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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 also known as SCA type 2
 
        B) The pathology involves the loss of motor and sensory neurons
 
        C) It is not an inherited disorder
 
        D) Initial presentation may include arthrogryposis
 
        E) It is an inherited disorder, with an X-linked inheritance pattern.
 

 


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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 also known as SCA type 2

This answer is incorrect.


Werdnig-Hoffman disease, or infantile spinal muscular atrophy,is also known as SMA-1. SCA type 2 refers to Spino-cerebellar atrophy, which is a different disease.  (See References)

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B) 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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C) it is not an inherited disorder

This answer is incorrect.


Werdnig-Hoffman disease is an inherited disorder, with an autosomal recessive inheritance pattern.  (See References)

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D) initial presentation may include arthrogryposis

This answer is correct.


Children with Werdnig-Hoffman disease may be born with arthrogrypotic deformities.  (See References)

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E) it is an inherited disorder, with an X-linked inheritance pattern.

This answer is incorrect.


Werdnig-Hoffman disease is an inherited disorder, with an autosomal recessive inheritance pattern.  (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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