Neurocutaneous Syndromes 02

Topic: Adult

Created on Saturday, February 17 2007 by jdmiles

Last modified on Saturday, February 17 2007.

 
        A) Cafe au lait spots
 
        B) Retinal astrocytoma
 
        C) Bilateral acoustic Schwannoma
 
        D) Lisch nodules
 
        E) Plexiform neurofibromas
 

 

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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) cafe au lait spots

This answer is incorrect.

Skin changes, including cafe au lait spots, molluscum fobrosum (cutaneous tumors), and freckling in the axillae and groin, are typical of neurofibromatosis type 1 (NF1), but are not characteristic of NF2.  (See References)

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B) retinal astrocytoma

This answer is incorrect.

Retinal astrocytomas are not characteristic of NF2. They are associated with tuberous sclerosis.  (See References)

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C) bilateral acoustic Schwannoma

This answer is correct.

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

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D) Lisch nodules

This answer is incorrect.

Lisch nodules are small, whitish spots (hamartomas) in the iris of the eye. They are pathognomonic for neurofibromatosis type 1 (NF1), and are not seen in NF2.  (See References)

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E) plexiform neurofibromas

This answer is incorrect.

Plexiform neurofibromas are common in neurofibromatosis type 1 (NF1), but are not characteristic of 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 02
Question ID: 021707088
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 02/17/2007
Modified: 02/17/2007
Estimated Permutations: 491400