Gelastic seizures

Elizabeth E Gerard MD (Dr. Gerard of the Comprehensive Epilepsy Center at Northwestern University is principal investigator for a trial funded by SAGE Pharmaceuticals.)
Lawrence J Hirsch MD (Dr. Hirsch of Yale University School of Medicine received consultation fees from Ceribell, Marinus, Monteris, Neuropace, Sun Neuroscience, Sunovion, and Upsher Smith; research support from Lundbeck, Acorda, Upsher Smith, Eisai, and Sunovion; and honoraria for speaking from Neurospace.)
Jerome Engel Jr MD PhD, editor. (Dr. Engel of the David Geffen School of Medicine at the University of California, Los Angeles, has no relevant financial relationships to disclose.)
Originally released May 7, 2001; last updated August 6, 2015; expires August 6, 2018

This article includes discussion of gelastic seizures, gelastic epilepsy, hypothalamic hamartoma, and focal epilepsy. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Advances in imaging and surgical techniques have improved the recognition, treatment, and understanding of gelastic seizures due to hypothalamic hamartomas. It is now clear that in many cases the hamartoma is critical in generating the laughing seizures. Ictal laughter, however, can also occur in the absence of a hamartoma and is typically associated with frontal or temporal lobe epilepsy. The authors review the clinical presentation and differential diagnosis of paroxysmal laughter and comment on what the various etiologies may be able to tell us about the neuroanatomy of laughter. New and promising therapies such as laser thermal ablation are also reviewed.

Key points

 

• Gelastic seizures are characterized by recurrent bouts of unprovoked and stereotyped laughter.

 

• The differential diagnosis for gelastic seizures includes pseudobulbar laughter, gelastic cataplexy, and gelastic syncope.

 

• Gelastic seizures are classically associated with hypothalamic hamartoma but may also be secondary to focal pathology in the frontal or temporal lobes, and rarely the parietal lobe.

 

• When they are associated with hypothalamic hamartomas, gelastic seizures are typically intractable to medical therapy and may be 1 of the first manifestations of a syndrome characterized by multiple seizure types, precocious puberty, and an epileptic encephalopathy.

 

• The most effective treatments for gelastic seizures associated with hypothalamic hamartoma involve removal or ablation of the hamartoma.

Historical note and terminology

Daly and Mulder coined the term "gelastic epilepsy" from the Greek word gelos, meaning “laughter,” to emphasize the main character of these seizures (Daly and Mulder 1957). The possibility of sudden emotions as a manifestation of an epileptic seizure had been recognized since the end of the 19th century (Sher and Brown 1976). These emotions were usually characterized as unpleasant. Emotions of fear were most often described. Laughing seizures were first described by Trousseau (Trousseau 1877). Gowers observed emotions "with a cheerful character" as part of a seizure (Gowers 1881). Gascon and Lombroso suggested the following criteria for the diagnosis of gelastic epilepsy: stereotyped recurrence; absence of external precipitants; concomitance of other manifestations generally accepted as epileptic; presence of interictal or ictal epileptiform discharges on EEG; and absence of conditions in which pathologic laughter might occur (Gascon and Lombroso 1971). Arguments were raised against the concept of gelastic seizures as an epileptic syndrome because the definition of the syndrome was based on a single symptom (Loiseau et al 1971). Currently, the ILAE classifies gelastic seizures as a type of focal seizure, which may be a component of various epilepsy syndromes (Engel 2001; Engel 2006). The updated ILAE proposal recognizes gelastic seizures associated with hypothalamic hamartomas as a specific epilepsy constellation (Berg et al 2010).

Gelastic seizures are classically associated with hypothalamic hamartomas, but ictal laughing has also been described in different epileptic conditions associated with the temporal or frontal lobes.

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