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The Causes of Epilepsy The Causes of Epilepsy
Common and Uncommon Causes in Adults and Children
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Chapter 64 - Hypothalamic hamartoma and gelastic epilepsy

from Section 3 - Symptomatic epilepsy

Published online by Cambridge University Press:  05 March 2012

Simon D. Shorvon
Affiliation:
National Hospital for Neurology and Neurosurgery, London
Frederick Andermann
Affiliation:
Montreal Neurological Hospital and Institute
Renzo Guerrini
Affiliation:
Child Neurology Unit, Meyer Pediatric Hospital, Florence
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Summary

Hypothalamic hamartomas (HH) are an uncommon human pathology resulting in a distinctive and often severe epilepsy syndrome, usually including gelastic (laughing) seizures. This chapter focus on HH associated with epilepsy. The gross anatomy of HH lesions determines the nature of their clinical symptoms. Epilepsy associated with HH is a progressive disease for the majority of patients, with development of multiple seizure types, and coincident deterioration in cognition and behavior. Antiepilepsy drugs (AEDs) are unsuccessful in managing seizures associated with HH, and eradication or complete disconnection of the HH is successful for controlling seizures, and may help ameliorate the comorbid problems with cognition and behavior. Treatment choice is guided by the individual circumstances of the case, including the clinical course of the disease and an assessment of the size and attachment of the HH.
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The Causes of Epilepsy
Common and Uncommon Causes in Adults and Children
, pp. 449 - 453
Publisher: Cambridge University Press
Print publication year: 2011

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