Publications by authors named "Shin-Ichiro Hamano"

82 Publications

Comparison of adrenocorticotropic hormone efficacy between aetiologies of infantile spasms.

Seizure 2021 Feb 17;85:6-11. Epub 2020 Dec 17.

Division of Neurology, Saitama Children's Medical Center, 1-2 Shintoshin, Chuo-ku, Saitama-city, Saitama, Japan.

Purpose: We aimed to study the efficacy of adrenocorticotropic hormone (ACTH) treatment on infantile spasms with different aetiologies. In particular, we were interested in patients with structural-acquired aetiology.

Methods: Patients with infantile spasms, who were treated with ACTH, were divided into three groups based on the aetiologies: unknown aetiology with normal development (unknown-normal), structural-acquired, and combined-congenital aetiologies that included genetic, metabolic, structural-congenital, or unknown aetiology with developmental delay.

Results: Of the 107 patients included (58 males, 49 females), 25 patients had unknown-normal aetiology [median age at onset 5 months, standard deviation (SD) 3.12, range 2-16 months]; 20 patients had structural-acquired aetiology (median age at onset 6.5 months, SD 3.85 months, range 4-17 months); and 62 patients had combined-congenital aetiologies (median age at onset 5 months, SD 2.73 months, range 2-16 months). The efficacy of ACTH was 64.0 %, 65 %, and 30.6 % in the unknown-normal aetiology, structural-acquired aetiology, and combined-congenital aetiologies, respectively (p < 0.01). Multivariate analysis showed a statistically significant higher efficacy in the unknown-normal aetiology [Odds ratio (OR) 4.63, 95 % confidence interval (CI) 1.60-13.30] and structural-acquired aetiology (OR 3.41, 95 % CI 1.01-11.50) compared to that in the combined-congenital aetiologies.

Conclusion: Infantile spasms with structural-acquired aetiology had greater response to ACTH treatment than those with combined-congenital aetiologies. The efficacy of standard therapy of infantile spasms should be considered based on aetiology.
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http://dx.doi.org/10.1016/j.seizure.2020.12.008DOI Listing
February 2021

Efficient detection of copy-number variations using exome data: Batch- and sex-based analyses.

Hum Mutat 2021 Jan 11;42(1):50-65. Epub 2020 Nov 11.

Department of Human Genetics, Yokohama City University Graduate School of Medicine, Yokohama, Japan.

Many algorithms to detect copy number variations (CNVs) using exome sequencing (ES) data have been reported and evaluated on their sensitivity and specificity, reproducibility, and precision. However, operational optimization of such algorithms for a better performance has not been fully addressed. ES of 1199 samples including 763 patients with different disease profiles was performed. ES data were analyzed to detect CNVs by both the eXome Hidden Markov Model (XHMM) and modified Nord's method. To efficiently detect rare CNVs, we aimed to decrease sequencing biases by analyzing, at the same time, the data of all unrelated samples sequenced in the same flow cell as a batch, and to eliminate sex effects of X-linked CNVs by analyzing female and male sequences separately. We also applied several filtering steps for more efficient CNV selection. The average number of CNVs detected in one sample was <5. This optimization together with targeted CNV analysis by Nord's method identified pathogenic/likely pathogenic CNVs in 34 patients (4.5%, 34/763). In particular, among 142 patients with epilepsy, the current protocol detected clinically relevant CNVs in 19 (13.4%) patients, whereas the previous protocol identified them in only 14 (9.9%) patients. Thus, this batch-based XHMM analysis efficiently selected rare pathogenic CNVs in genetic diseases.
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http://dx.doi.org/10.1002/humu.24129DOI Listing
January 2021

Pyridoxal in the Cerebrospinal Fluid May Be a Better Indicator of Vitamin B6-dependent Epilepsy Than Pyridoxal 5'-Phosphate.

Pediatr Neurol 2020 12 2;113:33-41. Epub 2020 Sep 2.

Department of Child Neurology, Okayama University Hospital, Okayama, Japan; Department of Child Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.

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.
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http://dx.doi.org/10.1016/j.pediatrneurol.2020.08.020DOI Listing
December 2020

Zonisamide Therapy for Patients With Paroxysmal Kinesigenic Dyskinesia.

Pediatr Neurol 2020 10 30;111:23-26. Epub 2020 Jun 30.

Division of Child Health and Human Development, Saitama Children's Medical Center, Saitama, Japan.

Background: We evaluated zonisamide therapy in patients with paroxysmal kinesigenic dyskinesia (PKD).

Methods: We analyzed zonisamide therapy in 17 patients with PKD at Saitama Children's Medical Center between November 1994 and April 2020. We collected information regarding family history, previous history, age at onset, age at zonisamide commencement, dyskinesia characteristics, brain magnetic resonance imaging, interictal electroencephalography, treatment lag, zonisamide efficacy, zonisamide dose, serum zonisamide concentration, and adverse effects. We evaluated PKD frequency at six months after zonisamide therapy commencement.

Results: Fourteen patients met the inclusion criteria. The median age at zonisamide therapy commencement was 12.8 (9.4 to 16.3) years. Zonisamide therapy was effective in 13 of 14 (92.9%) patients: complete remission for more than three months after zonisamide therapy (n = 7), decreased dyskinesia frequency by more than 90% (n = 4), dyskinesia frequency by 75% to 90% (n = 2), and no change of dyskinesia frequency (n = 1). The initial and maintenance zonisamide doses were 2.0 (1.4 to 3.8) and 2.0 (1.5 to 5.9) mg/kg/day, respectively. The median duration between zonisamide therapy commencement and dyskinesia decrease or cessation was 4 (1 to 60) days: 10 of 14 (71.4%) patients responded to zonisamide within one week after zonisamide therapy commencement. Regarding adverse effects, two patients experienced somnolence and one developed reduced perspiration.

