This Article Full Text (OnlineFirst PDF) All Versions of this Article: 0883073807308703v1 23/2/235    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Saved Citations Download to citation manager Request Permissions Request Reprints Add to My Marked Citations Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Citing Articles via Scopus Google Scholar Articles by Shahar, E. Articles by Keidar, Z. Search for Related Content PubMed PubMed Citation Articles by Shahar, E. Articles by Keidar, Z. Pubmed/NCBI databases Compound via MeSH Substance via MeSH Hazardous Substances DB CLONAZEPAM Medline Plus Health Information Seizures Social Bookmarking                         What's this?

Intractable Gelastic Seizures During Infancy: Ictal Positron Emission Tomography (PET) Demonstrating Epileptiform Activity Within the Hypothalamic Hamartoma

¹ Meyer Children Hospital, Rambam Medical Center, Haifa, Israel
² Institute of Nuclear Medicine, Rambam Medical Center, Haifa, Israel

^* To whom correspondence should be addressed. E-mail: e_shahar{at}rambam.health.gov.il.

	Abstract

Gelastic seizures comprise a very rare form of epilepsy. They present with recurrent bursts of laughter voices without mirth and are most commonly associated with the evolution of a hypothalamic hamartoma. The purpose of this article is to describe the second reported ictal fluorodeoxyglucose-positron emission tomography study in a unique case of an infant with intractable gelastic seizures since the neonatal period associated with a hypothalamic hamartoma. The patient presented at 4 months old with recurrent, almost persistent, gelastic seizures consisting of laughter bouts without mirth. The seizures were noticeable at the first week of life and increased in frequency to last up to 12 hours, namely status gelasticus. These gelastic fits were accompanied with focal motor seizures, including unilateral right-eye blinking and mouth twitching. Developmental milestones were intact for age. Magnetic resonance imaging of the cortex demonstrated a large hypothalamic hamartoma within the third ventricle, hampering cerebrovascular fluid drainage of the lateral ventricles. An electroencephalography was nondiagnostic. Ictal fluorodeoxyglucose-positron emission tomography demonstrated a large circumscribed hypermetabolic region within the location of the hypothalamic hamartoma, representing localized intense epileptiform activity. The infant became instantly free of all seizure types given minute doses of oral benzodiazepine (clonazepam) and remains completely controlled after 12 months. Her overall development remains intact. This ictal fluorodeoxyglucose-positron emission tomography is the second reported study verifying that the main source of the epileptic activity inducing gelastic seizures originates from the hypothalamic hamartoma itself; therefore, a complementary fluorodeoxyglucose-positron emission tomography study should be considered in any patient presenting with intractable gelastic seizures, especially in those associated with hypothalamic hamartoma, in order to localize the region of epileptiform activity amenable to surgical resection if intensive drug therapy fails.

First published on December 26, 2007, doi:10.1177/0883073807308703

Journal of Child Neurology 2008;23:235.

A more recent version of this article appeared on February 1, 2008


CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter    What's this?

This article has been cited by other articles:

				Y.-t. Ng Correct Definition and Reference of the Term "Status Gelasticus" J Child Neurol, September 1, 2008; 23(9): 1088 - 1088. [PDF]