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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) The pathology involves the loss of upper and lower motor neurons
 
        B) It is a common cause of neonatal hypotonia
 
        C) It is also known as SCA type 3
 
        D) Onset is most frequently after the first decade
 
        E) Prognosis is fair
 

 


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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) the pathology involves the loss of upper and lower motor neurons

This answer is incorrect.


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

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B) it is a common cause of neonatal hypotonia

This answer is correct.


After the immediate postnatal period, Werdnig-Hoffman disease is considered to be the most common cause of neonatal hypotonia.  (See References)

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C) it is also known as SCA type 3

This answer is incorrect.


Werdnig-Hoffman disease, or infantile spinal muscular atrophy,is also known as SMA-1. SCA type 3 refers to Spino-cerebellar atrophy, which is a different disease.  (See References)

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D) 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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E) prognosis is fair

This answer is incorrect.


Prognosis of Werdnig-Hoffman disease, or infantile spinal muscular atrophy is poor.  (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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