Conclusions: We suggest that zonisamide monotherapy is effective for patients with PKD as a first-line treatment. We can evaluate the efficacy of zonisamide therapy within one week. Because zonisamide lacks the enzyme-inducing effects of carbamazepine and phenytoin, it may be useful for PKD treatment.
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http://dx.doi.org/10.1016/j.pediatrneurol.2020.06.017DOI Listing
October 2020

Novel Cardiocerebral Channelopathy Associated with a KCND3 V392I Mutation.

Int Heart J 2020 Sep 12;61(5):1049-1055. Epub 2020 Sep 12.

Department of Cardiovascular Medicine, Gunma University Graduate School of Medicine.

While a KCND3 V392I mutation uniquely displays a mixed electrophysiological phenotype of Kv4.3, only limited clinical information on the mutation carriers is available. We report two teenage siblings exhibiting both cardiac (early repolarization syndrome and paroxysmal atrial fibrillation) and cerebral phenotypes (epilepsy and intellectual disability), in whom we identified the KCND3 V392I mutation. We propose a link between the KCND3 mutation with a mixed electrophysiological phenotype and cardiocerebral phenotypes, which may be defined as a novel cardiocerebral channelopathy.
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http://dx.doi.org/10.1536/ihj.20-203DOI Listing
September 2020

A recurrent TMEM106B mutation in hypomyelinating leukodystrophy: A rapid diagnostic assay.

Brain Dev 2020 Sep 25;42(8):603-606. Epub 2020 Jun 25.

Department of Biochemistry, Hamamatsu University School of Medicine, Hamamatsu, Japan.

Introduction: Hypomyelinating leukodystrophies (HLDs) are genetically heterogeneous syndromes, presenting abnormalities in myelin development in the central nervous system. Recently, a recurrent de novo mutation in TMEM106B was identified to be responsible for five cases of HLD. We report the first Japanese case of TMEM106B gene mutation.

Case Study: A 3-year-old patient presented with nystagmus and muscle hypotonia in his neonatal period, followed by delayed psychomotor development. Brain magnetic resonance images showed delayed myelination. Wave III and subsequent components were not presented by his auditory brainstem response. These features were similar to those observed in Pelizaeus-Merzbacher disease (PMD).

Methods: Proteolipid protein 1 (PLP1) gene screening, Mendelian disease panel exome, and whole-exome sequencing (WES) were sequentially performed.

Results: After excluding mutations in either PLP1 or other known HLD genes, WES identified a mutation c.754G > A, p.(Asp252Asn) in TMEM106B, which appeared to occur de novo, as shown by Sanger sequencing and SalI restriction enzyme digestion of PCR products.

Discussion: This is the sixth case of HLD with a TMEM106B mutation. All six cases harbored the same variant. This specific TMEM106B mutation should be investigated when a patient shows PMD-like features without PLP1 mutation. Our PCR-SalI digestion assay may serve as a tool for rapid HLD diagnosis.
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http://dx.doi.org/10.1016/j.braindev.2020.06.002DOI Listing
September 2020

High-power, frontal-dominant ripples in absence status epilepticus during childhood.

Clin Neurophysiol 2020 06 19;131(6):1204-1209. Epub 2020 Mar 19.

Division of Neurology, Saitama Children's Medical Center, 2-1 Shin-toshin, Chuou-ku, Saitama-city, Saitama 330-8777, Japan; Department of Pediatrics, Jikei University School of Medicine, 3-19-18 Nishi-shinbashi, Minato-ku, Tokyo 105-8471, Japan.

Objective: Absence status epilepticus (ASE) is a form of non-convulsive status epilepticus characterized by ongoing or intermittent epileptic activity accompanied by behavioral and cognitive changes. Herein, we assessed high-frequency oscillations in the ripple band in patients with ASE and typical absence seizures.

Methods: We enrolled five patients with ASE, 26 patients with childhood absence epilepsy (CAE), and 15 patients with juvenile absence epilepsy (JAE). We performed time-frequency analysis of electroencephalogram data for ictal absence seizures at each electrode to assess the high frequency activity (HFA) rate, peak frequency, and peak power.

Results: The average HFA rates were 60.7%, 20.8%, and 12.9% in ASE, CAE, and JAE patients, respectively. The average peak frequencies were 126.4 Hz, 120.9 Hz, and 126.1 Hz in ASE, CAE, and JAE patients, respectively. The average peak power values were 2,388.8 μV, 120.9 μV, and 126.1 μV in ASE, CAE, and JAE patients, respectively, and all epilepsy groups exhibited frontal-dominant ripple distribution.

Conclusion: ASE patients presented higher power and frontal dominant ripples of absence seizure, compared to CAE and JAE patients.

Significance: Future studies should utilize scalp-recorded ripples as a biomarker of absence epilepsy. This may aid in the development of novel treatment strategies for ASE.
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http://dx.doi.org/10.1016/j.clinph.2020.02.024DOI Listing
June 2020

Use of Perampanel and a Ketogenic Diet in Nonketotic Hyperglycinemia: A Case Report.

Neuropediatrics 2020 12 16;51(6):417-420. Epub 2020 Mar 16.

Division of Medical Genetics, Saitama Children's Medical Center, Saitama-City, Saitama, Japan.

Background: Nonketotic hyperglycinemia is a severe form of early onset epileptic encephalopathy caused by disturbances in the glycine cleavage system; the neurological damage is mainly attributed to overstimulation of the -methyl-D-aspartate receptor.

Case: The patient presented with a severe form of nonketotic hyperglycinemia and experienced frequent epileptic spasms and focal seizures, which were resistant to vigabatrin, adrenocorticotropic hormone therapy, and combined dextromethorphan and sodium benzoate treatments. By 9 months of age, perampanel reduced epileptic spasms by >50%. At 14 months of age, the ketogenic diet markedly reduced focal seizures and glycine levels in the cerebrospinal fluid.

Conclusion: Perampanel reduced fast excitatory neuronal activity, which was induced by an α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid receptor, followed by prolonged electrical depolarizations due to an -methyl-D-aspartate receptor. Furthermore, the ketogenic diet may have modulated the excessive neurotoxic cascade through the -methyl-D-aspartate receptor. Perampanel and ketogenic diet were effective for seizure control in our patient.
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http://dx.doi.org/10.1055/s-0040-1708536DOI Listing
December 2020

Clinical features and electroclinical evolution in 22 cases with epileptic spasms without hypsarrhythmia.

