1.Hemophagocytic lymphohistiocytosis in a child with rotavirus encephalopathy
Hideo Enoki ; Takuya Yokota MD ; Tadashi Matsubayashi
Neurology Asia 2009;14(2):153-155
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.
2.Gelastic seizures in a child with frontal lobe epilepsy controlled by topiramate monotherapy
Hideo Enoki ; Takuya Yokota ; Mitsuyo Nishimura ; Yuki Sasaki ; Ayataka Fujimoto ; Takamichi Yamamoto
Neurology Asia 2014;19(1):89-92
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.