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Ceroid Lipofuscinoses 01

Topic: Pediatric

Created on Thursday, October 18 2007 by jdmiles

Last modified on Thursday, October 18 2007.

What are the cardinal features of ceroid lipofuscinosis?

 
        A) Intracellular accumulation of autofluorescent material, progressive ataxia, cognitive and/or motor regression, and peripheral neuropathy
 
        B) Intracellular accumulation of autofluorescent material, epilepsy, progressive deafness, and cognitive and/or motor regression
 
        C) Progressive deafness, cardiac malformations, intracellular accumulation of autofluorescent material, and ataxia
 
        D) Intracellular accumulation of autofluorescent material, progressive blindness, cognitive and/or motor regression, and epilepsy
 
        E) Intracellular accumulation of non-fluorescent material, cognitive and/or motor regression, epilepsy, and progressive blindness
 

 


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This question was created on October 18, 2007 by jdmiles.
This question was last modified on October 18, 2007.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) intracellular accumulation of autofluorescent material, progressive ataxia, cognitive and/or motor regression, and peripheral neuropathy

This answer is incorrect.


Typical features that are shared among the neuronal ceroid lipofuscinoses (also called Batten disease) are progressive blindness, congitive/motor regression, epilepsy, and intracellular accumulation of autofluorescent material.

Not all patients present with all of these features.

  (See References)

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B) intracellular accumulation of autofluorescent material, epilepsy, progressive deafness, and cognitive and/or motor regression

This answer is incorrect.


Typical features that are shared among the neuronal ceroid lipofuscinoses (also called Batten disease) are progressive blindness, congitive/motor regression, epilepsy, and intracellular accumulation of autofluorescent material.

Not all patients present with all of these features.

  (See References)

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C) progressive deafness, cardiac malformations, intracellular accumulation of autofluorescent material, and ataxia

This answer is incorrect.


Typical features that are shared among the neuronal ceroid lipofucsinoses (also called Batten disease) are progressive blindness, congitive/motor regression, epilepsy, and intracellular accumulation of autofluorescent material.

Not all patients present with all of these features.

  (See References)

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D) intracellular accumulation of autofluorescent material, progressive blindness, cognitive and/or motor regression, and epilepsy

This answer is correct.


Typical features that are shared among the neuronal ceroid lipofuscinoses (also called Batten disease) are progressive blindness, congitive/motor regression, epilepsy, and intracellular accumulation of autofluorescent material.

Not all patients present with all of these features.

  (See References)

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E) intracellular accumulation of non-fluorescent material, cognitive and/or motor regression, epilepsy, and progressive blindness

This answer is incorrect.


Typical features that are shared among the neuronal ceroid lipofuscinoses (also called Batten disease) are progressive blindness, congitive/motor regression, epilepsy, and intracellular accumulation of autofluorescent material.

Not all patients present with all of these features.

  (See References)

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References:

1. Mitchison, H.M., and Mole, S.E. (2001). "Neurodegenerative disease: the neuronal ceroid lipofuscinoses (Batten disease)." Curr Opin Neurol, 14(6) 795-803. (PMID:11723391)
2. Wisniewski, K.E., Zhong, N., and Philippart, M. (2001). "Pheno/genotypic correlations of neuronal ceroid lipofuscinoses." Neurology, 57(4) 576-81. (PMID:11548735)
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pediatric
Ceroid Lipofuscinoses 01
Question ID: 101807115
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 10/18/2007
Modified: 10/18/2007
Estimated Permutations: 1800

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