Epileptic Disord 2020 Feb;22(1):73-82

Division of Neurology, Saitama Children's Medical Center.

This study aimed to investigate the general presentation of epileptic spasms without hypsarrhythmia (ESwoH) and retrospectively determine whether there are differences in treatment effects related to ACTH therapy, long-term seizure outcome, and evolution of EEG features according to pre-treatment EEG patterns. According to the pattern of background activity, we divided our cohort into two groups: Group 1: normal background activity or with localized intermittent slow waves; Group 2: intermittent slow waves appearing generalized or in two or more lobes. Subjects included 22 children (Group 1: n=10; Group 2: n=12) diagnosed with ESwoH who received treatment from 2007 to 2017. The median age at onset of epileptic spasms was 5.5 months and the follow-up period lasted for 40.5 months. ACTH therapy was performed for seven patients from Group 1 and eight patients from Group 2. Only one patient from Group 2 responded to ACTH. Patients receiving effective treatments at early stages had excellent seizure outcome. Refractory cases included six patients in Group 1 and eight patients in Group 2; subsequent follow-up EEGs indicated hypsarrhythmia in one patient in Group 1 (17%) and six patients (75%) in Group 2, including one patient whose EEG pattern indicated progression to Lennox-Gastaut syndrome. Overall, ACTH is ineffective for patients with epileptic spasms without hypsarrhythmia. The EEG may indicate possible future development of hypsarrhythmia if epileptic spasms are resistant to treatment, especially in patients with diffuse slow waves on pre-treatment EEG. The efficacy of treatment introduced at early stages from onset may predict long-term seizure outcome.
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http://dx.doi.org/10.1684/epd.2020.1130DOI Listing
February 2020

Predictors of recurrent febrile seizures during the same febrile illness in children with febrile seizures.

J Neurol Sci 2020 Apr 13;411:116682. Epub 2020 Jan 13.

Division of Neurology, Saitama Children's Medical Center, Saitama, Japan.

Febrile seizures (FS) are common in childhood. Of children who experience an FS, 14-24% experience recurrence within 24 h, during the same febrile illness (RFS). The aim of this pilot study was to identify the predictors of RFS among children who experience FS. We conducted a retrospective cohort study of children aged 6-60 months, who visited the emergency department (ED) at Atsugi City Hospital in Japan for treatment of an FS between December 1, 2018 and February 28, 2019. Exclusion criteria included multiple seizures before visiting the ED, diazepam administration before visiting the ED or on departure, seizures lasting >15 min, underlying diseases such as epilepsy, and absence of laboratory test results. The primary outcome was RFS. Fifty-one patients fulfilled the inclusion criteria, of whom nine (17.6%) had RFS. The incidence of RFS was significantly higher in children with a body temperature ≤ 39.8 °C during the ED visit (P = .01). The combination of male sex and a body temperature ≤ 39.8 °C had a sensitivity, specificity and negative predictive value of 88.9%, 76.2%, and 97.0%, respectively. In conclusion, the incidence of RFS was 17.6%. The major predictors of RFS were male sex and a body temperature ≤ 39.8 °C.
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http://dx.doi.org/10.1016/j.jns.2020.116682DOI Listing
April 2020

Predictive factors of first dosage intravenous immunoglobulin-related adverse effects in children.

PLoS One 2020 13;15(1):e0227796. Epub 2020 Jan 13.

Department of Pediatrics, The Jikei University School of Medicine, Tokyo, Japan.

Background: Intravenous immunoglobulin (IVIG) therapy is used in the treatment of various diseases, and IVIG-related adverse effects (IVIG-AEs) vary from mild to severe. However, the mechanisms underlying IVIG-AEs and the potential predictive factors are not clear. This study investigated whether certain IVIG-AEs can be predicted before IVIG administration.

Study Design And Methods: This retrospective cohort study at the Division of Neurology, Saitama Children's Medical Center included patients enrolled from 2008 to 2018 who were < 18 years old and received IVIG for the first time. IVIG-AEs were classified according to the Common Terminology Criteria for Adverse Events version 5.0.

Results: A total of 104 patients fulfilled the inclusion criteria. The rate of IVIG-AEs was 37.5% (39/104). The most frequent IVIG-AEs were fever (41.0% [16/39]) and headache (38.5% [15/39]). AEs were below grade 2 in all except one patient and there were no grade 4 AEs. High serum total protein (TP) level was significantly related to the occurrence of IVIG-AEs (odds ratio, 14.8; 95% confidence interval, 2.4-90.5; P < 0.01). The optimal cutoff TP level was 6.7 g/dL. Although low WBC count and immunoglobulin G level may be predictive risk factors of IVIG-AEs, it was not confirmed in this study.

Conclusion: IVIG-AEs occurred in 37.5% of cases, and most were mild. TP was the best predictive risk factor of IVIG-AEs before IVIG administration. These results may aid in elucidating the mechanism underlying IVIG-AEs.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0227796PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6957294PMC
April 2020

Serum matrix metallopeptidase-9 and tissue inhibitor of metalloproteinase-1 levels in autoimmune encephalitis.

Brain Dev 2020 Mar 13;42(3):264-269. Epub 2019 Dec 13.

National Epilepsy Center, NHO Shizuoka Institute of Epilepsy and Neurological Disorders, 886, Urushiyama, Aoi-ku, Shizuoka, Japan.

Objective: Some pediatric patients with autoimmune encephalitis (AE) experience sequelae in spite of immunotherapy. In this study, we aimed to evaluate the association of serum matrix metallopeptidase-9 (MMP-9) and tissue inhibitor of metalloproteinase-1 (TIMP-1) levels with the neurological prognosis of AE.

Methods: We retrospectively included 13 patients with AE who had been referred to Saitama Children's Medical Center from February 2011 to May 2019. We compared serum MMP-9 levels, TIMP-1 levels, and MMP-9/TIMP-1 ratio in the acute period (within 30 days from the onset of AE) and subacute period (30-day period following the acute period). We also compared these biomarker levels between patients with (group A) and without sequelae (group B). Sequelae were evaluated at discharge or the last visit.

