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.
3.Early-onset response is a predictor of better longterm outcome of vagus nerve stimulation therapy
Ayataka Fujimoto ; Tohru Okanishi ; Sotaro Kanai ; Keishiro Sato ; Mitsuyo Nishimura ; Hideo Enoki
Neurology Asia 2017;22(2):117-121
Background & Objective: It is well established that the effectiveness of vagus nerve stimulation
(VNS) therapy increases over 2-3 years. When increasing the dose of VNS, some patients were noted
to respond even at low-dose stimulation in the first few months. The purpose of this study was to
evaluate the relationship between an initial response to VNS and long-term response in a retrospective
study of patients with intractable epilepsy. Method: We retrospectively analysed 56 patients who had
VNS implantation in our centre. All patients had undergone complete presurgical evaluation. After
implantation, the patients were examined at regular intervals of one month for 6-9 months and then
followed up regularly for more than 2 years. Their seizure frequency and intensity were documented
in their seizure logs. Results: Six patients achieved Engel class I (11%) seizure outcome, 16 achieved
Engel class II (28%), and 19 achieved Engel class III (34%). Of the 22 patients with Engel I and II,
the 19 in Engel class I (100%) and II (81%) showed an initial response within 6 months, an earlyonset
response of VNS implantation.
Conclusions: Early-onset response could be an independent predictor for achievement of Engel class
I and II in long-term follow-up.
Keyword: Background & Objective: It is well established that the effectiveness of vagus nerve stimulation
(VNS) therapy increases over 2-3 years. When increasing the dose of VNS, some patients were noted
to respond even at low-dose stimulation in the first few months. The purpose of this study was to
evaluate the relationship between an initial response to VNS and long-term response in a retrospective
study of patients with intractable epilepsy. Method: We retrospectively analysed 56 patients who had
VNS implantation in our centre. All patients had undergone complete presurgical evaluation. After
implantation, the patients were examined at regular intervals of one month for 6-9 months and then
followed up regularly for more than 2 years. Their seizure frequency and intensity were documented
in their seizure logs. Results: Six patients achieved Engel class I (11%) seizure outcome, 16 achieved
Engel class II (28%), and 19 achieved Engel class III (34%). Of the 22 patients with Engel I and II,
the 19 in Engel class I (100%) and II (81%) showed an initial response within 6 months, an earlyonset
response of VNS implantation.
Conclusions: Early-onset response could be an independent predictor for achievement of Engel class
I and II in long-term follow-up.
Keyword: Vagus nerve stimulation, long term outcome, early onset response, predictor, epilepsy
4.Vagus nerve stimulation therapy improves quality of life in patients with intractable postencephalitic epilepsy, a study of five patients
Ayataka FUJIMOTO ; Toru OKANISHI ; Mitsuyo NISHIMURA ; Sotaro KANAI ; Keishiro SATO ; Hideo ENOKI
Neurology Asia 2018;23(1):1-5
Introduction: To evaluate the utility of vagus nerve stimulation (VNS) therapy for patients with intractable postencephalitic epilepsy in the reduction of seizure frequency and quality of life (QOL). Methods: We studied five patients with intractable postencephalitic epilepsy, the age ranged from 21 to 46 years. QOL of the patients was evaluated with the questionnaire, QOLIE-31-P. Results: VNS therapy improved seizure frequency in four patients (80%). One patient (20%) had no reduction of seizure frequency. Three patients had improvements in QOLIE-31-P (p < 0.024) and became socially independent. Two other patients continued to be dependent, and have lesser degree of improvements in their QOLIE-31-P scores.Conclusion: VNS is effective for patients with intractable postencephalitic epilepsy and is able to improve the QOL.