start-ver=1.4
cd-journal=joma
no-vol=
cd-vols=
no-issue=
article-no=
start-page=
end-page=
dt-received=
dt-revised=
dt-accepted=
dt-pub-year=1987
dt-pub=19870331
dt-online=
en-article=
kn-article=
en-subject=
kn-subject=
en-title=
kn-title=大脳半球間脳波コヒーレンスの発達に関する研究
en-subtitle=
kn-subtitle=
en-abstract=
kn-abstract=
en-copyright=
kn-copyright=
en-aut-name=
en-aut-sei=
en-aut-mei=
kn-aut-name=小林勝弘
kn-aut-sei=小林
kn-aut-mei=勝弘
aut-affil-num=1
ORCID=
affil-num=1
en-affil=
kn-affil=岡山大学
END
start-ver=1.4
cd-journal=joma
no-vol=123
cd-vols=
no-issue=2
article-no=
start-page=91
end-page=95
dt-received=
dt-revised=
dt-accepted=
dt-pub-year=2011
dt-pub=20110801
dt-online=
en-article=
kn-article=
en-subject=
kn-subject=
en-title=A long-term follow-up study of Dravet syndrome up to adulthood
kn-title=成人期に達したDravet症候群の長期経過に関する研究
en-subtitle=
kn-subtitle=
en-abstract=
kn-abstract=
en-copyright=
kn-copyright=
en-aut-name=AkiyamaMari
en-aut-sei=Akiyama
en-aut-mei=Mari
kn-aut-name=秋山麻里
kn-aut-sei=秋山
kn-aut-mei=麻里
aut-affil-num=1
ORCID=
en-aut-name=KobayashiKatsuhiro
en-aut-sei=Kobayashi
en-aut-mei=Katsuhiro
kn-aut-name=小林勝弘
kn-aut-sei=小林
kn-aut-mei=勝弘
aut-affil-num=2
ORCID=
en-aut-name=YoshinagaHarumi
en-aut-sei=Yoshinaga
en-aut-mei=Harumi
kn-aut-name=吉永治美
kn-aut-sei=吉永
kn-aut-mei=治美
aut-affil-num=3
ORCID=
en-aut-name=OhtsukaYoko
en-aut-sei=Ohtsuka
en-aut-mei=Yoko
kn-aut-name=大塚頌子
kn-aut-sei=大塚
kn-aut-mei=頌子
aut-affil-num=4
ORCID=
affil-num=1
en-affil=
kn-affil=岡山大学大学院医歯薬学総合研究科 発達神経病態学
affil-num=2
en-affil=
kn-affil=岡山大学大学院医歯薬学総合研究科 発達神経病態学
affil-num=3
en-affil=
kn-affil=岡山大学大学院医歯薬学総合研究科 発達神経病態学
affil-num=4
en-affil=
kn-affil=岡山大学大学院医歯薬学総合研究科 発達神経病態学
en-keyword=Dravet症候群
kn-keyword=Dravet症候群
en-keyword=乳児重症ミオクロニーてんかん
kn-keyword=乳児重症ミオクロニーてんかん
en-keyword=予後
kn-keyword=予後
en-keyword=けいれん性てんかん重積状態
kn-keyword=けいれん性てんかん重積状態
END
start-ver=1.4
cd-journal=joma
no-vol=66
cd-vols=
no-issue=5
article-no=
start-page=369
end-page=376
dt-received=
dt-revised=
dt-accepted=
dt-pub-year=2012
dt-pub=201210
dt-online=
en-article=
kn-article=
en-subject=
kn-subject=
en-title=
kn-title=Dravet Syndrome : A Genetic Epileptic Disorder
en-subtitle=
kn-subtitle=
en-abstract=
kn-abstract=Dravet syndrome (DS), or severe myoclonic epilepsy in infancy, is one of the most severe types of genetic epilepsy. It is characterized by the initial occurrence of febrile or afebrile seizures that often evolve into status epilepticus in infants with normal development, and by the subsequent appearance of myoclonic and/or atypical absence seizures as well as complex partial seizures. The key feature that characterizes DS is fever sensitivity, although photosensitivity and pattern-sensitivity are also often seen. The prognosis is unfavorable in most cases. Seizures become drug-resistant and persist, with many patients suffering from motor and cognitive impairment. Mutations of SCN1A, which encodes the voltage-gated sodium channel NaV1.1, are the most frequent genetic cause of this syndrome. SCN1A mutations and/or microchromosomal rearrangements involving SCN1A are detected in about 85% of patients. Mutations of PCDH19 have also been reported in female patients with clinical findings compatible with DS. PCDH19 mutations might account for 5% of overall DS cases. Thirty years after its first description, DS is considered as a model of channelopathy. This survey reviews recent developments in the research literature on DS, focusing on the clinical course, as well as its genetic causes.
en-copyright=
kn-copyright=
en-aut-name=AkiyamaMari
en-aut-sei=Akiyama
en-aut-mei=Mari
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=1
ORCID=
en-aut-name=KobayashiKatsuhiro
en-aut-sei=Kobayashi
en-aut-mei=Katsuhiro
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=2
ORCID=
en-aut-name=OhtsukaYoko
en-aut-sei=Ohtsuka
en-aut-mei=Yoko
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=3
ORCID=
affil-num=1
en-affil=
kn-affil=Department of Child Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, and Okayama University Hospital
affil-num=2
en-affil=
kn-affil=Department of Child Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, and Okayama University Hospital
affil-num=3
en-affil=
kn-affil=Department of Child Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, and Okayama University Hospital
en-keyword=Dravet syndrome
kn-keyword=Dravet syndrome
en-keyword=long-term outcome
kn-keyword=long-term outcome
en-keyword=SCN1A
kn-keyword=SCN1A
en-keyword=PCDH19
kn-keyword=PCDH19
END
start-ver=1.4
cd-journal=joma
no-vol=66
cd-vols=
no-issue=6
article-no=
start-page=487
end-page=492
dt-received=
dt-revised=
dt-accepted=
dt-pub-year=2012
dt-pub=201212
dt-online=
en-article=
kn-article=
en-subject=
kn-subject=
en-title=
kn-title=Successful Treatment of Epilepsy by Resection of Periventricular Nodular Heterotopia
en-subtitle=
kn-subtitle=
en-abstract=
kn-abstract=We report on a case of successful surgical treatment of drug-resistant epilepsy associated with a solitary lesion of periventricular nodular heterotopia (PNH). In the reported patient, intracranial ictal electroencephalography disclosed that seizures did not originate from the heterotopic nodules. However, the seizures were completely suppressed by lesionectomy of PNH alone. Epileptogenesis associated with PNH likely involves a very complex network between PNH and the surrounding cortex, and the disruption of this network may be an effective means of curing intractable, PNH-associated epilepsy.