Results: Group A (median age, 7.8 years; range, 5.3-10.7 years) and group B (median age, 13.3 years; range, 11.1-15.4 years) had 6 patients each; 1 patient was excluded because the time of AE onset was unknown. In the acute period, there were no significant differences in MMP-9 levels, TIMP-1 levels, and MMP-9/TIMP-1 ratio between groups A and B. In the subacute period, serum MMP-9/TIMP-1 ratio was higher in group A than in group B (p < 0.01). There were no significant differences in MMP-9 and TIMP-1 levels between groups A and B.

Conclusions: Patients with sequelae of AE showed a high MMP-9/TIMP-1 ratio in the subacute period. Our study demonstrates that elevation of serum MMP-9/TIMP-1 ratio in the subacute period may be a predictive factor of sequelae of AE.
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http://dx.doi.org/10.1016/j.braindev.2019.11.010DOI Listing
March 2020

Efficacy and serum concentrations of perampanel for treatment of drug-resistant epilepsy in children, adolescents, and young adults: comparison of patients younger and older than 12 years.

Seizure 2019 Dec 5;73:75-78. Epub 2019 Nov 5.

Department for Child Health and Human Development, Saitama Children's Medical Center, 2-1 Shin-toshin, Chuou-ku, Saitama-city, Saitama 330-8777, Japan.

Purpose: Perampanel (PER) is a selective, non-competitive antagonist of the alpha-amino-3-hydroxy-5-methyl-4-isoxazole-propionic acid (AMPA) receptor. In Japan, PER is approved for patients with epilepsy who are at least 12 years old for the adjunctive treatment of primary generalised tonic-clonic seizures and partial-onset seizures (with or without secondary generalization). We surveyed the efficacy, adverse effects, and serum concentrations of PER, focusing especially on patients younger than 12 years of age.

Methods: We retrospectively surveyed the clinical information of patients treated with PER and assessed the efficacy at 6 months after treatment initiation. We compared efficacy, adverse effects, and serum concentration in patients younger or older than 12 years of age. Responders were defined as those who experienced a ≥50% seizure reduction.

Results: Eighty-four patients were enrolled. The average age of the younger group was 7.1 ± 3.3 (standard deviation) years compared to 16.4 ± 3.7 years in the older group. The responder rate was 42.9% (36/84). The responder rate did not differ between the two age groups (<12 years, 20/44, 45.4%; >12 years, 16/40, 40.0%; p = 0.78). The younger age group had a significantly lower concentration-to-dose (CD) ratio than the older age group (<12 years, 1849.8 ± 2209.3; >12 years, 3076.3 ± 3352.2, p = 0.02). Treatment-emergent adverse events (TEAEs) were observed in 22.6% (19/84) of patients, with the most common being somnolence (8/84, 9.5%).

Conclusion: PER may be an alternative to treat seizures in paediatric drug-resistant epilepsy. Serum concentrations of PER might be lower in patients younger than 12 years than in older patients.
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http://dx.doi.org/10.1016/j.seizure.2019.10.023DOI Listing
December 2019

Phenotype-genotype correlations in patients with GNB1 gene variants, including the first three reported Japanese patients to exhibit spastic diplegia, dyskinetic quadriplegia, and infantile spasms.

Brain Dev 2020 Feb 15;42(2):199-204. Epub 2019 Nov 15.

Department of Pediatric Neurology, Miyagi Children's Hospital Hospital, Sendai 989-3126, Japan. Electronic address:

We report the first three Japanese patients with missense variants in the GNB1 gene. Patients exhibited severe dyskinetic quadriplegia with cortical blindness and epileptic spasms, West syndrome (but with good outcomes), and hypotonic quadriplegia that later developed into spastic diplegia. Whole-exome sequencing revealed two recurrent GNB1 variants (p.Leu95Pro and p.Ile80Thr) and one novel variant (p.Ser74Leu). A recent investigation revealed large numbers of patients with GNB1 variants. Functional studies of such variants and genotype-phenotype correlation are required to enable future precision medicine.
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http://dx.doi.org/10.1016/j.braindev.2019.10.006DOI Listing
February 2020

The effect of the guidelines for management of febrile seizures 2015 on clinical practices: Nationwide survey in Japan.

Brain Dev 2020 Jan 8;42(1):28-34. Epub 2019 Oct 8.

Working Group for Guidelines for Management of Febrile Seizures, Japanese Society of Child Neurology, Nagoya, Japan; Faculty of Health and Medical Sciences, Tokoha University Hamamatsu Campus, Hamamatsu, Japan.

Objective: To investigate the effect of guidelines for management of febrile seizures on the clinical practice, we conducted a nationwide survey in Japan.

Methods: The Japanese guidelines for management of febrile seizures 2015 (GL2015) was released in 2015. In 2016, a questionnaire was sent to all 512 certified hospitals (3 pediatricians each) of the Japan Pediatric Society and all 47 prefecture Pediatric Associations (10 private pediatricians each) in Japan asking about management policies for febrile seizures (FSs) during 2013-2014 and 2016. The questionnaires were about the following procedures: (1) lumbar punctures, blood examinations, and diazepam suppositories for children after a first simple FS at emergency departments; and (2) prophylactic diazepam during febrile illnesses in children with two or three past simple FSs, with no known predictors of recurrence.

Results: A total of 1327 pediatricians (66.2%) answered the questionnaire. Numbers of pediatricians performing lumbar punctures and blood examinations, and giving diazepam suppositories after a first simple FS were less in 2016 than in 2013-2014 (1.2% and 2.0%, 53.1% and 61.3%, and 36.7% and 51.9%, respectively). Pediatricians recommending prophylactic diazepam for children with two and three FSs decreased from 45.7% and 82.4% in 2013-2014 to 31.0% and 65.0% in 2016, respectively.

