ABSTRACT
We report a childhood case of localization-related epilepsy manifesting frequent gelastic seizures, which were successfully treated with topiramate (TPM) monotherapy. The seizures were not associated with feelings of mirth. High-resolution three-tesla magnetic resonance imaging revealed no structural abnormality. Interictal 18F-fluorodeoxyglucose positron emission tomography showed hypometabolism over the entire right hemisphere. Single-photon emission computed tomography imaging, both ictal and interictal, demonstrated no significant findings. Interictal electroencephalography (EEG) showed paroxysms in the right frontal region. Ictal video EEG demonstrated diffuse attenuation, followed by fast activities and spike-wave complexes predominantly over the right hemisphere. At the ictal EEG onset, low amplitude paroxysmal fast activity was recorded over the F8-T4 region. The seizures were considered to have originated from the right frontal lobe. TPM monotherapy resulted in complete cessation of the seizure. We suggest that TPM should be considered as a valuable tool for treating childhood intractable gelastic seizures, which are not due to hypothalamic hamartoma.
ABSTRACT
We describe a 13-month-old girl who developed convulsive status during an episode of rotavirus gastroenteritis. Persistent disturbance of consciousness, recurrent seizures, slowing on EEG, high signal areas on diffusion-weighted MRI, and normal cerebrospinal fl uid confi rmed the diagnosis of encephalopathy. In addition, hepatosplenomegaly, cytopenias, and a histopathological fi nding of hemophagocytosis in bone marrow were all observed in this case, and these three conditions together meet the diagnosis of hemophagocytic lymphohistiocytosis. The patient improved after commencement of steroid pulse therapy. This case suggests that the pathogenesis of rotavirus-associated encephalopathy may share some basic pathophysiologic mechanisms with hemophagocytic lymphohistiocytosis.