en-copyright=
kn-copyright=
en-aut-name=AgariTakashi
en-aut-sei=Agari
en-aut-mei=Takashi
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=1
ORCID=
en-aut-name=MiharaTadahiro
en-aut-sei=Mihara
en-aut-mei=Tadahiro
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=2
ORCID=
en-aut-name=BabaKoichi
en-aut-sei=Baba
en-aut-mei=Koichi
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=3
ORCID=
en-aut-name=KobayashiKatsuhiro
en-aut-sei=Kobayashi
en-aut-mei=Katsuhiro
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=4
ORCID=
en-aut-name=UsuiNaotaka
en-aut-sei=Usui
en-aut-mei=Naotaka
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=5
ORCID=
en-aut-name=TeradaKiyohito
en-aut-sei=Terada
en-aut-mei=Kiyohito
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=6
ORCID=
en-aut-name=NakamuraFumihiro
en-aut-sei=Nakamura
en-aut-mei=Fumihiro
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=7
ORCID=
en-aut-name=MatsudaKazumi
en-aut-sei=Matsuda
en-aut-mei=Kazumi
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=8
ORCID=
en-aut-name=DateIsao
en-aut-sei=Date
en-aut-mei=Isao
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=9
ORCID=
affil-num=1
en-affil=
kn-affil=Department of Neurological Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences and Okayama University Hospital
affil-num=2
en-affil=
kn-affil=National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders
affil-num=3
en-affil=
kn-affil=National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders
affil-num=4
en-affil=
kn-affil=Department of Child Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences and Okayama University Hospital
affil-num=5
en-affil=
kn-affil=National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders
affil-num=6
en-affil=
kn-affil=National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders
affil-num=7
en-affil=
kn-affil=National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders
affil-num=8
en-affil=
kn-affil=National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders
affil-num=9
en-affil=
kn-affil=Department of Neurological Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences and Okayama University Hospital
en-keyword=periventricular nodular heterotopia
kn-keyword=periventricular nodular heterotopia
en-keyword=epilepsy
kn-keyword=epilepsy
en-keyword=surgery
kn-keyword=surgery
en-keyword=ictal electroencephalography
kn-keyword=ictal electroencephalography
END
start-ver=1.4
cd-journal=joma
no-vol=128
cd-vols=
no-issue=3
article-no=
start-page=183
end-page=189
dt-received=
dt-revised=
dt-accepted=
dt-pub-year=2016
dt-pub=20161201
dt-online=
en-article=
kn-article=
en-subject=
kn-subject=
en-title=Electroencephalography:an old examination tool with a new meaning for childhood epilepsy
kn-title=脳波:小児てんかんにおける古くて新しい検査
en-subtitle=
kn-subtitle=
en-abstract=
kn-abstract=
en-copyright=
kn-copyright=
en-aut-name=KobayashiKatsuhiro
en-aut-sei=Kobayashi
en-aut-mei=Katsuhiro
kn-aut-name=小林勝弘
kn-aut-sei=小林
kn-aut-mei=勝弘
aut-affil-num=1
ORCID=
affil-num=1
en-affil=Department of Child Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences
kn-affil=岡山大学大学院医歯薬学総合研究科 発達神経病態学
en-keyword=頭皮脳波
kn-keyword=頭皮脳波
en-keyword=点頭てんかん
kn-keyword=点頭てんかん
en-keyword=てんかん外科
kn-keyword=てんかん外科
en-keyword=高周波振動
kn-keyword=高周波振動
en-keyword=時間・周波数分析
kn-keyword=時間・周波数分析
END
start-ver=1.4
cd-journal=joma
no-vol=74
cd-vols=
no-issue=1
article-no=
start-page=65
end-page=72
dt-received=
dt-revised=
dt-accepted=
dt-pub-year=2020
dt-pub=202002
dt-online=
en-article=
kn-article=
en-subject=
kn-subject=
en-title=
kn-title=Metabolic Profiling of the Cerebrospinal Fluid in Pediatric Epilepsy
en-subtitle=
kn-subtitle=
en-abstract=
kn-abstract= To characterize metabolic profiles within the central nervous system in epilepsy, we performed gas chromatography-tandem mass spectrometry (GC-MS/MS)-based metabolome analysis of the cerebrospinal fluid (CSF) in pediatric patients with and without epilepsy. The CSF samples obtained from 64 patients were analyzed by GC-MS/MS. Multivariate analyses were performed for two age groups, 0-5 years of age and 6-17 years of age, to elucidate the effects of epilepsy and antiepileptic drugs on the metabolites. In patients aged 0-5 years (22 patients with epilepsy, 13 without epilepsy), epilepsy patients had reduced 2-ketoglutaric acid and elevated pyridoxamine and tyrosine. In patients aged 6-17 years (12 with epilepsy, 17 without epilepsy), epilepsy patients had reduced 1,5-anhydroglucitol. Valproic acid was associated with elevated 2-aminobutyric acid, 2-ketoisocaproic acid, 4-hydroxyproline, acetylglycine, methionine, N-acetylserine, and serine. Reduced energy metabolism and alteration of vitamin B6 metabolism may play a role in epilepsy in young children. The roles of 1,5-anhydroglucitol in epilepsy in older children and in levetiracetam and zonisamide treatment remain to be explained. Valproic acid influenced the levels of amino acids and related metabolites involved in the metabolism of serine, methionine, and leucine.