Conclusion: GL2015 had an effect on the clinical practices of pediatricians. On the other hand, 65% recommended prophylactic diazepam to children with three simple FSs even though GL2015 did not recommend use of diazepam based on number of previous FS. Anxiety about frequent seizures may affect pediatricians' clinical practice.
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http://dx.doi.org/10.1016/j.braindev.2019.08.009DOI Listing
January 2020

Understanding of and misunderstandings regarding epilepsy: A survey of teachers in schools for special needs education in Japan.

Epilepsy Behav 2019 07 28;96:160-164. Epub 2019 May 28.

Division of Neurology, Saitama Children's Medical Center, Saitama, Japan.

The present study surveyed the understanding of epilepsy and attitudes toward epilepsy among teachers in Japanese schools for special needs education. An adapted version of a self-reported questionnaire, consisting of 27 questions, was sent to 3474 teachers at all schools for special needs education in Saitama Prefecture. The response rate to the questionnaire was 62%, corresponding to 2109 valid responses. The responses to three basic questions were as follows: 99% had heard about "epilepsy", 90% had seen epileptic seizures, and 63% had an acquaintance who had epilepsy. Numerous questionnaire items gave low correct answer rates for knowledge- and attitude-related questions. Correct responses to knowledge questions and a positive attitude toward epilepsy were associated with whether individuals had acquaintance with epilepsy, experience seeing a seizure, and read or heard about epilepsy. There was skepticism and uncertainty about whether people with epilepsy should be allowed to drive, likely due to vague fears of the risks of driving with epilepsy. According to our results, knowledge about epilepsy does not necessarily lead to a positive attitude. However, respondents who had acquaintances with epilepsy were most likely to demonstrate a positive attitude. Thus, familiarity is an essential factor in the likelihood of having a positive attitude toward epilepsy. Therefore, it is essential for teachers to understand how to manage and respond to epilepsy. There were a variety of concerns related to people with epilepsy driving. This finding highlights the importance of disseminating correct information on the risks of driving with epilepsy.
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http://dx.doi.org/10.1016/j.yebeh.2019.04.023DOI Listing
July 2019

Perampanel in lissencephaly-associated epilepsy.

Epilepsy Behav Case Rep 2019 11;11:67-69. Epub 2019 Jan 11.

Department for Child Health and Human Development, Saitama Children's Medical Center, 2-1 Shin-toshin, Chuou-ku, Saitama-city, Saitama 330-8777, Japan.

We retrospectively investigated whether perampanel (PER) could serve as an alternative for treating drug-resistant seizures in lissencephaly. We investigated the following data: age at onset of epilepsy, age at start of PER, etiology, brain MRI findings, seizure type, seizure frequency, adverse effects, and concomitant anti-epileptic drugs. There were 5 patients with lissencephaly, including 2 with Miller-Dieker syndrome. Four out of five patients exhibited ≥ 50% seizure reduction. Myoclonic seizures disappeared in 1 patient. PER was an effective adjunctive anti-seizure drug in our series of patients with lissencephaly.
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http://dx.doi.org/10.1016/j.ebcr.2019.01.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6351285PMC
January 2019

Efficacy and safety of pyridoxal in West syndrome: A retrospective study.

Brain Dev 2019 May 6;41(5):413-419. Epub 2018 Dec 6.

Division of Neurology, Saitama Children's Medical Center, 1-2, Shintoshin, Chuo-ku, Saitama, Japan. Electronic address:

Objective: To evaluate the efficacy and safety of pyridoxal for treating West syndrome.

Methods: We retrospectively investigated pyridoxal's efficacy and safety in 117 patients with West syndrome at Saitama Children's Medical Center from July 1993 to May 2016. Pyridoxal was administered at doses of 10-50 mg/kg/day. We evaluated seizure outcomes and electroencephalographic findings at 4 weeks after pyridoxal therapy. The responders were those with complete cessation of spasms for more than 4 weeks and those with resolution of hypsarrhythmia on EEG at 1-4 weeks after pyridoxal therapy.

Results: Five of the 117 patients (4.3%) were responders. The median duration between pyridoxal therapy to spasm cessation was 6 (5-13) days. Among the responders, four had hypsarrhythmia resolution, no spasm relapse, and no other seizure types more than 2 years after pyridoxal therapy. One responder had partial seizures and spasm relapse. No serious adverse effects occurred. There were no significant differences in sex, etiologies, complication, other seizure types preceding the spasms, onset age of spasms, age of pyridoxal therapy, treatment lag, initial and maintenance doses of pyridoxal, and adverse effects between pyridoxal responders and non-responders.

Conclusions: The efficacy rate of pyridoxal monotherapy as first-line treatment for West syndrome was low. However, pyridoxal therapy showed a rapid response within 1 week and was safe. We consider pyridoxal therapy as a kind of challenge therapy during the evaluation period concerning differential diagnosis and etiologies of West syndrome and immunological risks before adrenocorticotrophic hormone therapy or vigabatrin therapy.
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http://dx.doi.org/10.1016/j.braindev.2018.11.010DOI Listing
May 2019

Efficacy, safety, and pharmacokinetics of intravenous midazolam in Japanese children with status epilepticus.

J Neurol Sci 2019 01 4;396:150-158. Epub 2018 Oct 4.

Department of Pediatrics, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, Japan. Electronic address:

Background: No dosing regimen has been established for the initial treatment of pediatric status epilepticus with intravenous midazolam. We therefore evaluated the efficacy, safety, and pharmacokinetics of bolus and continuous midazolam infusion.

Methods: This open-label, prospective, multicenter study involved 34 Japanese children with status epilepticus unresponsive to diazepam. An initial bolus of 0.15 mg/kg midazolam was given, with additional doses of 0.1-0.3 mg/kg up to a cumulative dose of 0.6 mg/kg. A continuous infusion was initiated at 0.1 mg/kg/h (maximum 0.4 mg/kg/h) for patients at high risk of recurrence or in whom seizure reduction was achieved, and continued for 24 h after seizure cessation. Seizure cessation was assessed based on clinical observation (disappearance of motor symptoms regardless of recovery of consciousness), rather than the disappearance of electroencephalography abnormalities.

