Hideki Shimomura

Publications (7)9.74 Total impact

• Article: [Efficacy, safety and tolerability of high-dose topiramate with rapid dose titration in symptomatic West syndrome].

  Tomohiro Kumada, Tomoko Miyajima, Nozomi Oda, Hideki Shimomura, Keiko Saito, Ikuko Hiejima, Fumihito Nozaki, Tatsuya Fujii
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  ABSTRACT: We investigated the efficacy, safety, and tolerability of high-dose topiramate with rapid dose titration in 12 children with symptomatic West syndrome who suffered from severe motor and intellectual disabilities. Topiramate was introduced as add-on therapy at the daily dose of 1 mg/kg/day, followed by increments of 2 mg/kg at 3- or 4-day intervals, up to a maximum of 19 or 20 mg/kg/day. The ages at the start of topiramate therapy ranged from 5 to 22 months. Prior to the topiramate therapy, the patients had received 2 to 6 antiepileptic agents with (8 patients) or without ACTH (4 patients). Topiramate appeared to be effective in 8 of the 12 patients (67%); four became seizure-free;three showed greater than 90% seizure reduction; one showed greater than 50% seizure reduction. The maintenance dose was 7 to 20 mg/kg/day (mean:17.9 +/- 3.9 mg/kg/day). In 4 of these 8 patients (50%), the spasms relapsed several months after complete cessation or diminution in the frequency of the spasms following treatment with topiramate. All of the 8 topiramate-responsive patients could continue the topiramate therapy throughout this study. The duration of topiramate therapy was 7 to 42 months (median: 12.5 months). There were no severe side effects that necessitated discontinuation of topiramate, including kidney stones. High-dose topiramate with rapid dose titration was revealed to be effective, safe, and well-tolerated in children with symptomatic West syndrome.
  No to hattatsu. Brain and development 11/2012; 44(6):455-9.
• Article: [Ketogenic diet may control seizures by increasing the binding potential of the benzodiazepine receptor: a speculation from the [11C] flumazenil-PET study].

  Tomohiro Kumada, Ryuichi Nishii, Tatsuya Higashi, Tomoko Miyajima, Nozomi Oda, Hideki Shimomura, Keiko Saito, Tatsuya Fujii
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  ABSTRACT: The ketogenic diet (KD) is a high-fat, low-protein, low-carbohydrate diet which is effective in the treatment of medically refractory epilepsy. Several theories have been proposed to explain the mechanism underlying the anticonvulsant efficacy of the KD, however, the precise anticonvulsant mechanism of the KD is still unknown. We speculated the mechanism underlying the effect of the KD in patients with intractable epilepsy, based on the results of the [11C] flumazenil (FMZ)-positron emission tomography (PET) study. A patient developed frontal lobe epilepsy at the age of 2 years. At the age of 4 years 11 months, she was admitted to our hospital for the initiation of a KD. At the time of admission, she had several epileptic attacks each day: frequent postural tonic seizures, hypermotor seizures, head nodding, and intermittent loss of consciousness (non-convulsive status epilepticus). MR imaging showed no abnormal signal intensity in the brain. With the KD, the seizure frequency reduced dramatically on the fifth day. Interictal [11C] FMZ-PET was performed before and 2 months after the initiation of the KD. Before the KD, the [11C] FMZ-PET images and [11C] FMZ-PET binding potential (BP) images showed extremely low accumulation of FMZ throughout the cerebral cortex. Two months after the initiation of the KD, significantly increased binding potential of [11C] FMZ was observed, implying the increased binding potential of the benzodiazepine receptors, probably due to the anticonvulsant effect of the KD. These PET findings suggested that KD may control seizures by directly or indirectly increasing the binding potential of the benzodiazepine receptors.
  No to hattatsu. Brain and development 01/2012; 44(1):50-4.
• Article: Pyruvate therapy for mitochondrial DNA depletion syndrome.

  Keiko Saito, Nobusuke Kimura, Nozomi Oda, Hideki Shimomura, Tomohiro Kumada, Tomoko Miyajima, Kei Murayama, Masashi Tanaka, Tatsuya Fujii
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  ABSTRACT: Mitochondrial DNA depletion syndromes are a group of heterogeneous autosomal recessive disorders associated with a severe reduction in mitochondrial DNA in the affected tissues. Sodium pyruvate has been reported to have a therapeutic effect in mitochondrial diseases. We analyzed the effects of 0.5g/kg of sodium pyruvate administered through a nasogastric tube in a one-year-old patient with myopathic mitochondrial DNA depletion syndrome. To evaluate the improvement, we used the Newcastle Paediatric Mitochondrial Disease Scale (NPMDS) and manual muscle testing. As the improvement of motor functions in this severely disabled infant could not be comprehensively detected by NPMDS, we also observed the infant's ability to perform several tasks such as pouting, winking, and number of times she could tap a toy xylophone with a stick. Blood lactate and pyruvate levels were also monitored. After one month's treatment, the NPMDS score in section IV, the domain for the quality of life, improved from 17 to13. The infant became capable of raising her forearm, lower leg and wrist against gravity. The maximum number of times she could repeat each task increased and the movements became brisker and stronger. No significant change of the blood lactate level or lactate-to-pyruvate ratio, both of which were mildly increased at the initiation of the therapy, was observed despite the clinical improvement. Sodium pyruvate administered at 0.5g/kg improved the muscle strength and the NPMDS score of an infant with myopathic mitochondrial DNA depletion syndrome. Sodium pyruvate may be effective for ameliorating the clinical manifestations of mitochondrial diseases. This article is part of a Special Issue entitled: Biochemistry of Mitochondria.
  Biochimica et Biophysica Acta 08/2011; 1820(5):632-6. · 4.66 Impact Factor
• Article: Epilepsy in pervasive developmental disorder without brain MRI abnormalities.