en-copyright=
kn-copyright=
en-aut-name=AkiyamaTomoyuki
en-aut-sei=Akiyama
en-aut-mei=Tomoyuki
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=1
ORCID=
en-aut-name=SaigusaDaisuke
en-aut-sei=Saigusa
en-aut-mei=Daisuke
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=2
ORCID=
en-aut-name=HyodoYuki
en-aut-sei=Hyodo
en-aut-mei=Yuki
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=3
ORCID=
en-aut-name=UmedaKeiko
en-aut-sei=Umeda
en-aut-mei=Keiko
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=4
ORCID=
en-aut-name=SaijoReina
en-aut-sei=Saijo
en-aut-mei=Reina
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=5
ORCID=
en-aut-name=KoshibaSeizo
en-aut-sei=Koshiba
en-aut-mei=Seizo
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=6
ORCID=
en-aut-name=KobayashiKatsuhiro
en-aut-sei=Kobayashi
en-aut-mei=Katsuhiro
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=7
ORCID=
affil-num=1
en-affil=Department of Child Neurology, Okayama University Hospital
kn-affil=
affil-num=2
en-affil=Tohoku Medical Megabank Organization, Tohoku University
kn-affil=
affil-num=3
en-affil=Department of Child Neurology, Okayama University Hospital
kn-affil=
affil-num=4
en-affil=Tohoku Medical Megabank Organization, Tohoku University
kn-affil=
affil-num=5
en-affil=Tohoku Medical Megabank Organization, Tohoku University
kn-affil=
affil-num=6
en-affil=Tohoku Medical Megabank Organization, Tohoku University
kn-affil=
affil-num=7
en-affil=Department of Child Neurology, Okayama University Hospital
kn-affil=
en-keyword=antiepileptic drugs
kn-keyword=antiepileptic drugs
en-keyword=gas chromatography-tandem mass spectrometry
kn-keyword=gas chromatography-tandem mass spectrometry
en-keyword=metabolome analysis
kn-keyword=metabolome analysis
en-keyword=metabolomics
kn-keyword=metabolomics
END
start-ver=1.4
cd-journal=joma
no-vol=62
cd-vols=
no-issue=5
article-no=
start-page=587
end-page=592
dt-received=
dt-revised=
dt-accepted=
dt-pub-year=2020
dt-pub=20200119
dt-online=
en-article=
kn-article=
en-subject=
kn-subject=
en-title=
kn-title=Laboratory changes during adrenocorticotropic hormone therapy associated with renal calcified lesions
en-subtitle=
kn-subtitle=
en-abstract=
kn-abstract=Background
Renal calcified lesions are known as one of the complications during adrenocorticotropic hormone (ACTH) therapy for intractable epilepsy. However, laboratory changes during the therapy or laboratory features of high‐risk cases with renal calcified lesions are yet to be clarified.
Methods
In this study, 43 patients with West syndrome aged ≤2 years were included. We retrospectively reviewed age and body mass index at the beginning of ACTH therapy, as well as the amount of fluid intake, daily urinary volume, and laboratory data during therapy. In addition, we studied the urinary sediment of the cases with renal calcified lesions diagnosed by computed tomography.
Results
After initiating ACTH treatment, urinary calcium (Ca)/creatinine ratio and urinary pH increased within 2 weeks. Urinary crystals and renal tubular epithelial cells (RTECs) in urinary sediment were frequently found in most cases. Urinary Ca levels, proteinuria or frequency of urinary crystals, and number of RTECs in the urinary sediment were significantly higher in patients with epithelial casts (ECs) or hematuria than in patients without these findings. Among the seven patients who underwent abdominal CT, ECs or hematuria were found only in those with renal calcified lesions. These findings suggested that patients with ECs or hematuria were more likely to have calcified lesions.
Conclusions
The risk of renal calcified lesions increased after 2 weeks of ACTH treatment. Abnormal findings in urinary sediments might be an early sign of renal calcification during ACTH therapy.
en-copyright=
kn-copyright=
en-aut-name=MiyaharaHiroyuki
en-aut-sei=Miyahara
en-aut-mei=Hiroyuki
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=1
ORCID=
en-aut-name=AkiyamaTomoyuki
en-aut-sei=Akiyama
en-aut-mei=Tomoyuki
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=2
ORCID=
en-aut-name=HasegawaKosei
en-aut-sei=Hasegawa
en-aut-mei=Kosei
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=3
ORCID=
en-aut-name=AkiyamaMari
en-aut-sei=Akiyama
en-aut-mei=Mari
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=4
ORCID=
en-aut-name=OkaMakio
en-aut-sei=Oka
en-aut-mei=Makio
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=5
ORCID=
en-aut-name=KobayashiKatsuhiro
en-aut-sei=Kobayashi
en-aut-mei=Katsuhiro
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=6
ORCID=
en-aut-name=TsukaharaHirokazu
en-aut-sei=Tsukahara
en-aut-mei=Hirokazu
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=7
ORCID=
affil-num=1
en-affil=Department of Pediatrics, Okayama University Hospital
kn-affil=
affil-num=2
en-affil=Department of Child Neurology, Okayama University Hospital
kn-affil=
affil-num=3
en-affil=Department of Pediatrics, Okayama University Hospital
kn-affil=
affil-num=4
en-affil=Department of Child Neurology, Dentistry and Pharmaceutical Sciences, Okayama University Graduate School of Medicine
kn-affil=
affil-num=5
en-affil=Department of Child Neurology, Dentistry and Pharmaceutical Sciences, Okayama University Graduate School of Medicine
kn-affil=
affil-num=6
en-affil=Department of Child Neurology, Okayama University Hospital
kn-affil=
affil-num=7
en-affil=Department of Dentistry and Pharmaceutical Sciences, Okayama University Graduate School of Medicine
kn-affil=
en-keyword=adrenocorticotropic hormone therapy
kn-keyword=adrenocorticotropic hormone therapy
en-keyword=calcium
kn-keyword=calcium
en-keyword=crystal
kn-keyword=crystal
en-keyword=renal tubular epithelial cell
kn-keyword=renal tubular epithelial cell
en-keyword=urinary sediment
kn-keyword=urinary sediment
END
start-ver=1.4
cd-journal=joma
no-vol=113
cd-vols=
no-issue=
article-no=
start-page=33
end-page=41
dt-received=
dt-revised=
dt-accepted=
dt-pub-year=2020
dt-pub=202012
dt-online=
en-article=
kn-article=
en-subject=
kn-subject=
en-title=
kn-title=Pyridoxal in the Cerebrospinal Fluid May Be a Better Indicator of Vitamin B6–dependent Epilepsy Than Pyridoxal 5′-Phosphate
en-subtitle=
kn-subtitle=
en-abstract=
kn-abstract=Background
We aimed to demonstrate the biochemical characteristics of vitamin B6–dependent epilepsy, with a particular focus on pyridoxal 5′-phosphate and pyridoxal in the cerebrospinal fluid.
