Gelastic seizures and fever originating from a parietal cortical dysplasia
A 5-year-old boy was referred to our department for recurrent fever and uncontrolled epilepsy. The patient was the youngest one in a family of five children born out of a consanguineous marriage, but without any other significant familial history. He was born at full-term without any perinatal problem, and weighed 3,400g.\nThe neuro-psychomotor development of the patient was globally normal. He walked at the age of 13 months and acquired verbal language at the age of 18 months and his first seizures occurred by the age of 14 months. These attacks were characterized by generalized tonic clonic seizures with a frequency of 1–2 times per month. He was initially treated using phenobarbital then Valproate without any significant improvement. By his 4th year, the patient started expressing daily laughing attacks that were occurring 2–3 times a day while each was lasting for about a minute. This stereotypical laugh was unrelated to the context; the patient was generally unconscious during attacks and experienced post-ictal amnesia. Most often, an undescribed feeling occurred prior to seizure associated with left hemi-corporeal paresthesia. These seizures were associated to episodes of fever up to 39.5°C; these episodes occurred almost everyday and resolved spontaneously in less than 3 h. The fever rose with the start of gelastic attack and lasted 2–3 hours and sometimes occurred independently.\nThe physical and neurological evaluation of the patient was essentially normal. He was 104 cm tall (50th percentiles), weighting 17 kg (50th percentiles) with 50th percentiles head circumference.\nComplete paraclinical investigations were performed, this included C-reactive protein, sedimentation rate, leukocyte count, urinalysis, lumbar puncture, thorax radiography, cardiac and abdominal ultrasound - and were not suggestive of infection.\nA cerebral magnetic resonance imaging (MRI) revealed a lesion in the right parietal area, suggestive of cortical dysplasia. Functional MRI was performed using sensory tasks which showed activation in the sensory cortical area as well as in the lesion []. Interictal Electroencephalography (EEG) showed sporadic active spiking in the right temporal region []. Valproate was reintroduced without any improvement. Carbamazepine and later Clobazam were added but did not prevent seizures. Lamotrigine and vigabatrin were proposed before suggesting surgical treatment.