Neurocutaneous Syndromes 05

Topic: Imaging

Created on Friday, September 21 2007 by jdmiles

Last modified on Friday, September 21 2007.

 
        A) Tuberous sclerosis
 
        B) Ehlers-Danlos syndrome
 
        C) Neurofibromatosis type 1
 
        D) Neurofibromatosis type 2
 
        E) Von Hippel-Lindau disease
 

 

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This question was created on September 21, 2007 by jdmiles.
This question was last modified on September 21, 2007.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS

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A) Tuberous sclerosis

This answer is incorrect.

The MRI shows bilateral acoustic neuromas. This is a classic feature of neurofibromatosis type 2 (NF2).

Tuberous sclerosis (TS) does not typically present with acoustic neuromas.  Radiographically visible lesions typically associated wtih TS include subependymal hamartomas and cortical tubers.

  (See References)

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B) Ehlers-Danlos syndrome

This answer is incorrect.

The MRI shows bilateral acoustic neuromas. This is a classic feature of neurofibromatosis type 2 (NF2).

Ehlers-Danlos syndrome (EDS) does not typically present with acoustic neuromas.  Radiographically visible lesions typically associated wtih EDS include large, sometimes multiple aneurysms.

  (See References)

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C) Neurofibromatosis type 1

This answer is incorrect.

The MRI shows bilateral acoustic neuromas. This is a classic feature of neurofibromatosis type 2 (NF2).

Neurofibromatosis type 1 (NF1, von Recklinghausen disease) does not typically present with acoustic neuromas. Typical findings in NF1 include cafe au lait spots, axillary freckling, palpable neurofibromas, Lisch nodules, optic glioma, and bone lesions.

  (See References)

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D) Neurofibromatosis type 2

This answer is correct.

The MRI shows bilateral acoustic neuromas. This is a classic feature of neurofibromatosis type 2 (NF2).  (See References)

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E) von Hippel-Lindau disease

This answer is incorrect.

The MRI shows bilateral acoustic neuromas. This is a classic feature of neurofibromatosis type 2 (NF2).

Von Hippel-Lindau disease (vHL) does not typically present with acoustic neuromas.  Radiographically visible lesions typically associated wtih vHL include hemangioblastomas, especially in the cerebellum and retina.

  (See References)

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References:

1. Rowland, L.P. (Ed) (2000). Merritt's Neurology, 10th Edition. Lippincott Williams & Wilkins, Philadelphia.
2. Fenichel, G.M. (2005). Clinical Pediatric Neurology, 5th ed. Elsevier, Philadelphia.
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, Fourth Edition. Butterworth Heinemann, Philadelphia, pp. 1867-1900.
4. Victor, M., and Ropper, A.H. (2001). Adams and Victor's Principles of Neurology, 7th Edition. McGraw-Hill, New York.
5. Prayson, R.A., and Goldblum, J.R. (Eds.) (2005). Neuropathology. Elsevier Churchill Livingstone, Philadelphia.

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imaging
Neurocutaneous Syndromes 05
Question ID: 092107023
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 09/21/2007
Modified: 09/21/2007
Estimated Permutations: 120