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Hereditary Motor and Sensory Neuropathies 01

Topic: Pediatric

Created on Saturday, February 24 2007 by jdmiles

Last modified on Saturday, February 24 2007.

You examine a child with pes cavus, diminished ankle reflexes, scoliosis, and weakness with wasting of the calf muscles. On nerve conduction study, you find severe slowing of the conduction velocity. Of the following, which of the following syndromes is the most appropriate diagnosis?

A) Charcot Marie Tooth type 2C

B) Charcot Marie Tooth type 2A

C) Charcot Marie Tooth type 4A

D) Charcot Marie Tooth type 2B

E) Charcot Marie Tooth type 2D

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This question was created on February 24, 2007 by jdmiles.
This question was last modified on February 24, 2007.

ANSWERS AND EXPLANATIONS

A) Charcot Marie Tooth type 2C

This answer is incorrect.

Charcot Marie Tooth type 2C (Dejerine-Sottas syndrome) is a primarily axonal sensorimotor polyneuropathy. NCS would show normal or near-normal conduction velocities. Inheritance is autosomal dominant. (See References)


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B) Charcot Marie Tooth type 2A

This answer is incorrect.

Charcot Marie Tooth type 2A (Dejerine-Sottas syndrome) is a primarily axonal sensorimotor polyneuropathy. NCS would show normal or near-normal conduction velocities. Inheritance is autosomal dominant. (See References)


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C) Charcot Marie Tooth type 4A

This answer is correct.

Charcot Marie Tooth type 4A (Dejerine-Sottas syndrome) is a demyelinating inherited sensorimotor polyneuropathy. Nerve conduction velocities are slow on NCS. It has autosomal recessive inheritance. CSF protein is normal in this disorder. (See References)


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D) Charcot Marie Tooth type 2B

This answer is incorrect.

Charcot Marie Tooth type 2B (Dejerine-Sottas syndrome) is a primarily axonal sensorimotor polyneuropathy. NCS would show normal or near-normal conduction velocities. Inheritance is autosomal dominant. (See References)


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E) Charcot Marie Tooth type 2D

This answer is incorrect.

Charcot Marie Tooth type 2D (Dejerine-Sottas syndrome) is a primarily axonal sensorimotor polyneuropathy. NCS would show normal or near-normal conduction velocities. Inheritance is autosomal dominant. (See References)


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References:

1. Fenichel, G.M. (2005). Clinical Pediatric Neurology, 5th ed. Elsevier, Philadelphia.
2. Bosch, E.P., 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, 4th Edition. Butterworth Heinemann, Philadelphia. Pp. 2299-2401.


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pediatric
Hereditary Motor and Sensory Neuropathies 01
Question ID: 022407165
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 02/24/2007
Modified: 02/24/2007
Estimated Permutations: 33600