Methods
Using our laboratory database, we identified patients with vitamin B6–dependent epilepsy and extracted their data on the concentrations of pyridoxal 5′-phosphate, pyridoxal, pipecolic acid, α-aminoadipic semialdehyde, and monoamine neurotransmitters. We compared the biochemical characteristics of these patients with those of other epilepsy patients with low pyridoxal 5′-phosphate concentrations.
Results
We identified seven patients with pyridoxine-dependent epilepsy caused by an ALDH7A1 gene abnormality, two patients with pyridoxal 5′-phosphate homeostasis protein deficiency, and 28 patients with other epilepsies with low cerebrospinal fluid pyridoxal 5′-phosphate concentrations. Cerebrospinal fluid pyridoxal and pyridoxal 5′-phosphate concentrations were low in patients with vitamin B6–dependent epilepsy but cerebrospinal fluid pyridoxal concentrations were not reduced in most patients with other epilepsies with low cerebrospinal fluid pyridoxal 5′-phosphate concentrations. Increase in 3-O-methyldopa and 5-hydroxytryptophan was demonstrated in some patients with vitamin B6–dependent epilepsy, suggestive of pyridoxal 5′-phosphate deficiency in the brain.
Conclusions
Low cerebrospinal fluid pyridoxal concentrations may be a better indicator of pyridoxal 5′-phosphate deficiency in the brain in vitamin B6–dependent epilepsy than low cerebrospinal fluid pyridoxal 5′-phosphate concentrations. This finding is especially helpful in individuals with suspected pyridoxal 5′-phosphate homeostasis protein deficiency, which does not have known biomarkers.
en-copyright=
kn-copyright=
en-aut-name=AkiyamaTomoyuki
en-aut-sei=Akiyama
en-aut-mei=Tomoyuki
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=1
ORCID=
en-aut-name=HyodoYuki
en-aut-sei=Hyodo
en-aut-mei=Yuki
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=2
ORCID=
en-aut-name=HasegawaKosei
en-aut-sei=Hasegawa
en-aut-mei=Kosei
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=3
ORCID=
en-aut-name=OboshiTaikan
en-aut-sei=Oboshi
en-aut-mei=Taikan
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=4
ORCID=
en-aut-name=ImaiKatsumi
en-aut-sei=Imai
en-aut-mei=Katsumi
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=5
ORCID=
en-aut-name=IshiharaNaoko
en-aut-sei=Ishihara
en-aut-mei=Naoko
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=6
ORCID=
en-aut-name=DowaYuri
en-aut-sei=Dowa
en-aut-mei=Yuri
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=7
ORCID=
en-aut-name=KoikeTakayoshi
en-aut-sei=Koike
en-aut-mei=Takayoshi
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=8
ORCID=
en-aut-name=YamamotoToshiyuki
en-aut-sei=Yamamoto
en-aut-mei=Toshiyuki
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=9
ORCID=
en-aut-name=ShibasakiJun
en-aut-sei=Shibasaki
en-aut-mei=Jun
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=10
ORCID=
en-aut-name=ShimboHiroko
en-aut-sei=Shimbo
en-aut-mei=Hiroko
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=11
ORCID=
en-aut-name=FukuyamaTetsuhiro
en-aut-sei=Fukuyama
en-aut-mei=Tetsuhiro
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=12
ORCID=
en-aut-name=TakanoKyoko
en-aut-sei=Takano
en-aut-mei=Kyoko
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=13
ORCID=
en-aut-name=ShirakuHiroshi
en-aut-sei=Shiraku
en-aut-mei=Hiroshi
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=14
ORCID=
en-aut-name=TakeshitaSaoko
en-aut-sei=Takeshita
en-aut-mei=Saoko
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=15
ORCID=
en-aut-name=OkanishiTohru
en-aut-sei=Okanishi
en-aut-mei=Tohru
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=16
ORCID=
en-aut-name=BabaShimpei
en-aut-sei=Baba
en-aut-mei=Shimpei
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=17
ORCID=
en-aut-name=KubotaMasaya
en-aut-sei=Kubota
en-aut-mei=Masaya
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=18
ORCID=
en-aut-name=HamanoShin-ichiro
en-aut-sei=Hamano
en-aut-mei=Shin-ichiro
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=19
ORCID=
en-aut-name=KobayashiKatsuhiro
en-aut-sei=Kobayashi
en-aut-mei=Katsuhiro
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=20
ORCID=
affil-num=1
en-affil=Department of Child Neurology, Okayama University Hospital
kn-affil=
affil-num=2
en-affil=Department of Child Neurology, Okayama University Hospital
kn-affil=
affil-num=3
en-affil=Department of Pediatrics, Okayama University Hospital
kn-affil=
affil-num=4
en-affil=Department of Pediatric Neurology, Osaka Women’s and Children’s Hospital
kn-affil=
affil-num=5
en-affil=Department of Pediatrics, NHO Shizuoka Institute of Epilepsy and Neurological Disorders
kn-affil=
affil-num=6
en-affil=Department of Pediatrics, Fujita Health University School of Medicine
kn-affil=
affil-num=7
en-affil=Department of Neurology, Gunma Children’s Medical Center
kn-affil=
affil-num=8
en-affil=Department of Pediatrics, NHO Shizuoka Institute of Epilepsy and Neurological Disorders
kn-affil=
affil-num=9
en-affil=Institute of Clinical Genomics, Tokyo Women’s Medical University
kn-affil=
affil-num=10
en-affil=Department of Neonatology, Kanagawa Children’s Medical Center
kn-affil=
affil-num=11
en-affil=Clinical Institute, Kanagawa Children’s Medical Center
kn-affil=
affil-num=12
en-affil=Department of Pediatrics, Shinshu University
kn-affil=
affil-num=13
en-affil=Center for Medical Genetics, Shinshu University Hospital
kn-affil=
affil-num=14
en-affil=Department of Pediatrics, JA Toride Medical Center
kn-affil=
affil-num=15
en-affil=Department of Pediatrics, Yokohama City University Medical Center
kn-affil=
affil-num=16
en-affil=Department of Child Neurology, Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital
kn-affil=
affil-num=17
