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Pediatric Epilepsy Syndromes 11

Topic: Pediatric

Created on Sunday, March 9 2008 by jdmiles

Last modified on Sunday, March 9 2008.

A 9 year-old girl presents to your office accompanied by her parents. The parents state that on 2 occasions over the past several months, the girl has come into their room in the early morning, drooling and having difficulty talking, with a slight facial droop. These symptoms resolved fairly quickly on both occasions. Then, this morning, she had a generalized motor 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. An EEG is obtained, and is shown above. Which of the following EEGs is most characteristic of this patient's syndrome?

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This question was created on March 09, 2008 by jdmiles.
This question was last modified on March 09, 2008.

ANSWERS AND EXPLANATIONS

A)

This answer is incorrect.

This child's history is very typical of Benign Childhood Epilepsy with Centrotemporal Spikes (also called BECTS, or "Benign Rolandic Epilepsy"). 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. The EEG you selected is a normal EEG with no epileptiform discharges. (See References)


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B)

This answer is incorrect.

This child's history is very typical of Benign Childhood Epilepsy with Centrotemporal Spikes (also called BECTS, or "Benign Rolandic Epilepsy"). 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. The EEG you selected shows slowing in a hyperventilating child. This is a normal finding. (See References)


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C)

This answer is correct.

This child's history is very typical of Benign Childhood Epilepsy with Centrotemporal Spikes (also called BECTS, or "Benign Rolandic Epilepsy"), the most common focal childhood epilepsy syndrome. 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)

Courtesy of Wikimedia Commons

This answer is incorrect.

This child's history is very typical of Benign Childhood Epilepsy with Centrotemporal Spikes (also called BECTS, or "Benign Rolandic Epilepsy"). 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. The EEG you selected shows a 3 Hz spike and wave pattern, which is characteristic of absence epilepsy syndromes. (See References)


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E)

This answer is incorrect.

This child's history is very typical of Benign Childhood Epilepsy with Centrotemporal Spikes (also called BECTS, or "Benign Rolandic Epilepsy"). 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. The EEG you selected shows a normal EEG obtained during sleep. There is no epileptiform activity in this EEG. (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.
3. Floyd, T. (1999). "Bear-inflicted human injury and fatality." Wilderness Environ Med, 10(2) 75-87. (PMID:10442155)


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pediatric
Pediatric Epilepsy Syndromes 11
Question ID: 030908111
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 03/09/2008
Modified: 03/09/2008
Estimated Permutations: 8400