Hereditary Motor and Sensory Neuropathies 02

Topic: Pediatric

Created on Sunday, February 17 2008 by jdmiles

Last modified on Sunday, February 17 2008.

 
        A) 1
 
        B) 8
 
        C) 3
 
        D) 12
 
        E) 2
 

 

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This question was created on February 17, 2008 by jdmiles.
This question was last modified on February 17, 2008.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS

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A) 1

This answer is correct.

Charcot Marie Tooth (CMT) type 1 (including subtypes A-D) is a demyelinating inherited sensorimotor polyneuropathy. Nerve conduction velocities are slow on NCS. It has autosomal dominant inheritance. CMT1A is associated with the PMP22 gene on chromosome 17, and is the most common form of inherited neuropathy. CMT1B is associated with the MPZ gene on chromosome 1. CMT1C is associated with the LITAF gene on chromosome 16. CMT1D is associated with the EGR2 gene on chromosome 10.  (See References)

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B) 8

This answer is incorrect.

Charcot Marie Tooth (CMT) type 2E is a primarily axonal sensorimotor polyneuropathy. NCS would show normal or near-normal conduction velocities. CMT2E is associated with the NEFL gene on chromosome 8.  (See References)

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C) 3

This answer is incorrect.

Charcot Marie Tooth (CMT) type 2B is a primarily axonal sensorimotor polyneuropathy. NCS would show normal or near-normal conduction velocities. CMT2B is associated with the RAB7 gene on chromosome 3.  (See References)

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D) 12

This answer is incorrect.

Charcot Marie Tooth (CMT) type 2C is a primarily axonal sensorimotor polyneuropathy. NCS would show normal or near-normal conduction velocities. CMT2C is associated with chromosome 12.  (See References)

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E) 2

This answer is incorrect.

Charcot Marie Tooth (CMT) type 1 (including subtypes A-D) is a demyelinating inherited sensorimotor polyneuropathy. Nerve conduction velocities are slow on NCS. It has autosomal dominant inheritance. CMT1A is associated with the PMP22 gene on chromosome 17, and is the most common form of inherited neuropathy. CMT1B is associated with the MPZ gene on chromosome 1. CMT1C is associated with the LITAF gene on chromosome 16. CMT1D is associated with the EGR2 gene on chromosome 10.  (See References)

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References:

1. Fenichel, G.M. (2005). Clinical Pediatric Neurology, 5th ed. Elsevier, Philadelphia. (ISBN:1416001697)	Advertising:
2. Bosch, E.P., and Smith, B.E. (2004). Disorders of peripheral nerves. In Bradley, W.G., Daroff, R.B., Fenichel, G.M., and Jankovic, J. (Eds.). Neurology in Clinical Practice, Fourth Edition. Butterworth Heinemann, Philadelphia, pp. 2299-2401 (ISBN:0750674695).	Advertising:
3. Bashar Katirji. Electromyography in clinical practice: a case study approach. St. Louis, Mo. : Mosby, c2006. (ISBN:9780323028998)	Advertising:

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pediatric
Hereditary Motor and Sensory Neuropathies 02
Question ID: 021708139
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 02/17/2008
Modified: 02/17/2008
Estimated Permutations: 2400