Japanese Society Of Child Neurology Research Articles

The 65th Annual Meeting of the Japanese Society of Child Neurology

The 65th Annual Meeting of the Japanese Society of Child Neurology

Severe pediatric acute encephalopathy syndromes related to SARS-CoV-2.

To clarify whether severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection cause acute encephalopathy in children and which are the most common syndromes that cause them and what are the outcomes. A nationwide web-based survey among all members of the Japanese Society of Child Neurology to identify pediatric patients aged < 18 years who developed acute encephalopathy in Japan between 1 January 2020 and 31 May 2022 associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection confirmed by polymerase chain reaction or antigen tests using pharyngeal swabs. Acute encephalopathy was defined as acute onset of impaired consciousness lasting > 24 h or an altered mental state; neurological symptoms arising within 2 weeks of onset of COVID-19 or multisystem inflammatory syndrome in children (MIS-C)/pediatric inflammatory multisystem syndrome (PIMS); evidence of SARS-CoV-2 infection; and reasonable exclusion of other diseases. Patients were divided into the known clinico-radiological acute encephalopathy syndrome group and unexplained or unclassifiable acute encephalopathy group. Outcomes were assessed by pediatric cerebral performance category (PCPC) score at hospital discharge. Of the 3,802 society members, 217 representing institutions responded, and 39 patients with suspected acute encephalopathy were reported, of which 31 met inclusion criteria. Of these patients, 14 were diagnosed with known clinico-radiological acute encephalopathy syndromes, with acute encephalopathy with biphasic seizures and late reduced diffusion (five patients) being the most common. Five developed acute encephalopathy associated with MIS-C/PIMS. Among 31 patients, 9 (29.0%) had severe sequelae or died (PCPC ≥ 4). Two of three patients with encephalopathy with acute fulminant cerebral edema and two with hemorrhagic shock and encephalopathy syndrome died. The PCPC scores were higher in the known clinico-radiological acute encephalopathy syndrome group than in the unexplained or unclassifiable acute encephalopathy group (P < 0.01). Acute encephalopathy related to SARS-CoV-2 infection was demonstrated to be more severe than that caused by other viruses in Japan. Acute encephalopathy syndromes characterized by specific neuroradiological findings was associated with poor clinical outcomes.

The Japanese Society of Child Neurology from now onward

The Japanese Society of Child Neurology from now onward

Changes in the treatment of pediatric acute encephalopathy in Japan between 2015 and 2021: A national questionnaire-based survey

BackgroundAlthough acute encephalopathy (AE) is the most serious disorder associated with a viral infection in childhood and often causes death or neurological sequelae, standard treatments have not been established. In 2016, the Japanese Society of Child Neurology published the “Guidelines for the Diagnosis and Treatment of Acute Encephalopathy in Childhood 2016” (AE GL 2016). We conducted a questionnaire survey to evaluate the status of the treatment of pediatric AE in 2021 and the changes in treatment before and after the publication of the AE GL 2016. MethodsIn October 2021, questionnaires were mailed via the web to members of two mailing lists who were involved in the practice of pediatric neurological disorders. ResultsMost Japanese physicians (98 %) engaged in the treatment of pediatric AE used the AE GL 2016 as a clinical reference. From 2015 to 2021, the number of institutions that implemented targeted temperature management (TTM), vitamin administration, and continuous electroencephalographic monitoring increased significantly. Regarding the targeted temperature for TTM, the proportion of patients who were treated with normothermia (36.0–37.0 °C) increased from 2015 (55 %) to 2021 (79 %). The use of corticosteroids in patients with AE caused by a cytokine storm, which is recommended in the AE GL 2016, had already been implemented in most institutions by 2015. ConclusionThe AE GL 2016 could be used to disseminate the knowledge accumulated to date. Evidence of the efficacy and proper indication criteria for the treatment of AE is insufficient and must be further accumulated.

Perspective on transition from pediatric to adult health care for patients with neurological disease: current situation and issues

An improvement in efficacy treatment and development of the social support system has led to many patients with neurological disease being able to reach adulthood. Therefore health care for life from pediatrics to adulthood has become necessary. The Special Committee for Measures Against Transition from Pediatric to Adult Health Care of the Japanese Society of Neurology officially started to examine the current situation and issues of transition from pediatric to adult health care in July 2020. Pediatric neurologists and adult neurologists have an awareness of this issue of constructing a better transition from pediatric to adult health care. However, there are some tasks that need to be resolved in the medical system. We intend to improve the understanding of transition and assessment of medical service fees for transition in cooperation with the Japanese Society of Neurology and the Japanese Society of Child Neurology.

