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Gelastic seizures and hypothalamic hamartomas

Evaluation of patients undergoing chronic intracranial EEG monitoring and outcome of surgical treatment

Departments of Neurology (Drs. Cascino and Sharbrough) and Neurosurgery (Dr. Kelly), Mayo Clinic and Mayo Foundation, Rochester, MN; the Department of Neurology and Neurosurgery (Drs. Andermann, Olivier, and Feindel), McGill University, Montreal Neurological Institute and Hospital, Montreal, PQ, Canada; the Department of Neurology (Drs. Berkovic and Bladin), Austin Hospital, Melbourne, Australia; the Department of Neurology (Dr. Kuzniecky), University of Alabama at Birmingham, Birmingham, AL; and the Department of Neurology (Dr. Keene), Children's Hospital of Eastern Ontario, Ottawa, ON, Canada.

We retrospectively studied 12 consecutive patients with gelastic seizures and hypothalamic hamartomas who, because of intractable epilepsy, underwent chronic intracranial EEG monitoring or epilepsy surgery. All patients had medically refractory seizures that included laughter as an ictal behavior (gelastic seizures). The hypothalamic hamartomas were identified with neuroimaging studies (12 of 12) and by pathologic verification (four of 12). Associated clinical features included behavioral disorders (n = 5), developmental delay (n = 4), and precocious puberty (n = 2). Interictal extracranial EEG predominantly showed bihemispheric epileptiform changes suggesting a secondary generalized epileptic disorder. Intracranial EEG recordings, performed in eight patients, indicated the apparent focal onset of seizure activity (anterior temporal lobe [n = 7] and frontal lobe [n = 1]). None of the seven patients who underwent a focal cortical resection, however, experienced a significant reduction in seizure tendency. An anterior corpus callosotomy, performed in two patients with symptomatic generalized epilepsy, resulted in a worthwhile reduction in drop attacks. Results of this study may modify the surgical strategies in patients with gelastic seizures and hypothalamic hamartomas.

Presented in part at the annual meeting of the American Epilepsy Society, Philadelphia, PA, December 1991.

Address correspondence and reprint requests to Dr. G.D. Cascino, Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905.

Received May 27,1992. Accepted for publication in final form August 12,1992.

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