en-affil=Department of Child Neurology, Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital
kn-affil=
affil-num=18
en-affil=Division of Neurology, National Center for Child Health and Development
kn-affil=
affil-num=19
en-affil=Division of Neurology, Saitama Children’s Medical Center
kn-affil=
affil-num=20
en-affil=Department of Child Neurology, Okayama University Hospital
kn-affil=
en-keyword=ALDH7A1
kn-keyword=ALDH7A1
en-keyword=PLPBP
kn-keyword=PLPBP
en-keyword=PLPHP
kn-keyword=PLPHP
en-keyword=PROSC
kn-keyword=PROSC
en-keyword=Pyridoxal 5′-phosphate homeostasis protein deficiency
kn-keyword=Pyridoxal 5′-phosphate homeostasis protein deficiency
en-keyword=Pyridoxine-dependent epilepsy
kn-keyword=Pyridoxine-dependent epilepsy
END
start-ver=1.4
cd-journal=joma
no-vol=15
cd-vols=
no-issue=
article-no=
start-page=696882
end-page=
dt-received=
dt-revised=
dt-accepted=
dt-pub-year=2021
dt-pub=20210615
dt-online=
en-article=
kn-article=
en-subject=
kn-subject=
en-title=
kn-title=Exclusion of the Possibility of "False Ripples" From Ripple Band High-Frequency Oscillations Recorded From Scalp Electroencephalogram in Children With Epilepsy
en-subtitle=
kn-subtitle=
en-abstract=
kn-abstract=Aim Ripple-band epileptic high-frequency oscillations (HFOs) can be recorded by scalp electroencephalography (EEG), and tend to be associated with epileptic spikes. However, there is a concern that the filtration of steep waveforms such as spikes may cause spurious oscillations or "false ripples." We excluded such possibility from at least some ripples by EEG differentiation, which, in theory, enhances high-frequency signals and does not generate spurious oscillations or ringing. Methods The subjects were 50 pediatric patients, and ten consecutive spikes during sleep were selected for each patient. Five hundred spike data segments were initially reviewed by two experienced electroencephalographers using consensus to identify the presence or absence of ripples in the ordinary filtered EEG and an associated spectral blob in time-frequency analysis (Session A). These EEG data were subjected to numerical differentiation (the second derivative was denoted as EEG ''). The EEG '' trace of each spike data segment was shown to two other electroencephalographers who judged independently whether there were clear ripple oscillations or uncertain ripple oscillations or an absence of oscillations (Session B). Results In Session A, ripples were identified in 57 spike data segments (Group A-R), but not in the other 443 data segments (Group A-N). In Session B, both reviewers identified clear ripples (strict criterion) in 11 spike data segments, all of which were in Group A-R (p < 0.0001 by Fisher's exact test). When the extended criterion that included clear and/or uncertain ripples was used in Session B, both reviewers identified 25 spike data segments that fulfilled the criterion: 24 of these were in Group A-R (p < 0.0001). Discussion We have demonstrated that real ripples over scalp spikes exist in a certain proportion of patients. Ripples that were visualized consistently using both ordinary filters and the EEG '' method should be true, but failure to clarify ripples using the EEG '' method does not mean that true ripples are absent. Conclusion The numerical differentiation of EEG data provides convincing evidence that HFOs were detected in terms of the presence of such unusually fast oscillations over the scalp and the importance of this electrophysiological phenomenon.
en-copyright=
kn-copyright=
en-aut-name=KobayashiKatsuhiro
en-aut-sei=Kobayashi
en-aut-mei=Katsuhiro
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=1
ORCID=
en-aut-name=ShibataTakashi
en-aut-sei=Shibata
en-aut-mei=Takashi
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=2
ORCID=
en-aut-name=TsuchiyaHiroki
en-aut-sei=Tsuchiya
en-aut-mei=Hiroki
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=3
ORCID=
en-aut-name=AkiyamaTomoyuki
en-aut-sei=Akiyama
en-aut-mei=Tomoyuki
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=4
ORCID=
affil-num=1
en-affil=Department of Child Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences and Okayama University Hospital
kn-affil=
affil-num=2
en-affil=Department of Child Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences and Okayama University Hospital
kn-affil=
affil-num=3
en-affil=Department of Child Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences and Okayama University Hospital
kn-affil=
affil-num=4
en-affil=Department of Child Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences and Okayama University Hospital
kn-affil=
en-keyword=epilepsy
kn-keyword=epilepsy
en-keyword=child
kn-keyword=child
en-keyword=scalp EEG
kn-keyword=scalp EEG
en-keyword=false ripple
kn-keyword=false ripple
en-keyword=high-frequency oscillation (HFO)
kn-keyword=high-frequency oscillation (HFO)
en-keyword=fast oscillation (FO)
kn-keyword=fast oscillation (FO)
END
start-ver=1.4
cd-journal=joma
no-vol=107
cd-vols=
no-issue=
article-no=
start-page=52
end-page=59
dt-received=
dt-revised=
dt-accepted=
dt-pub-year=2023
dt-pub=202304
dt-online=
en-article=
kn-article=
en-subject=
kn-subject=
en-title=
kn-title=Comprehensive study of metabolic changes induced by a ketogenic diet therapy using GC/MS- and LC/MS-based metabolomics
en-subtitle=
kn-subtitle=
en-abstract=
kn-abstract=Objective: The ketogenic diet (KD), a high-fat and low-carbohydrate diet, is effective for a subset of patients with drug-resistant epilepsy, although the mechanisms of the KD have not been fully elucidated. The aims of this observational study were to investigate comprehensive short-term metabolic changes induced by the KD and to explore candidate metabolites or pathways for potential new therapeutic targets.
