Neurocutaneous Syndromes 01

Topic: Adult

Created on Saturday, February 17 2007 by jdmiles

Last modified on Saturday, February 17 2007.

 
        A) Bilateral acoustic Schwannoma
 
        B) Paucity or absence of cutaneous lesions
 
        C) Shagreen patches
 
        D) Lisch nodules
 
        E) Facial angiofibromas
 

 

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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) bilateral acoustic Schwannoma

This answer is incorrect.

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

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B) 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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C) shagreen patches

This answer is incorrect.

Shagreen patches are not characteristic of NF1. They are associated with tuberous sclerosis.   (See References)

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D) Lisch nodules

This answer is correct.

Lisch nodules are small, whitish spots (hamartomas) in the iris of the eye. They are seen in most adults with NF1, and are pathognomonic.   (See References)

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E) facial angiofibromas

This answer is incorrect.

Facial angiofibromas are not characteristic of NF1. They are associated with tuberous sclerosis.   (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