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Hereditary Neuropathy with Liability to Pressure Palsy 01

Topic: Adult

Created on Thursday, April 12 2007 by jdmiles

Last modified on Thursday, April 12 2007.

Which of the following statements about hereditary neuropathy with liability to pressure palsy (HNPP) is true?

 
        A) HNPP is associated with chromosomal location 1p35-36.
 
        B) HNPP has a non-Mendelian inheritance pattern
 
        C) HNPP is associated with chromosomal location 4q12.
 
        D) HNPP is associated with peripheral myelin protein 22 (PMP-22)
 
        E) HNPP shows an axonal loss pattern on electrodiagnostic studies.
 

 


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This question was created on April 12, 2007 by jdmiles.
This question was last modified on April 12, 2007.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) HNPP is associated with chromosomal location 1p35-36.

This answer is incorrect.


HNPP is associated with chromosomal location 17p11.2-12. Charcot-Marie-Tooth (CMT-2A) is associated with chromosomal location 1p35-36.  (See References)

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B) HNPP has a non-Mendelian inheritance pattern

This answer is incorrect.


HNPP is a hereditary disease with an autosomal dominant inheritance pattern.  (See References)

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C) HNPP is associated with chromosomal location 4q12.

This answer is incorrect.


HNPP is associated with chromosomal location 17p11.2-12. Muscular dystrophy is associated with chromosomal location 4q12.  (See References)

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D) HNPP is associated with peripheral myelin protein 22 (PMP-22)

This answer is correct.


HNPP is associated with peripheral myelin protein 22 (PMP-22).  (See References)

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E) HNPP shows an axonal loss pattern on electrodiagnostic studies.

This answer is incorrect.


HNPP shows a demyelinating pattern on electrodiagnostic studies.  (See References)

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References:

1. Bird, T.D., and Tapscott, S.J. (2004). Clinical neurogenetics. In Bradley, W.G., Daroff, R.B., Fenichel, G.M., and Jankovic, J. (Eds.). Neurology in Clinical Practice, Fourth Edition. Butterworth Heinemann, Philadelphia, pp. 781-808.
2. Horowitz, S.H., Spollen, L.E., and Yu, W. (2004). Hereditary neuropathy with liability to pressure palsy: fulminant development with axonal loss during military training. Journal of Neurology Neurosurgery and Psychiatry, 75, Pp. 1629-1631.
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adult
Hereditary Neuropathy with Liability to Pressure Palsy 01
Question ID: 041207086
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 04/12/2007
Modified: 04/12/2007
Estimated Permutations: 60480

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