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Pediatric Epilepsy Syndromes 01

Topic: Pediatric

Created on Thursday, June 7 2007 by jdmiles

Last modified on Thursday, June 7 2007.

A 2 month old infant presents with tonic spasms and myoclonic seizures. EEG shows a burst-suppression pattern.

Which of the following pediatric epilepsy syndromes does this patient have?


 
        A) West Syndrome
 
        B) Lennox-Gastaut syndrome
 
        C) Early myoclonic encephalopathy
 
        D) Benign rolandic epilepsy
 
        E) Childhood absence epilepsy
 

 


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This question was created on June 07, 2007 by jdmiles.
This question was last modified on June 07, 2007.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) West Syndrome

This answer is incorrect.


From the history and EEG, this patient has an early epileptic encephalopathy with suppression bursts, either Ohtahara syndrome or early myoclonic encephalopathy. West Syndrome (WS) is associated with an EEG pattern of hypsarrhythmia, not burst-suppression. Age of onset for WS is typically between 3 and 7 months. Clinical features of LGS include mental retardation and infantile spasms. Children with Ohtahara syndrome may progress into West Syndrome and then into LGS; but this child would not as yet be diagnosed with WS.  (See References)

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B) Lennox-Gastaut syndrome

This answer is incorrect.


From the history and EEG, this patient has an early epileptic encephalopathy with suppression bursts, either Ohtahara syndrome or early myoclonic encephalopathy. Lennox-Gastaut syndrome (LGS) is associated with an EEG pattern of diffuse slow spike-wave complexes, not burst-suppression. Age of onset for LGS is typically between 1 and 8 years. Clinical features of LGS include mental retardation and multiple seizure types. Children with Ohtahara syndrome may progress into West Syndrome and then into LGS; but this child would not as yet be diagnosed with LGS.  (See References)

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C) early myoclonic encephalopathy

This answer is correct.


This patient has early myoclonic encephalopathy. This syndrome typically has an onset between birth and 3 months of age. Tonic spasms and myoclonic seizures are characteristic. EEG shows a burst-suppresion pattern.  (See References)

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D) benign rolandic epilepsy

This answer is incorrect.


From the history and EEG, this patient has an early epileptic encephalopathy with suppression bursts, either Ohtahara syndrome or early myoclonic encephalopathy. Benign rolandic epilepsy (BRE) is associated with an EEG pattern of centrotemporal sharp waves, not burst-suppression. Age of onset for BRE is typically between 4 and 12 years. Seizures associated with BRE include perioral sensory and motor seizures, speech arrest, and GTC, not tonic spasms.  (See References)

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E) childhood absence epilepsy

This answer is incorrect.


From the history and EEG, this patient has an early epileptic encephalopathy with suppression bursts, either Ohtahara syndrome or early myoclonic encephalopathy. Childhood absence epilepsy (CAE) is associated with an EEG pattern of diffuse 3 Hz spike-wave complexes, not burst-suppression. Age of onset for CAE is typically between 4 and 10 years. Children with CAE will have absence seizures, and may also have GTC and myoclonic seizures; the patient in question has tonic spasms and myoclonic seizures.  (See References)

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References:

1. Fenichel, G.M. (2005). Clinical Pediatric Neurology, 5th ed. Elsevier, Philadelphia.
2. Levin, K.H., and Luders, H.O. (Eds.) (2000). Comprehensive Clinical Neurophysiology. W.B. Saunders Company, Philadelphia.
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pediatric
Pediatric Epilepsy Syndromes 01
Question ID: 060707151
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 06/07/2007
Modified: 06/07/2007
Estimated Permutations: 1200

User Comments About This Question:

1 user entries
 

jdmiles
pediatric Pediatric Epilepsy Syndromes Jun 07, 2007 @ 15:30

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