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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) It is a common cause of neonatal hypotonia
 
        B) It is also known as SMA type 2
 
        C) It is related to a defect on chromosome 10
 
        D) It is also known as SCA type 1
 
        E) The pathology involves the loss of upper and lower 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) 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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B) it is also known as SMA type 2

This answer is incorrect.


Werdnig-Hoffman disease is also called Spinal Muscular Atrophy type 1 (SMA-1).  (See References)

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C) it is related to a defect on chromosome 10

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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D) it is also known as SCA type 1

This answer is incorrect.


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

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E) 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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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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