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Acanthocytes 02

Topic: Pediatric

Created on Monday, November 10 2008 by jdmiles

Last modified on Monday, November 10 2008.

A patient presents with neuropathy, steatorrhea, and ataxia. Acanthocytes are found on the blood smear.
This is most consistent with which one of the following diagnoses?

 
        A) Hypobetalipoproteinemia
 
        B) Fabry disease
 
        C) Levine-Critchley syndrome
 
        D) Huntington disease
 
        E) Refsum disease
 

 


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This question was created on November 10, 2008 by jdmiles.
This question was last modified on November 10, 2008.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) hypobetalipoproteinemia

This answer is correct.


Hypobetalipoproteinemia is a disorder similar in presentation to abetalipoproteinemia or Bassen-Kornzweig syndrome. Both are genetic diseases with neurologic findings. They are characterized by acanthocytosis, neuropathy and ataxia.  (See References)

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B) Fabry disease

This answer is incorrect.


Acanthocytosis is not a characteristic feature of this disorder.  (See References)

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C) Levine-Critchley syndrome

This answer is incorrect.


Neuroacanthocytosis, also called Levine-Critchley syndrome or chorea-acanthocytosis, is a multisystem neurodegenerative disorder.
Characteristic features of this disorder are the presence of acanthocytes in the blood, and a hyperkinetic movement disorder. Neuropathy, ataxia, and steatorrhea with acanthocytosis should make you think of abetalipoproteinemia or hypobetalipoproteinemia.  (See References)

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D) Huntington disease

This answer is incorrect.


Acanthocytosis is not a characteristic feature of this disorder.  (See References)

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E) Refsum disease

This answer is incorrect.


Acanthocytosis is not a characteristic feature of this disorder.  (See References)

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References:

1. Rowland, L.P. (Ed) (2000). Merritt's Neurology, 10th Edition. Lippincott Williams & Wilkins, Philadelphia. (ISBN:0683304747) Advertising:
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pediatric
Acanthocytes 02
Question ID: 111008036
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 11/10/2008
Modified: 11/10/2008
Estimated Permutations: 360

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