Results: The seizure cessation rate with bolus midazolam was 88%. The cumulative dose was ≤0.3 mg/kg in 90% of patients who responded to bolus administration. Adverse events were observed in three patients; one had mild respiratory depression that required supplemental oxygen and bag-valve-mask ventilation. Elimination half-life was 0.999 ± 0.241 h in seven patients. Total body clearance ranged from 423 to 1220 mL/h/kg in older children but was notably higher in a 10-month-old infant (2010 mL/h/kg).

Conclusions: The efficacy and safety of midazolam were demonstrated in children with status epilepticus, suggesting that intravenous midazolam is suitable as first-line treatment.
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http://dx.doi.org/10.1016/j.jns.2018.09.035DOI Listing
January 2019

Quantitative evaluation of regional cerebral blood flow changes during childhood using I-N-isopropyl-iodoamphetamine single-photon emission computed tomography.

Brain Dev 2018 Nov 29;40(10):841-849. Epub 2018 Jun 29.

Division of Neurology, Saitama Children's Medical Center, 1-2 Shintoshin, Chuo-ku, Saitama-city, Saitama 330-8777, Japan; Department of Pediatrics, The Jikei University School of Medicine, 3-19-18 Nishi-shinbashi, Minato-ku, Tokyo 105-8461, Japan.

Objective: To quantitatively evaluate regional cerebral blood flow (rCBF) and regional developmental changes during childhood using I-N-isopropyl-iodoamphetamine single-photon emission computed tomography (SPECT) and autoradiography.

Methods: We retrospectively analyzed quantitative values of rCBF in 75 children (29 girls) aged between 16 days and 178 months (median: 12 months), whose brain images, including magnetic resonance imaging and SPECT data, were normal under visual inspection at Saitama Children's Medical Center between 2005 and 2015. The subjects had normal psychomotor development, no focal neurological abnormalities, and neither respiratory nor cardiac disease at the time of examination. Regions of interest were placed automatically using a three-dimensional stereotactic template.

Results: rCBF was lowest in neonates, who had greater rCBF in the lenticular nucleus, thalamus, and cerebellum than the cerebral cortices. rCBF increased rapidly during the first year of life, reaching approximately twice the adult levels at 8 years, and then fell to approximately adult levels in the late teenage years. Cerebral cortex rCBF sequentially increased in the posterior, central, parietal, temporal, and callosomarginal regions during infancy and childhood.

Conclusions: rCBF changed dramatically throughout childhood and ranged from lower than adult values to approximately two times higher than adult values. It had different trajectories in each region during brain development. Understanding this dynamic developmental change is necessary for SPECT image evaluation in children.
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http://dx.doi.org/10.1016/j.braindev.2018.06.008DOI Listing
November 2018

Biallelic loss-of-function UBA5 mutations in a patient with intractable West syndrome and profound failure to thrive.

Epileptic Disord 2018 Aug;20(4):313-318

Department of Pediatrics, Showa University School of Medicine, 1-5-8, Hatanodai, Shinagawa-ku, Tokyo, Japan.

Mutation of the gene encoding ubiquitin-like modifier-activating enzyme 5 (UBA5) causes autosomal recessive early-onset epileptic encephalopathy. UBA5 acts as an E1-activating enzyme in the ubiquitin-fold modifier 1 pathway, which is important for unfolded protein elimination and regulation of apoptosis, and has been linked to human diseases. We identified biallelic mutations in UBA5 in a Japanese boy with intractable West syndrome, profound failure to thrive, and severe cerebral and cerebellar atrophy. The boy presented with epileptic spasms and hypsarrhythmia at the age of three months. He was diagnosed with West syndrome, however, treatments with adrenocorticotropic hormone and several antiepileptic drugs were ineffective. MRI findings were initially normal, but subsequently showed a progression of cerebellar and cerebral atrophy. By the age of seven years, he had not achieved any developmental milestones; he had daily epileptic spasms and tonic seizures and profound failure to thrive. Gene analysis revealed novel compound heterozygous mutations in UBA5; a microdeletion encompassing the entire UBA5 gene and a putative disease-causing missense mutation in the catalytic domain. These biallelic variants may have caused loss of function, accounting for the observed clinical symptoms. Intractable infantile epileptic spasms, failure to thrive, and severe neurological impairment may be characteristic of patients with UBA5 mutations.
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http://dx.doi.org/10.1684/epd.2018.0981DOI Listing
August 2018

Enhancement and bilateral synchronization of ripples in atypical benign epilepsy of childhood with centrotemporal spikes.

Clin Neurophysiol 2018 09 6;129(9):1920-1925. Epub 2018 Jul 6.

Division of Neurology, Saitama Children's Medical Center, 2-1Shin-toshin, Chuou-ku, Saitama-city, Saitama 330-8777, Japan.

Objective: To determine whether the characteristics of scalp-recorded high frequency oscillations, especially ripples, can predict the "atypical forms" of benign epilepsy of childhood with centrotemporal spikes (ABECTS), in BECTS.

Methods: Seven patients with ABECTS and eighteen patients with BECTS underwent electroencephalography (EEG) in the secondary bilateral synchrony (SBS) and non-SBS periods for ABECTS patients. SBS period is that when more than 50% of the interictal epileptiform discharges (IEDs) are bilaterally synchronized. We determined the IED-ripple co-occurrence rate, performed time frequency analysis, and calculated the asymmetry index (AI).

Results: The IEDs-ripple co-occurrence rate increased in the SBS compared to the non-SBS period. Time frequency analysis showed higher high-frequency activity rate and peak power in the SBS than in the non-SBS period. The AI was lower in ABECTS than BECTS, both in the non-SBS and SBS periods.

Conclusions: Ripples were enhanced in the SBS period of ABECTS, and bilaterally synchronized both in the non-SBS and SBS periods, whereas ripples in BECTS were localized unilaterally.