Nationwide Survey of Childhood Guillain-Barré Syndrome, Fisher Syndrome, and Bickerstaff Brainstem Encephalitis in Japan

Background: Guillain-Barre syndrome (GBS), Fisher syndrome (FS), and Bickerstaff brainstem encephalitis (BBE) are immune-mediated neuropathies presenting with symptoms such as muscle weakness, ophthalmoplegia, ataxia, and consciousness disturbances. Although the epidemiology of GBS and BBE in patients of all ages has been reported, childhood data have not been well-investigated. We aimed to determine the clinical features, therapeutics, and prognoses of childhood GBS, FS, and BBE in Japan. Methods: We sent questionnaires to 1068 pediatric neurologists in Japan from 2014 to 2016 to determine the number of children less than 15 years old with GBS, FS, or BBE and their age and sex. We subsequently performed a secondary survey to investigate the clinical features, laboratory data, treatment, and prognosis. This study was approved by the local ethics committee of the Graduate School of Medicine, Chiba University. Findings: Five-hundred thirty-eight pediatric neurology specialists (50·4%) responded to the first survey. The total number of children with GBS, FS, and BBE in Japan from 2014 to 2016 were 87, 10, and 6, respectively, suggesting annual incidence rates of was 0·19, 0·023, and 0·0071 per 100,000 children, respectively, in the pediatric population (0-14 years). GBS was classified as acute inflammatory demyelinating neuropathy (39·7%), acute motor axonal neuropathy (24·4%), or acute motor-sensory axonal neuropathy (11·5%), with a male-to-female ratio of 1·29:1·0 and a wide distribution of onset ages. The disease severities of GBS, FS, and BBE were variable, but all children were able to walk within one year, indicating an excellent childhood prognosis. No in-hospital deaths occurred in this study. Interpretation: The prognoses of childhood GBS, FS, and BBE were generally favorable, as long as the patient was promptly treated with either intravenous immunoglobulin or plasma exchange. Clinical Trial Registration Details: The research scheme was registered at the UMIN Clinical Trials Registry (UMIN-CTR) as UMI18739. Funding Information: This work was supported by the institutional budget of the Chiba University Graduate School of Medicine (grant reference J09KF00284). Declaration of Interests: The authors declare no conflict of interest. Ethics Approval Statement: The study protocol was approved by the institutional ethics committee of the Graduate School of Medicine, Chiba University (No. 319), and the Collaborative Research Committee of the Japanese Society of Child Neurology (No. 13-04).

The reports of the collaborative studies supported by the Japanese Society of Child Neurology

The reports of the collaborative studies supported by the Japanese Society of Child Neurology

Call for abstracts for oral and poster presentations: The 63rd Annual Meeting of the Japanese Society of Child Neurology (JSCN 2021) “Ignite Evolution at the Dawn of a New Age”

Call for abstracts for oral and poster presentations: The 63rd Annual Meeting of the Japanese Society of Child Neurology (JSCN 2021) “Ignite Evolution at the Dawn of a New Age”

Relationship between Sedative Antihistamines and the Duration of Febrile Seizures.

Some studies have shown that sedative antihistamines prolong febrile seizure duration. Although the collective evidence is still mixed, the Japanese Society of Child Neurology released guidelines in 2015 that contraindicated the use of sedative antihistamines in patients with febrile seizure. Focused on addressing limitations of previous studies, we conducted a cross-sectional study to evaluate the relationship between febrile seizure duration and the use of sedative antihistamines. Data were collected from patients who visited St. Luke's International Hospital due to febrile seizure between August 2013 and February 2016. Patients were divided into groups based on their prescribed medications: sedative antihistamine, nonsedative antihistamine, and no antihistamine. Seizure duration was the primary outcome and was examined using multivariate analyses. Of the 426 patients included, sedative antihistamines were administered to 24 patients. The median seizure duration was approximately 3 minutes in all three groups. There was no statistical difference in the bivariate (p = 0.422) or multivariate analyses (p = 0.544). Our results do not support the relationship between sedative antihistamine use and prolonged duration of febrile seizure. These results suggest that the use of antihistamines may be considered for patients with past history of febrile seizure, when appropriate.

The reports of the collaborative studies supported by the Japanese Society of Child Neurology

The reports of the collaborative studies supported by the Japanese Society of Child Neurology

｢10年後の小児神経専門医のなすべきこと・30年後の小児神経学会の姿｣ の報告

｢10年後の小児神経専門医のなすべきこと・30年後の小児神経学会の姿｣ の報告

The reports of the collaborative studies supported by the Japanese Society of Child Neurology

The reports of the collaborative studies supported by the Japanese Society of Child Neurology

The reports of the collaborative studies supported by the Japanese Society of Child Neurology

The reports of the collaborative studies supported by the Japanese Society of Child Neurology

Meet the experts : Proposal on the collaborative studies supported by the Japanese Society of Child Neurology

Meet the experts : Proposal on the collaborative studies supported by the Japanese Society of Child Neurology

The reports of the collaborative studies supported by the Japanese Society of Child Neurology

The reports of the collaborative studies supported by the Japanese Society of Child Neurology

How Physicians Support Mothers of Children with Duchenne Muscular Dystrophy.

