Neurocutaneous Syndromes 01

Topic: Adult

Created on Saturday, February 17 2007 by jdmiles

Last modified on Saturday, February 17 2007.

 
        A) Genetic abnormality on chromosome 22
 
        B) Malignant transformation of tumors
 
        C) Paucity or absence of cutaneous lesions
 
        D) Poliosis
 
        E) Bilateral acoustic Schwannoma
 

 

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This question was created on February 17, 2007 by jdmiles.
This question was last modified on February 17, 2007.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS

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A) genetic abnormality on chromosome 22

This answer is incorrect.

The genetic defect in NF1 is on chromosome 17. A defect on chromosome 22 is associated with neurofibromatosis type 2 (NF2).   (See References)

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B) malignant transformation of tumors

This answer is correct.

Patients with NF1 and plexiform neurofibromas have a lifetime risk of about 5% for malignant transformaiton.   (See References)

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C) paucity or absence of cutaneous lesions

This answer is incorrect.

Cutaneous lesions are characteristic of NF1. Lesions include cafe au lait spots, molluscum fobrosum (cutaneous tumors), and freckling in the axillae and groin. A paucity or absence of cutaneous lesions is typical of neurofibromatosis type 2 (NF2).   (See References)

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D) poliosis

This answer is incorrect.

Poliosis (presence of a small patch of white hair) is not characteristic of NF1. It is associated with tuberous sclerosis.   (See References)

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E) bilateral acoustic Schwannoma

This answer is incorrect.

Bilateral acoustic Schwannoma is associated with neurofibromatosis type 2 (NF2).   (See References)

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References:

1. Neurofibromatosis Fact Sheet, NINDS. NIH Publication No. 06-2126
2. Victor, M., and Ropper, A.H. (2001). Adams and Victor's Principles of Neurology, 7th Edition. McGraw-Hill, New York. Pp. 1069-1076.
3. Santos, C.C., Miller, V.S., and Roach, E.S. (2004). Neurocutaneous syndromes. In Bradley, W.G., Daroff, R.B., Fenichel, G.M., and Jankovic, J. (Eds.). Neurology in Clinical Practice, 4th Edition. Butterworth Heinemann, Philadelphia. Pp. 1867-1900.

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adult
Neurocutaneous Syndromes 01
Question ID: 021707087
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 02/17/2007
Modified: 02/17/2007
Estimated Permutations: 84000