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Pediatric Epilepsy Syndromes 03

Topic: Pediatric

Created on Tuesday, October 2 2007 by jdmiles

Last modified on Tuesday, October 2 2007.

An 8 year old girl presents to your office accompanied by her parents.  The parents state that on 2 occasions over the last several months, the girl has come into their room in the early morning, drooling and having difficulty talking, with a slight droop on the right side of her face.  These symptoms resolved fairly quickly on both occasions.  Then, this morning, she had a generalized tonic clonic seizure.  There is no significant past medical history, and no family history of epilepsy.  The patient had a normal birth history, and has consistently met her developmental milestones.  Physical exam is normal.  What is the most likely diagnosis?


 
        A) Landau-Kleffner syndrome
 
        B) Childhood epilepsy with occipital paroxysms
 
        C) BECTS
 
        D) Rasmussen syndrome
 
        E) Benign occipital epilepsy
 

 


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This question was created on October 02, 2007 by jdmiles.
This question was last modified on October 02, 2007.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) Landau-Kleffner syndrome

This answer is incorrect.


This child's history and physical exam are more typical of Benign Childhood Epilepsy with Centrotemporal Spikes (also called BECTS, or "Benign Rolandic Epilepsy").

  (See References)

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B) Childhood epilepsy with occipital paroxysms

This answer is incorrect.


This child's history and physical exam are more typical of Benign Childhood Epilepsy with Centrotemporal Spikes (also called BECTS, or "Benign Rolandic Epilepsy").

  (See References)

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C) BECTS

This answer is correct.


Benign Childhood Epilepsy with Centrotemporal Spikes (also called BECTS, or "Benign Rolandic Epilepsy") is the most common focal childhood epilepsy syndrome.  This child's presentation is fairly typical.  Onset of symptoms is between 4 and 12 years, most commonly at age 8 or 9.  Seizure types are simple partial motor seizures involving the face, which usually occur during sleep or when the child wakes up.  Children are usually brought to clinical attention after a generalized tonic clonic seizure, which represents the secondary generalization of one of these partial motor seizures. 

There is a family history of epilepsy in 40% of cases.

This syndrome is strongly associated with EEG findings of centrotemporal sharp waves.  These sharp waves have a maximal amplitude where the rolandic and sylvian fissures meet.    

Children with this syndrome are otherwise normal neurologically, and typically remit spontaneously.  Often, these seizures are not treated.  If treated, seizures are usually responsive to monotherapy with most antiepileptic agents. 

  (See References)

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D) Rasmussen syndrome

This answer is incorrect.


This child's history and physical exam are more typical of Benign Childhood Epilepsy with Centrotemporal Spikes (also called BECTS, or "Benign Rolandic Epilepsy").

  (See References)

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E) Benign occipital epilepsy

This answer is incorrect.


This child's history and physical exam are more typical of Benign Childhood Epilepsy with Centrotemporal Spikes (also called BECTS, or "Benign Rolandic Epilepsy").

  (See References)

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References:

1. Levin, K.H., and Luders, H.O. (Eds.) (2000). Comprehensive Clinical Neurophysiology. W.B. Saunders Company, Philadelphia.
2. Trescher, W.H., and Lesser, R.P. (2004). The epilepsies. In Bradley, W.G., Daroff, R.B., Fenichel, G.M., and Jankovic, J. (Eds.). Neurology in Clinical Practice, Fourth Edition. Butterworth Heinemann, Philadelphia, pp. 1953-1992.
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pediatric
Pediatric Epilepsy Syndromes 03
Question ID: 100207152
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 10/02/2007
Modified: 10/02/2007
Estimated Permutations: 257400

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