Significance: Bilaterally synchronized ripples in the non-SBS period of ABECTS may distinguish ABECTS from BECTS in the non-SBS period of IEDs, and may be helpful for early detection of progressive neurophysiological regression leading to early intervention.
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http://dx.doi.org/10.1016/j.clinph.2018.06.023DOI Listing
September 2018

Treatment of infantile spasms by pediatric neurologists in Japan.

Brain Dev 2018 Sep 3;40(8):685-692. Epub 2018 May 3.

Department for Child Health and Human Development, Saitama Children's Medical Center, Saitama, Japan.

Objective: To clarify changes in clinical practice for infantile spasms, including West syndrome, in Japan over the past two decades.

Methods: We investigated common treatment strategies for infantile spasms among 157 pediatric neurologists from a designated training facility for pediatric neurology and/or a designated training facility for epilepsy in Japan. A questionnaire was used to investigate use of adrenocorticotropic hormone (ACTH) therapy including daily dose, treatment duration, and tapering off period, and preferred first to fifth-line treatment choices.

Results: Among 119 responses (75.8%), 107 enabled analysis of ACTH therapy and 112 were used to determine preferred order of first to fifth-line treatments. Over 80% respondents reported an initial ACTH dose of ≤0.0125 mg/kg/day, with a treatment duration of 14 days and various tapering periods. Following an unfavorable response of seizures to ACTH, 80% respondents increased the dose and/or extended treatment duration. The same ACTH therapy regimen was performed for symptomatic and cryptogenic patients at 95 facilities (88.8%). Preferred orders of therapeutic agents were the same for both symptomatic and cryptogenic patients at 64 facilities (57.1%). Over half the respondents selected vitamin B6 or valproate as the first and second-line treatments instead of ACTH therapy, while ACTH therapy was the most frequently selected third-line treatment.

Conclusions: Current ACTH therapy regimens have lower doses and shorter durations than previously reported. However, treatment strategies for infantile spasms have not changed much in two decades. ACTH therapy should be the first/second-line treatment rather than third-line or later, especially for cryptogenic infantile spasms.
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http://dx.doi.org/10.1016/j.braindev.2018.04.006DOI Listing
September 2018

Maturational Changes of Gamma-Aminobutyric Acid A Receptors Measured With Benzodiazepine Binding of Iodine 123 Iomazenil Single-Photon Emission Computed Tomography.

Pediatr Neurol 2018 05 3;82:19-24. Epub 2018 Apr 3.

Department of Pediatrics, Jikei University School of Medicine, Tokyo, Japan.

Background: Iodine 123 (I-123) iomazenil is a specific ligand of the central benzodiazepine receptor, which is a part of the postsynaptic gamma-aminobutyric acid A receptor complex. We performed statistical image processing of I-123 iomazenil single-photon emission computed tomography to elucidate maturational changes in the GABAergic system.

Methods: Thirty patients (18 boys and 12 girls, aged 17 days to 14 years) with cryptogenic focal epilepsy were enrolled and underwent I-123 iomazenil single-photon emission computed tomography. We used a semiquantitative analytical method consisting of brain surface extraction, anatomic normalization, and a three-parameter exponential model. We then assessed developmental changes in benzodiazepine receptor binding activity in 18 regions of interest in both hemispheres.

Results: The highest benzodiazepine receptor binding activity was observed during early infancy in all regions of interest. Benzodiazepine receptor binding activity then decreased exponentially across development. Benzodiazepine receptor binding in the primary sensorimotor cortex, primary visual cortex, cerebellar vermis, and striatum declined more rapidly than that in the cerebellar hemispheres and the frontal cortex. The pons and the thalamus had the lowest benzodiazepine receptor binding activities during the neonatal period, and benzodiazepine receptor binding in these areas declined gradually after infancy toward adolescence. There were no differences in adjusted benzodiazepine receptor binding activity according to laterality or sex.

Conclusions: Benzodiazepine receptor binding activity decreased exponentially during infancy in all regions of interest. Binding activity in the primary somatosensory and motor cortices (M1 and S1), the primary and association visual areas, the cerebellar vermis, and the striatum (caudate nucleus and putamen) tended to decline more rapidly than that in the cerebellar hemisphere and the frontal association cortex.
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http://dx.doi.org/10.1016/j.pediatrneurol.2018.02.008DOI Listing
May 2018

First Patient With Salla Disease Confirmed by Genomic Analysis in Japan.

Pediatr Neurol 2018 04 31;81:52-53. Epub 2018 Jan 31.

Division of Medical Genetics, Saitama Children's Medical Center, Saitama, Japan.

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http://dx.doi.org/10.1016/j.pediatrneurol.2018.01.002DOI Listing
April 2018

Epilepsy with myoclonic atonic seizures and chronic cerebellar symptoms associated with antibodies against glutamate receptors N2B and D2 in serum and cerebrospinal fluid.

Epileptic Disord 2017 Mar;19(1):94-98

National Epilepsy Center Shizuoka Institute of Epilepsy and Neurological Disorders, NHO, 886, Urushiyama, Aoi-ku, Shizuoka, Japan.

A 3-year-old boy with normal development presented with acute cerebellitis at one year and 10 months of age. His truncal ataxia resolved without treatment. He experienced a relapse of truncal ataxia and atonic seizures at 2 years and one month of age. Five months later, he experienced myoclonic atonic seizures. By 3 years of age, the truncal ataxia had become severe, and the frequency of myoclonic atonic seizures increased. Compared to controls, we found higher levels of anti-C-terminal GluN2B and anti-N terminal GluD2 antibodies in the serum, and anti-N terminal GluN2B and anti-C terminal GluD2 antibodies in the cerebrospinal fluid (CSF). A cell-based assay revealed the presence of anti-NMDA-type glutamate receptor antibody in the serum, but absence in the CSF. Ictal EEG of myoclonic atonic seizures showed generalized spike and wave complexes. The patient was diagnosed with myoclonic atonic epilepsy. Adrenocorticotrophic hormone therapy resolved the truncal ataxia and myoclonic atonic seizures, along with the decreased serum anti-C-terminal GluN2B and anti-N-terminal GluD2 antibodies, and CSF anti-N-terminal GluN2B and anti-C-terminal anti-GluD2 antibodies. Our results suggest that the anti-GluN2B and anti-GluD2 antibodies may be associated with myoclonic atonic epileptic seizures and chronic cerebellitis.
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http://dx.doi.org/10.1684/epd.2017.0895DOI Listing
March 2017

Relationship between the change of language symptoms and the change of regional cerebral blood flow in the recovery process of two children with acquired aphasia.

