ABSTRACT
OBJECTIVE AND IMPORTANCE: To discuss the physiopathology and surgical handling of seizures due to hamartoma of the floor of the fourth ventricle in two children. CLINICAL PRESENTATION: Two girls aged 3 years at the time of their operations presented with seizures due to a lesion of the floor of the fourth ventricle. The seizures began within the first days of life and consisted of hemifacial contraction, then head deviation, blinking of the eyelids, and intermittent dysautonomic manifestations. The interictal neurological condition seemed normal in one patient and showed a slight development delay in the other. An ictal electroencephalogram showed slow waves in the posterior areas. A magnetic resonance imaging scan revealed a mass that remained unchanged on serial examinations bordering the fourth ventricle, with an isointense signal on T1-weighted sequences and high-intensity signals on T2-weighted sequences without gadolinium enhancement. An ictal single-photon emission computed tomographic scan showed hyperperfusion in the lesion in both girls. INTERVENTION: The operation consisted of resection and disconnection of the lesion. An electrical recording was obtained in one patient during the operation while she was anesthetized; the recording, made by means of a depth electrode with five contacts inside the lesion, indicated that repetitive theta rhythmic discharges were present. Neuropathology was consistent with a hamartoma. In both girls, the seizures disappeared after their operations, and antiepileptic drugs were withdrawn (follow-up periods, 8 and 3 yr, respectively). CONCLUSION: Considering the results of single-photon emission computed tomography, the intralesional electrical record, and the relief of seizures after the operation, we postulate that the seizures arose from inside the lesion. This particular kind of noncortical seizure is similar to gelastic seizure due to hypothalamic hamartoma.