  Tomoko Miyajima, Nobusuke Kimura, Tomohiro Kumada, Nozomi Oda, Hideki Shimomura, Tatsuya Fujii
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  ABSTRACT: Epilepsy has been reported in patients with pervasive developmental disorder (PDD), with an incidence ranging from 5% to 40%; however most of the studies included patients with brain magnetic resonance imaging (MRI) abnormalities (e.g., tuberous sclerosis) and patients with epilepsy whose seizure onset was in the first year of life. We retrospectively examined 67 patients (45 males, 22 females) with PDD and epilepsy, who did not have brain MRI abnormalities. Patients who had seizures in the first year of life were excluded. We divided the patients into two groups: group A included 34 patients with an IQ<50, and group B included 33 patients with an IQ≥50. The median age of epilepsy onset was higher in group A than group B (8.8 vs. 5.2 years, P<0.01). Only one patient (3%) in group A and nine patients (27%) in group B were classified with generalized epilepsy (P<0.05). At the last observation, 16 patients (47%) in group A and 25 patients (76%) in group B were seizure-free for ≥1year (not statistically significant). The relationship between PDD and epilepsy may be different between patients with lower (group A) and higher (group B) IQs in patients who do not have brain MRI abnormalities.
  Brain & development 06/2011; 33(6):504-7. · 1.74 Impact Factor
• Article: [Prednisolone treatment for Duchenne muscular dystrophy].

  Hideki Shimomura, Tatsuya Fujii, Tomoko Miyajima, Tomohiro Kumada, Nobusuke Kimura, Nozomi Oda, Keiko Saito
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  ABSTRACT: Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder and is the most common form of muscular dystrophy. Affected children develop muscle weakness in early childhood. Only steroids have been shown with evidence to improve muscle function in patients with DMD. We report the long-term effects of prednisolone treatment in patients with DMD, comparing the age at which 14 treated patients and 15 control patients lost their ability to walk. The prednisolone-treated patients were assigned to one of five regimens: 0.5 mg/kg/day given for the first 10 days of every month (n = 6), 0.75 mg/kg/day given for the first 10 days of every month (n = 3), 0.5 mg/kg on alternate days (n = 1), 0.75 mg/kg on alternate days (n = 1), or 5 mg/kg twice a week (n = 3). No significant difference in age of losing ambulation ability was observed between the treated group and the untreated group (mean age; 10 years and 6 months in both groups). However, 13 of the 14 patients showed an improvement in their activity of daily living other than ambulation in the treated group. The results of this study showed that the prednisolone treatment regimens used in this study did not prolong the period of ambulation.
  No to hattatsu. Brain and development 01/2011; 43(1):24-9.
• Article: Efficacy and tolerability of modified Atkins diet in Japanese children with medication-resistant epilepsy.

  Tomohiro Kumada, Tomoko Miyajima, Nozomi Oda, Hideki Shimomura, Keiko Saito, Tatsuya Fujii
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  ABSTRACT: Ten Japanese patients aged 1.5-17years with medication-resistant epilepsy were placed on the modified Atkins diet (MAD) for 3weeks during admission to our hospital. Dietary carbohydrate was restricted to 10g per day. We studied the efficacy of the diet regarding the seizure frequency and tolerability of the diet at the end of the 3weeks on the diet. Those who decided to continue the MAD at the time of discharge were followed up in the out-patient clinic to observe the effect of the diet on the seizure frequency. Three of the 10 patients could not continue the diet during the 3-week admission; one had rotavirus enterocolitis and the other 2 disliked the diet. Among the remaining 7 patients who could continue the diet for 3weeks, 3 achieved the seizure reduction; 2 became seizure-free and 1 showed about 75% reduction in the seizure frequency within 10days on the diet. All of these 3 patients continued the diet after the 3-week admission. The other 4 patients did not show a reduction of the seizure frequency by the end of the 3weeks on the diet. Two of them discontinued the diet on discharge. The remaining 2 still continued the diet at home and one became seizure-free 3months after the start of the diet. In total, 4 of 10 patients achieved>75% reduction in the seizure frequency, although relapse occurred in 2 of the patients, at 5months and 2years after seizure reduction, respectively. The MAD was effective and well-tolerated in children with medication-resistant epilepsy in Japan.
  Brain & development 01/2011; 34(1):32-8. · 1.74 Impact Factor
• Article: Modified Atkins diet for the treatment of nonconvulsive status epilepticus in children.

  Tomohiro Kumada, Tomoko Miyajima, Nobusuke Kimura, Keiko Saito, Hideki Shimomura, Nozomi Oda, Tatsuya Fujii
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  ABSTRACT: The authors describe the use of a modified Atkins diet for the treatment of 2 children with nonconvulsive status epilepticus. Patient 1 was a 4-year-and-11-month-old girl diagnosed with frontal lobe epilepsy. Since the age of 3 years and 10 months, she had daily nonconvulsive status epilepticus resistant to antiepileptic agents. Patient 2 was a 5-year-and-5-month-old girl with subcortical band heterotopia. She had nonconvulsive status epilepticus daily since the age of 5 years. They were treated with the modified Atkins diet, in which carbohydrate intake was restricted to 10 g/d without restriction on protein, caloric, or fluid intake. The nonconvulsive status epilepticus disappeared 5 and 10 days after the initiation of the diet treatment, respectively. They have been on the diet treatment and free from nonconvulsive status epilepticus for 19 and 4 months, respectively. The modified Atkins diet appears to be very effective for the treatment of nonconvulsive status epilepticus.
  Journal of child neurology 09/2009; 25(4):485-9. · 1.59 Impact Factor