Methods: Subjects included patients with intractable epilepsy who had undergone the KD therapy (the medium-chain triglyceride [MCT] KD or the modified Atkins diet using MCT oil). Plasma and urine samples were obtained before and at 2–4 weeks after initiation of the KD. Targeted metabolome analyses of these samples were performed using gas chromatography-tandem mass spectrometry (GC/MS/MS) and liquid chromatography-tandem mass spectrometry (LC/MS/MS).
Results: Samples from 10 and 11 patients were analysed using GC/MS/MS and LC/MS/MS, respectively. The KD increased ketone bodies, various fatty acids, lipids, and their conjugates. In addition, levels of metabolites located upstream of acetyl-CoA and propionyl-CoA, including catabolites of branched-chain amino acids and structural analogues of γ-aminobutyric acid and lactic acid, were elevated.
Conclusions: The metabolites that were significantly changed after the initiation of the KD and related metabolites may be candidates for further studies for neuronal actions to develop new anti-seizure medications.
en-copyright=
kn-copyright=
en-aut-name=AkiyamaMari
en-aut-sei=Akiyama
en-aut-mei=Mari
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=1
ORCID=
en-aut-name=AkiyamaTomoyuki
en-aut-sei=Akiyama
en-aut-mei=Tomoyuki
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=2
ORCID=
en-aut-name=SaigusaDaisuke
en-aut-sei=Saigusa
en-aut-mei=Daisuke
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=3
ORCID=
en-aut-name=HishinumaEiji
en-aut-sei=Hishinuma
en-aut-mei=Eiji
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=4
ORCID=
en-aut-name=MatsukawaNaomi
en-aut-sei=Matsukawa
en-aut-mei=Naomi
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=5
ORCID=
en-aut-name=ShibataTakashi
en-aut-sei=Shibata
en-aut-mei=Takashi
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=6
ORCID=
en-aut-name=TsuchiyaHiroki
en-aut-sei=Tsuchiya
en-aut-mei=Hiroki
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=7
ORCID=
en-aut-name=MoriAtsushi
en-aut-sei=Mori
en-aut-mei=Atsushi
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=8
ORCID=
en-aut-name=FujiiYuji
en-aut-sei=Fujii
en-aut-mei=Yuji
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=9
ORCID=
en-aut-name=MogamiYukiko
en-aut-sei=Mogami
en-aut-mei=Yukiko
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=10
ORCID=
en-aut-name=TokorodaniChiho
en-aut-sei=Tokorodani
en-aut-mei=Chiho
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=11
ORCID=
en-aut-name=KuwaharaKozue
en-aut-sei=Kuwahara
en-aut-mei=Kozue
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=12
ORCID=
en-aut-name=Numata-UematsuYurika
en-aut-sei=Numata-Uematsu
en-aut-mei=Yurika
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=13
ORCID=
en-aut-name=InoueKenji
en-aut-sei=Inoue
en-aut-mei=Kenji
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=14
ORCID=
en-aut-name=KobayashiKatsuhiro
en-aut-sei=Kobayashi
en-aut-mei=Katsuhiro
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=15
ORCID=
affil-num=1
en-affil=Department of Child Neurology, Okayama University Hospital
kn-affil=
affil-num=2
en-affil=Department of Paediatrics (Child Neurology), Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences
kn-affil=
affil-num=3
en-affil=Tohoku Medical Megabank Organization, Tohoku University
kn-affil=
affil-num=4
en-affil=Tohoku Medical Megabank Organization, Tohoku University
kn-affil=
affil-num=5
en-affil=Tohoku Medical Megabank Organization, Tohoku University
kn-affil=
affil-num=6
en-affil=Department of Child Neurology, Okayama University Hospital
kn-affil=
affil-num=7
en-affil=Department of Child Neurology, Okayama University Hospital
kn-affil=
affil-num=8
en-affil=Department of Neurology, Shiga Medical Centre for Children
kn-affil=
affil-num=9
en-affil=Department of Paediatrics, Hiroshima City Funairi Citizens Hospital
kn-affil=
affil-num=10
en-affil=Department of Paediatric Neurology, Osaka Women's and Children's Hospital
kn-affil=
affil-num=11
en-affil=Department of Paediatrics, Kochi Health Sciences Centre
kn-affil=
affil-num=12
en-affil=Department of Paediatrics, Ehime Prefectural Central Hospital,
kn-affil=
affil-num=13
en-affil=Department of Paediatrics, Tohoku University School of Medicine
kn-affil=
affil-num=14
en-affil=Department of Neurology, Shiga Medical Centre for Children
kn-affil=
affil-num=15
en-affil=Department of Paediatrics (Child Neurology), Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences
kn-affil=
en-keyword=Amino acids
kn-keyword=Amino acids
en-keyword=Biomarkers
kn-keyword=Biomarkers
en-keyword=Intractable epilepsy
kn-keyword=Intractable epilepsy
en-keyword=Ketone bodies
kn-keyword=Ketone bodies
en-keyword=Organic acids
kn-keyword=Organic acids
END
start-ver=1.4
cd-journal=joma
no-vol=135
cd-vols=
no-issue=2
article-no=
start-page=98
end-page=99
dt-received=
dt-revised=
dt-accepted=
dt-pub-year=2023
dt-pub=20230801
dt-online=
en-article=
kn-article=
en-subject=
kn-subject=
en-title=The 65th Annual Meeting of the Japanese Society of Child Neurology
kn-title=第65回日本小児神経学会学術集会の開催報告
en-subtitle=
kn-subtitle=
en-abstract=
kn-abstract=
en-copyright=
kn-copyright=
en-aut-name=KobayashiKatsuhiro
en-aut-sei=Kobayashi
en-aut-mei=Katsuhiro
kn-aut-name=小林勝弘
kn-aut-sei=小林
kn-aut-mei=勝弘
aut-affil-num=1
ORCID=
affil-num=1
en-affil=Department of Pediatrics (Child Neurology), Faculty of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University
kn-affil=岡山大学学術研究院医歯薬学域 発達神経病態学
END
start-ver=1.4
cd-journal=joma
no-vol=77
cd-vols=
no-issue=5
article-no=
start-page=561
end-page=566
dt-received=
dt-revised=
dt-accepted=
dt-pub-year=2023
dt-pub=202310
dt-online=
en-article=
kn-article=
en-subject=
kn-subject=
en-title=
kn-title=Effective Epilepsy Surgery for Post-Traumatic West Syndrome Following Abusive Head Trauma
en-subtitle=
kn-subtitle=
en-abstract=
kn-abstract=West syndrome, an infantile developmental and epileptic encephalopathy with a deleterious impact on long-term development, requires early treatment to minimize developmental abnormality; in such cases, epilepsy surgery should be considered a powerful therapeutic option. We describe a 10-month-old female admitted with West syndrome associated with a hemispheric lesion following abusive head trauma. Her seizures were suppressed by hemispherotomy at 12 months of age, leading to developmental improvement. Surgical treatment of West syndrome following traumatic brain injury has not been reported previously but is worth considering as a treatment option, depending on patient age and brain plasticity.
