Satoshi Maniwa

Matsuyama Red Cross Hospital, Matuyama, Ehime, Japan

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Publications (7)13.89 Total impact

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    ABSTRACT: We report two female infants with early myoclonic encephalopathy (EME) whose intractable focal seizures were suppressed with lidocaine and carbamazepine (CBZ). Although EME is a form of early-onset epileptic encephalopathy characterised by myoclonus and focal seizures that are highly resistant to treatment, lidocaine and CBZ may prove effective in treating this disorder. Future studies should be performed in order to determine whether there are common specific mechanisms of seizure generation related to the sodium channel in these patients.
    No preview · Article · Jul 2013 · Epileptic disorders: international epilepsy journal with videotape
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    ABSTRACT: Our aim was to develop a screening test to predict Dravet syndrome before the first birthday based on the clinical characteristics of infants and the SCN1A mutation analysis. Ninety-six patients who experienced febrile seizures before the age of one were enrolled. The patients were divided into two groups-the Dravet syndrome group (n = 46) and the non-Dravet syndrome group (n = 50). We compared the clinical characteristics before one year of age of the two groups. We analyzed all coding exons of the SCN1A gene by the direct sequencing method. Scores from 0 to 3 were assigned to each risk factor based on the odds ratio and p-value. An age of onset of febrile seizure <or= 7 months, a total number of seizures >or= 5, and prolonged seizures lasting more than 10 min. were regarded as significant risk factors for Dravet syndrome. Other factors highly predictive of this syndrome were hemiconvulsions, partial seizures, myoclonic seizures, and hot water-induced seizures. A total clinical score of six or above was the cutoff value indicating a high risk of Dravet syndrome. SCN1A missense and truncated mutations were detected significantly more often in the Dravet syndrome group than in the non-Dravet syndrome group. This simple screening test was designed to be used by general pediatricians. It could help to predict Dravet syndrome before one year of age. If the sum of the clinical risk score is >or= 6, then the performance of an SCN1A mutation analysis is recommended.
    Full-text · Article · May 2008 · Epilepsia
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    ABSTRACT: We classified 28 patients with severe myoclonic epilepsy in infancy (SME) according to the presence or absence of myoclonic seizures and/or atypical absences. Eleven of the patients had myoclonic seizures and/or atypical absences, and we refer to this condition as 'typical SME (TSME)'. Seventeen of the patients had only segmental myoclonias, and we refer to this condition as 'borderline SME (BSME)'. We then analyzed the electroclinical and genetic characteristics of these two groups. Ten of the 11 TSME patients had a photoparoxysmal response at some time during their clinical course, while none of the BSME patients showed this response. TSME and BSME showed a significant difference in regard to gender ratio: female dominance in TSME and male dominance in BSME (P=0.008). The detection rate of the voltage-gated sodium channel alpha1-subunit (SCN1A) gene mutations was 72.7 and 88.2% in TSME and BSME, respectively. There was no difference in the type or rate of mutation between TSME and BSME. We conclude that TSME and BSME show distinct differences in photoparoxysmal response and gender, which might be caused by some genetic mechanism(s) other than the SCN1A gene mutation.
    No preview · Article · Nov 2003 · Brain and Development
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    ABSTRACT: Three hundred eight patients with childhood and adolescent epilepsy were examined to clarify the incidence of epileptic discharges on initial and follow-up electroencephalograms. Epileptic discharges were found in 75.6% patients on the initial electroencephalogram, which is higher than figures previously reported for adults. The cumulative incidence of epileptic discharges was 92.3% by the third electroencephalogram recording. However, in 17.1% patients with nonspecific idiopathic generalized epilepsy, no epileptic discharges were found even after three electroencephalogram recordings. The incidence of epileptic discharges in patients with generalized epilepsy (84.3%) was significantly higher than in patients with localization-related epilepsy (71.6%). The incidence of epileptic discharges in patients with partial seizures was lower than those in patients with generalized seizures. The incidence of epileptic discharges was low in the 0- to 3-year-old and 15- to 20-year-old groups, and high in the 3- to 12-year-old groups. In the positive epileptic discharge patients, 38.8% of electroencephalograms were abnormal only during the waking or sleeping portion of the recordings. Knowing the incidence of epileptic discharges for each type of epilepsy will be useful in planning further electroencephalogram research and performing electroencephalograms in the clinical setting.
    No preview · Article · Aug 2001 · Pediatric Neurology
  • K Kobayashi · S Maniwa · T Ogino · H Yoshinaga · Y Ohtsuka · E Oka
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    ABSTRACT: We report on a patient who showed an unusual transition from complex partial seizures to a combination of myoclonic seizures and partial seizures, and then to isolated myoclonic seizures. The pathophysiological mechanism of the myoclonic seizures in this uncommon condition was studied. The interhemispheric small time differences were estimated in the ictal EEG discharges of myoclonic seizures for differentiation between primary and secondary bilateral synchrony. The estimation was performed by coherence and phase analysis based on the two-dimensional autoregressive model. The estimated interhemispheric time differences were at most 27.4 ms. The ictal activity of the myoclonic seizures was suggested to originate from a cortical focus in the right hemisphere, which was also the origin of the partial seizures. The patient was considered to have an unusual type of myoclonic seizures with a probable pathophysiological mechanism of secondary bilateral synchrony.
    No preview · Article · Nov 2000 · Clinical Neurophysiology

  • No preview · Article · Sep 1992 · Pediatric Neurology

  • No preview · Article · Jul 1991 · The Japanese journal of psychiatry and neurology

Publication Stats

116 Citations
13.89 Total Impact Points


  • 2001-2013
    • Matsuyama Red Cross Hospital
      Matuyama, Ehime, Japan
  • 1991-2003
    • Okayama University
      • Department of Child Neurology
      Okayama, Okayama, Japan