pathology involves the loss of upper motor neurons
 

 


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This question was created on September 28, 2008 by jdmiles.
This question was last modified on September 28, 2008.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) onset is most frequently after the first decade

This answer is incorrect.


Onset of Werdnig-Hoffman disease, or infantile spinal muscular atrophy, is in the neonatal period.  (See References)

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B) it is related to a defect on the APP gene

This answer is incorrect.


Werdnig-Hoffman disease, or infantile spinal muscular atrophy, is related to a defect on the survival motor neuron gene (SMN), which is on chromosome 5.  (See References)

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C) the pathology involves the loss of motor and sensory neurons

This answer is incorrect.


Werdnig-Hoffman disease involves the loss of lower motor neurons.  (See References)

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D) it is related to a defect in the SMN gene

This answer is correct.


Werdnig-Hoffman disease, or infantile spinal muscular atrophy, is related to a defect on the survival motor neuron gene (SMN), which is on chromosome 5.  (See References)

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E) the pathology involves the loss of upper motor neurons

This answer is incorrect.


Werdnig-Hoffman disease involves the loss of lower motor neurons.  (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. (ISBN:0070674973)Advertising:
2. Leistikow, E.A., Jones, N.E., Josephson, K.D., de Sierra, T.M., Costakos, D.T., Sprague, D., Gorch, D.H., and Asonye, U.O. (1999). "Migrating atelectasis in Werdnig-Hoffmann disease: pulmonary manifestations in two cases of spinal muscular atrophy type 1." Pediatr Pulmonol, 28(2) 149-53. (PMID:10423316)
3. Rowland, L.P. (Ed) (2000). Merritt's Neurology, 10th Edition. Lippincott Williams & Wilkins, Philadelphia. (ISBN:0683304747)Advertising:
4. Prayson, R.A., and Goldblum, J.R. (Eds.) (2005). Neuropathology. Elsevier Churchill Livingstone, Philadelphia. (ISBN:0443066582)Advertising:
5. Chitnis, T., and Khoury, S.J. (2004). Neuroimmunology. In Bradley, W.G., Daroff, R.B., Fenichel, G.M., and Jankovic, J. (Eds.). Neurology in Clinical Practice, Fourth Edition. Butterworth Heinemann, Philadelphia, pp. 809-829 (ISBN:0750674695).Advertising:
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pediatric
Werdnig-Hoffman Disease
Question ID: 092808131
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 09/28/2008
Modified: 09/28/2008
Estimated Permutations: 3304800

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