en-copyright=
kn-copyright=
en-aut-name=TsuchiyaHiroki
en-aut-sei=Tsuchiya
en-aut-mei=Hiroki
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=1
ORCID=
en-aut-name=ShibataTakashi
en-aut-sei=Shibata
en-aut-mei=Takashi
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=2
ORCID=
en-aut-name=SasakiTatsuya
en-aut-sei=Sasaki
en-aut-mei=Tatsuya
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=3
ORCID=
en-aut-name=InoueTakushi
en-aut-sei=Inoue
en-aut-mei=Takushi
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=4
ORCID=
en-aut-name=DateIsao
en-aut-sei=Date
en-aut-mei=Isao
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=5
ORCID=
en-aut-name=AkiyamaTomoyuki
en-aut-sei=Akiyama
en-aut-mei=Tomoyuki
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=6
ORCID=
en-aut-name=KobayashiKatsuhiro
en-aut-sei=Kobayashi
en-aut-mei=Katsuhiro
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=7
ORCID=
affil-num=1
en-affil=Department of Pediatrics (Child Neurology), Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences and Okayama University Hospital
kn-affil=
affil-num=2
en-affil=Department of Pediatrics (Child Neurology), Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences and Okayama University Hospital
kn-affil=
affil-num=3
en-affil=Department of Neurological Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences and Okayama University Hospital
kn-affil=
affil-num=4
en-affil=Department of Pediatrics, National Hospital Organization Okayama Medical Center
kn-affil=
affil-num=5
en-affil=Department of Neurological Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences and Okayama University Hospital
kn-affil=
affil-num=6
en-affil=Department of Pediatrics (Child Neurology), Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences and Okayama University Hospital
kn-affil=
affil-num=7
en-affil=Department of Pediatrics (Child Neurology), Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences and Okayama University Hospital
kn-affil=
en-keyword=abusive head trauma
kn-keyword=abusive head trauma
en-keyword=developmental and epileptic encephalopathy
kn-keyword=developmental and epileptic encephalopathy
en-keyword=epilepsy surgery
kn-keyword=epilepsy surgery
en-keyword=epileptic spasms
kn-keyword=epileptic spasms
en-keyword=hemispherotomy
kn-keyword=hemispherotomy
END
start-ver=1.4
cd-journal=joma
no-vol=135
cd-vols=
no-issue=3
article-no=
start-page=116
end-page=122
dt-received=
dt-revised=
dt-accepted=
dt-pub-year=2023
dt-pub=20231201
dt-online=
en-article=
kn-article=
en-subject=
kn-subject=
en-title=Child neurology and childhood-onset intractable diseases
kn-title=小児神経学と小児期発症難病
en-subtitle=
kn-subtitle=
en-abstract=
kn-abstract=
en-copyright=
kn-copyright=
en-aut-name=KobayashiKatsuhiro
en-aut-sei=Kobayashi
en-aut-mei=Katsuhiro
kn-aut-name=小林勝弘
kn-aut-sei=小林
kn-aut-mei=勝弘
aut-affil-num=1
ORCID=
affil-num=1
en-affil=Department of Pediatrics (Child Neurology), Faculty of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University
kn-affil=岡山大学学術研究院医歯薬学域 発達神経病態学
en-keyword=指定難病
kn-keyword=指定難病
en-keyword=小児慢性特定疾患
kn-keyword=小児慢性特定疾患
en-keyword=発達性てんかん性脳症
kn-keyword=発達性てんかん性脳症
en-keyword=先天性代謝異常症
kn-keyword=先天性代謝異常症
END
start-ver=1.4
cd-journal=joma
no-vol=136
cd-vols=
no-issue=1
article-no=
start-page=22
end-page=25
dt-received=
dt-revised=
dt-accepted=
dt-pub-year=2024
dt-pub=20240401
dt-online=
en-article=
kn-article=
en-subject=
kn-subject=
en-title=Progressive myoclonus epilepsy
kn-title=進行性ミオクローヌスてんかんについて
en-subtitle=
kn-subtitle=
en-abstract=
kn-abstract=
en-copyright=
kn-copyright=
en-aut-name=KobayashiKatsuhiro
en-aut-sei=Kobayashi
en-aut-mei=Katsuhiro
kn-aut-name=小林勝弘
kn-aut-sei=小林
kn-aut-mei=勝弘
aut-affil-num=1
ORCID=
affil-num=1
en-affil=Department of Pediatrics (Child Neurology), Faculty of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University
kn-affil=岡山大学学術研究院医歯薬学域 発達神経病態学
en-keyword=難治てんかん
kn-keyword=難治てんかん
en-keyword=ミオクローヌス
kn-keyword=ミオクローヌス
en-keyword=失調
kn-keyword=失調
en-keyword=知的退行
kn-keyword=知的退行
en-keyword=ポリグラフ
kn-keyword=ポリグラフ
END
start-ver=1.4
cd-journal=joma
no-vol=46
cd-vols=
no-issue=4
article-no=
start-page=180
end-page=186
dt-received=
dt-revised=
dt-accepted=
dt-pub-year=2024
dt-pub=202404
dt-online=
en-article=
kn-article=
en-subject=
kn-subject=
en-title=
kn-title=Exploration of urine metabolic biomarkers for new-onset, untreated pediatric epilepsy: A gas and liquid chromatography mass spectrometry-based metabolomics study
en-subtitle=
kn-subtitle=
en-abstract=
kn-abstract=Objective: The discovery of objective indicators for recent epileptic seizures will help confirm the diagnosis of epilepsy and evaluate therapeutic effects. Past studies had shortcomings such as the inclusion of patients under treatment and those with various etiologies that could confound the analysis results significantly. We aimed to minimize such confounding effects and to explore the small molecule biomarkers associated with the recent occurrence of epileptic seizures using urine metabolomics.
