Abstracts

Rationale: We present a case of a 50 year old male with an extensive malformation of cortical development, predominantly affecting the right side and a hypothalamic hamartoma, discovered incidentally at the age of 43, following neurimaging after a motor vehicle accident. In the same year, he was found to have global hormonal insufficiency. Prior to this point he had never reported seizures. He became actively involved in a support organization for patients with brain tumors and subsequently developed multiple seizure types including one form that involved uncontrolled spells of laughter. Methods: The patient, a 50 year old male with normal IQ, was admitted for video-EEG monitoring as a prelude to possible epilepsy surgery. He was accompanied at admission by a large stuffed animal which he kept in his bed throughout monitoring. He was monitored for 4 days during which 5 of his typical seizure-like events were captured Results: Interictal EEG was normal. The seizure-like events started with his reporting right-sided tingling, followed by stereotyped non-clonic, right-sided hand movements, humming noises, calling out 'Mama' interspersed with repetitive spoken "ha-ha-ha" sounds, while inconsistently obeying commands to raise his arms or repeat words. After some of the episodes there was post-ictal weeping while clutching his stuffed animal. Surface EEG remained completely normal throughout these events. One event was triggered by hyperventilation and events ranged in duration from 2 to 8 minutes. Conclusions: The phenomenon of gelastic seizures in the setting of hypothalamic hamartoma is a classic presentation in neurology. It is not clear however, how frequent the finding is within the population of patients with hypothalamic hamartomas and it does not ocurr in all cases. Given the impressive neuroradiological findings in this case, the immediate clinical bias was to assume that the seizures were true gelastic seizures and to proceed with surgical work-up on this basis. Careful attention to the history revealed that seizures, including gelastic seizures, started only after the patient was alerted to the presence of the hamartoma and became aware of its associations. A weakness of this study is the fact that there was no depth recording from the hamartoma itself, however the clinical presentation of the events included numerous clues to a non-epileptic basis, a conclusion supported by a significant reduction in frequency of the laughing episodes after counseling, despite reduction in doses of anti-epileptic medications. Another feature of interest in this case is that the presence of a stuffed animal, accompanying an adult with normal IQ to the Epilepsy Monitoring Unit was a sensitive indicator of non-epileptic seizures.