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396 Frontal lobe epilepsy with gelastic seizures
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  1. Sanja Delin
  1. General Hospital Zadar, Department of Pediatrics, Zadar, School of Medicine, J. J. Strossmayer University in Osijek, Osijek, Croatia

Abstract

Gelastic epilepsy are an uncommon seizure type most often symptomatic associated with hypothalamic harmatomas, with a prevalence rate of about 0,5 per 100 000. However, idiopathic and cryptogenic cases with no evidence of cortical structural lesions have also been described. The term gelastic comes from Greek word gelos meaning laughter. Laughter is pathological in nature and can be spontaneus without obvious cause.

We present a 13-year-old girl without structural lesions, manifesting gelastic seizures. The seizures were not associated with feelings of mirth.

Repeated 1,5T MRI revealed no structural abnormality. Interictal EEG showed paroxysms in the right frontal region. Ictal EEG demonstrated diffuse attenuation,followed by fast activities and spike-wave complexes predominantly over the right hemisphere. Unlike the seizure from temporal lobe, semiological investigations revealed that the laughter in our case was not accompanied by a subjective feeling of mirth, and an interictal EEG showed frontal spikes. Results of neurological examination were normal. The Wechsler intelligence scale for children IV(WISC-IV) revealed a mild mental retardation with the intelligence quotient score<70. An interictal FDG-PET CT showed hypometabolisam over the right superior frontal lobe and right medial temporal lobe. The seizures were resistant to oxcarbazepine, levetiracetam, valproate and lamotrigine and were suppressed by topiramate monotherapy. Further clinical examinations (high-resolution 3T MRI, ictal and interictal SPECT) will be done.

In conclusion, gelastic seizures are commonly associated with hypothalamic hamartomas but ictal laughing, has been reported with temporal lobe and frontal lobe seizures. Although gelastic seizures have been described as intractable, a few medications including valproic acid, lamotrigine, levetiracetam and steroids were reported to be effective in patients without hypothalamic hamartoma. In ours patient topiramate monotherapy proved to be effective in the treatement of gelastic seizures without hypothalamic hamartoma.

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