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Neurocutaneous Syndromes 06

Topic: Imaging

Created on Saturday, September 22 2007 by jdmiles

Last modified on Saturday, September 22 2007.

This MRI is most consistent with which of the following diagnoses?


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

 


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This question was created on September 22, 2007 by jdmiles.
This question was last modified on September 22, 2007.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) Ehlers-Danlos syndrome

This answer is incorrect.


The MRI shows an optic glioma behind the left eye.  Optic gliomas are a common finding in neurofibromatosis type 1 (NF1).

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

  (See References)

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B) Neurofibromatosis type 1

This answer is correct.


The MRI shows an optic glioma behind the left eye.  Optic gliomas are a common finding in neurofibromatosis type 1 (NF1).

Other characteristic features of NF1 include cafe au lait spots, axillary freckling, palpable neurofibromas, Lisch nodules, and bone lesions.

  (See References)

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C) Tuberous sclerosis

This answer is incorrect.


The MRI shows an optic glioma behind the left eye.  Optic gliomas are a common finding in neurofibromatosis type 1 (NF1).

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

  (See References)

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D) Neurofibromatosis type 2

This answer is incorrect.


The MRI shows an optic glioma behind the left eye.  Optic gliomas are a common finding in neurofibromatosis type 1 (NF1).

Optic gliomas are not a common finding in neurofibromatosis type 2 (NF2).  Acoustic neuromas are more commonly associated with NF2.

  (See References)

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E) von Hippel-Lindau disease

This answer is incorrect.


The MRI shows an optic glioma behind the left eye.  Optic gliomas are a common finding in neurofibromatosis type 1 (NF1).

Von Hippel-Lindau disease (vHL) does not typically present with an optic glioma. 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 06
Question ID: 092207081
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 09/22/2007
Modified: 09/22/2007
Estimated Permutations: 120

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