Communicating about Duchenne muscular dystrophy and its prognosis can be difficult for affected children and their family. We focused on how physicians provide support to the mothers of children with Duchenne muscular dystrophy who have difficulty communicating about the condition with their child. The eligible participants were certified child neurologists of the Japanese Society of Child Neurology. Participants responded to questionnaires consisting of free descriptions of a vignette of a child with Duchenne muscular dystrophy and a mother. We analyzed 263 responses of the participants. We found 4 themes on advising mothers, involving encouraging communication, family autonomy, supporting family, and considering the child's concerns. These results provide a better understanding of the communication between physicians and family members who need help sharing information with a child with Duchenne muscular dystrophy. These findings will assist clinical practitioners in supporting families and the affected children throughout the course of their illness.

Joint researches supported by Japanese Society of Child Neurology (JSCN) the committee of joint researches

Joint researches supported by Japanese Society of Child Neurology (JSCN) the committee of joint researches

てんかんのキャリーオーバーについての研究報告―神経内科医師へのアンケート結果―

We conducted a questionnaire survey on 7,500 members of the Societas Neurologica Japonica regarding the carry-over from pediatric to adult epilepsy care. The response rate was 16.9%. Analysis of the responses showed that 46% of the neurologists were aware of the term "carry-over" in epilepsy care, and 78% felt difficulties with epilepsy care in general. The most common reasons included "not familiar with interpreting EEG" and "not familiar with the laws, regulations and medical and welfare systems specific to epilepsy". Among the neurologists who felt difficulties when accepting epilepsy patients aged 20 years or older referred from the pediatric department, 68% had experienced accepting these patients. The two major reasons for feeling difficulties when accepting these patients were "difficult to have good understanding of the disease course from infancy" and "not familiar with the epilepsy syndrome specific to infancy". The above findings highlight the importance of recognizing the existence of the issue of carry-over in adult epilepsy care and its significance, and to resolve the factors that hinder the transition of care. To achieve this goal, joint activities of the Japanese Society of Child Neurology and the Japan Epilepsy Society in collaboration with the Societas Neurologica Japonica, the Japanese Society of Psychiatry and Neurology, and the Japan Neurosurgical Society are critical.

Professor Norimitsu Yoshikura (1902–1988). A leading Japanese authority in paediatric neurology

Dr. Norimitsu Yoshikura, Professor of Pediatrics, Surugadai Hospital, Nihon University School of Medicine, was born in Tokyo on 18th January, 1902. He first graduated from the Faculty of Literature, Waseda University, and thereafter from the Jikei University School of Medicine in 1933. He was a member of the committee of the Japanese Pediatric Neurology Association from 1964 to 1976, and an honorary member of the French Neurological Society. In 1966 he was president at the 6th annual meeting of the Japanese Society of Child Neurology. He retired from

Management of and prophylaxis against status epilepticus in children with severe myoclonic epilepsy in infancy (SMEI; Dravet syndrome) – A nationwide questionnaire survey in Japan

The aim of this study was to establish strategies for prophylaxis against status epilepticus (SE) associated with high fever and for management of ongoing SE in children with severe myoclonic epilepsy in infancy (SMEI). Methods: The investigation was performed retrospectively using a questionnaire, asking about medications, which was distributed to epilepsy specialists throughout Japan. All respondents were members of the Japan Epilepsy Society (JES) and/or the Japanese Society of Child Neurology (JSCN). Data from 109 SMEI patients (51 males and 58 females), 1–37 (M ± SD, 10.7 ± 6.53) years old, were used for this study. Of these 109 patients, 10 were excluded because they had not experienced SE, such that data from 99 patients were analyzed. Results: Among the anti-epileptic drugs (AEDs) used daily, excellent efficacy against SE evolution was obtained with the following: potassium bromide (KBr) (41.7%), zonisamide (ZNS) (13.5%), clobazam (CLB) (10.0%), valproate (VPA) (8.0%), phenobarbital (PB) (6.7%), and phenytoin (PHT) (2.6%). Excellent efficacy was not obtained with either clonazepam (CZP) or carbamazepine (CBZ). The diazepam (DZP) suppository was the most frequently given drug for prophylaxis against SE triggered by fever, but only 2 (2.4%) cases showed excellent results. Excellent efficacy in terminating ongoing SE was obtained with the following medications; intravenous barbiturates (75–100%), intravenous midazolam (MDZ) (68.8%), intravenous DZP (54.3%), intravenous lidocaine (Lid) (21.4%), and intravenous PHT (15.4%). Conclusions: Daily KBr was most efficacious for controlling seizures in SMEI patients. Early use of intravenous barbiturates is the most effective strategy in stopping SE in a subset of patients. Reliable efficacy in SE was not obtained with prophylactic DZP, intravenous benzodiazepines (BZPs), PHT and Lid.