Brain Dev 2017 Jun 31;39(6):493-505. Epub 2017 Jan 31.

Division of Neurology, Saitama Children's Medical Center, Japan.

Objective: This study aimed to investigate the relationship between the change of language symptoms and the change of regional cerebral blood flow (rCBF) in the recovery process of two children with acquired aphasia caused by infarctions from Moyamoya disease with an onset age of 8years.

Methods: We compared the results for the Standard Language Test of Aphasia (SLTA) with rCBF changes in 7 language regions in the left hemisphere and their homologous regions in the right hemisphere at 4 time points from 3weeks for up to 5years after the onset of aphasia, while controlling for the effect of age.

Results: In both cases, strong correlations were seen within a hemisphere between adjacent regions or regions that are connected by neuronal fibers, and between some language regions in the left hemisphere and their homologous regions in the right hemisphere. Conversely, there were differences between the two cases in the time course of rCBF changes during their recovery process.

Conclusion: Consistent with previous studies, the current study suggested that both hemispheres were involved in the long-term recovery of language symptoms in children with acquired aphasia. We suggest that the differences between both cases during their recovery process might be influenced by the brain states before aphasia, by which hemisphere was affected, and by the timing of the surgical revascularization procedure. However, the changes were observed in the data obtained for rCBF with strong correlations with the changes in language performance, so it is possible that rCBF could be used as a biomarker for language symptom changes.
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http://dx.doi.org/10.1016/j.braindev.2017.01.002DOI Listing
June 2017

Measurement of pyridoxal 5'-phosphate, pyridoxal, and 4-pyridoxic acid in the cerebrospinal fluid of children.

Clin Chim Acta 2017 Mar 28;466:1-5. Epub 2016 Dec 28.

Department of Child Neurology, Okayama University Hospital, Okayama, Japan; Department of Child Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.

Background: We quantified pyridoxal 5'-phosphate (PLP), pyridoxal (PL), and 4-pyridoxic acid (PA) in the cerebrospinal fluid (CSF) of children and to investigate the effect of age, sex, epilepsy, and anti-epileptic drug (AED) therapy on these vitamers.

Methods: CSF samples prospectively collected from 116 pediatric patients were analyzed. PLP, PL, and PA were measured using high-performance liquid chromatography with fluorescence detection, using pre-column derivatization by semicarbazide. Effects of age, sex, epilepsy, and AEDs on these vitamers and the PLP/PL ratio were evaluated using multiple linear regression models.

Results: The PLP, PL, and PA concentrations were correlated negatively with age and the PLP/PL ratio was correlated positively with age. Multiple regression analysis revealed that the presence of epilepsy was associated with lower PLP concentrations and PLP/PL ratios but sex and AED therapy had no influence on these values. The observed ranges of these vitamers in epileptic and non-epileptic patients were demonstrated.

Conclusions: We showed the age dependence of PLP and PL in CSF from pediatric patients. Epileptic patients had lower PLP concentrations and PLP/PL ratios than non-epileptic patients, but it is unknown whether this is the cause, or a result, of epilepsy.
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http://dx.doi.org/10.1016/j.cca.2016.12.027DOI Listing
March 2017

The clinical features of patients suffering from psychogenic non-epileptic seizures with or without epileptic seizures.

No To Hattatsu 2016 Nov;48(6):425-9

Objective: We investigated the clinical characteristics of pediatric psychogenic non-epileptic seizures (PNES). Methods: We studied 15 children and adolescents with PNES, who were divided into 3 groups : 1) a group with epilepsy (7 patients), 2) a group without epilepsy and mental retardation (MR) (7 patients), and 3) a group with MR (1 patient), according to the guideline for the diagnosis and treatment of PNES established by the Japan Epilepsy Society. Results: Remission of epilepsy and PNES was achieved in only 2 patients in the group with epilepsy. In the group without epilepsy and MR, antiepileptic drugs (AEDs) could be discontinued entirely in all the patients, however, the treatment for PNES could be completed in only one patient. Treatment of epilepsy and PNES could be completed in the one patient with MR. Conclusions: It is important for pediatric neurologists to explain the good news to the parents of children in the group without epilepsy or MR, that the patient does not have epilepsy and does not require treatment with AEDs. For the group with MR, understanding should be encouraged about the development of the child. Because treatment is difficult in the group with epilepsy and PNES, cooperation among the pediatric neurologists, pediatric psychiatrists and clinical psychologists is more important.
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November 2016

New guidelines for management of febrile seizures in Japan.

Brain Dev 2017 Jan 6;39(1):2-9. Epub 2016 Sep 6.

Faculty of Health and Medical Sciences, Tokoha University Hamamatsu Campus, Hamamatsu, Japan.

In 2015, the Japanese Society of Child Neurology released new guidelines for the management of febrile seizures, the first update of such guidelines since 1996. In 1988, the Conference on Febrile Convulsions in Japan published "Guidelines for the Treatment of Febrile Seizures." The Task Committee of the Conference proposed a revised version of the guidelines in 1996; that version released in 1996 was used for the next 19years in Japan for the clinical management of children with febrile seizures. Although the guidelines were very helpful for many clinicians, new guidelines were needed to reflect changes in public health and the dissemination of new medical evidence. The Japanese Society of Child Neurology formed a working group in 2012, and published the new guidelines in March 2015. The guidelines include emergency care, application of electroencephalography, neuroimaging, prophylactic diazepam, antipyretics, drugs needing special attention, and vaccines. While the new guidelines contain updated clinical recommendations, many unsolved questions remain. These questions should be clarified by future clinical research.
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http://dx.doi.org/10.1016/j.braindev.2016.06.003DOI Listing
January 2017