Werdnig-Hoffman Disease

Topic: Pediatric

Created on Sunday, September 28 2008 by jdmiles

Last modified on Sunday, September 28 2008.

 
        A) The pathology involves the loss of upper and lower motor neurons
 
        B) The pathology involves the loss of motor neurons
 
        C) It is related to a defect on chromosome 4
 
        D) The pathology involves the loss of sensory neurons
 
        E) It is an inherited disorder, with an autosomal dominant 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

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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) the pathology involves the loss of motor neurons

This answer is correct.

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

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C) it is related to a defect on chromosome 4

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) the pathology involves the loss of sensory neurons

This answer is incorrect.

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

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E) it is an inherited disorder, with an autosomal dominant 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