Methods: This is a multicenter prospective study. Subjects included pediatric patients aged 2 to 12 years old with new-onset, untreated epilepsy, who had had the last seizure within 1 month before urine collection. Controls included healthy children aged 2 to 12 years old. Those with underlying or chronic diseases, acute illnesses, or recent administration of medications or supplements were excluded. Targeted metabolome analysis of spot urine samples was conducted using gas chromatography (GC)- and liquid chromatography (LC)-tandem mass spectrometry (MS/MS).
Results: We enrolled 17 patients and 21 controls. Among 172 metabolites measured by GC/MS/MS and 41 metabolites measured by LC/MS/MS, only taurine was consistently reduced in the epilepsy group. This finding was subsequently confirmed by the absolute quantification of amino acids. No other metabolites were consistently altered between the two groups.
Conclusions: Urine metabolome analysis, which covers a larger number of metabolites than conventional biochemistry analyses, found no consistently altered small molecule metabolites except for reduced taurine in epilepsy patients compared to healthy controls. Further studies with larger samples, subjects with different ages, expanded target metabolites, and the investigation of plasma samples are required.
en-copyright=
kn-copyright=
en-aut-name=AkiyamaTomoyuki
en-aut-sei=Akiyama
en-aut-mei=Tomoyuki
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=1
ORCID=
en-aut-name=SaigusaDaisuke
en-aut-sei=Saigusa
en-aut-mei=Daisuke
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=2
ORCID=
en-aut-name=InoueTakushi
en-aut-sei=Inoue
en-aut-mei=Takushi
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=3
ORCID=
en-aut-name=TokorodaniChiho
en-aut-sei=Tokorodani
en-aut-mei=Chiho
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=4
ORCID=
en-aut-name=AkiyamaMari
en-aut-sei=Akiyama
en-aut-mei=Mari
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=5
ORCID=
en-aut-name=MichiueRie
en-aut-sei=Michiue
en-aut-mei=Rie
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=6
ORCID=
en-aut-name=MoriAtsushi
en-aut-sei=Mori
en-aut-mei=Atsushi
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=7
ORCID=
en-aut-name=HishinumaEiji
en-aut-sei=Hishinuma
en-aut-mei=Eiji
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=8
ORCID=
en-aut-name=MatsukawaNaomi
en-aut-sei=Matsukawa
en-aut-mei=Naomi
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=9
ORCID=
en-aut-name=ShibataTakashi
en-aut-sei=Shibata
en-aut-mei=Takashi
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=10
ORCID=
en-aut-name=TsuchiyaHiroki
en-aut-sei=Tsuchiya
en-aut-mei=Hiroki
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=11
ORCID=
en-aut-name=KobayashiKatsuhiro
en-aut-sei=Kobayashi
en-aut-mei=Katsuhiro
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=12
ORCID=
affil-num=1
en-affil=Department of Pediatrics (Child Neurology), Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences
kn-affil=
affil-num=2
en-affil=Laboratory of Biomedical and Analytical Sciences, Faculty of Pharma-Science, Teikyo University
kn-affil=
affil-num=3
en-affil=Department of Pediatric Neurology, NHO Okayama Medical Center
kn-affil=
affil-num=4
en-affil=Department of Pediatrics, Kochi Health Sciences Center
kn-affil=
affil-num=5
en-affil=Department of Pediatrics (Child Neurology), Okayama University Hospital
kn-affil=
affil-num=6
en-affil=Department of Pediatrics (Child Neurology), Okayama University Hospital
kn-affil=
affil-num=7
en-affil=Department of Neurology, Shiga Medical Center for Children
kn-affil=
affil-num=8
en-affil=Tohoku Medical Megabank Organization, Tohoku University
kn-affil=
affil-num=9
en-affil=Tohoku Medical Megabank Organization, Tohoku University
kn-affil=
affil-num=10
en-affil=Department of Pediatrics (Child Neurology), Okayama University Hospital
kn-affil=
affil-num=11
en-affil=Department of Pediatrics (Child Neurology), Okayama University Hospital
kn-affil=
affil-num=12
en-affil=Department of Pediatrics (Child Neurology), Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences
kn-affil=
en-keyword=Amino acids
kn-keyword=Amino acids
en-keyword=Gas chromatography
kn-keyword=Gas chromatography
en-keyword=Liquid chromatography
kn-keyword=Liquid chromatography
en-keyword=Mass spectrometry
kn-keyword=Mass spectrometry
en-keyword=New-onset epilepsy
kn-keyword=New-onset epilepsy
END