[Palliative Care for Persons with Amyotrophic Lateral Sclerosis].

Palliative care in Japan is available mainly for patients with cancer, and palliative care specialists do not have sufficient experience with management of palliation in persons with amyotrophic lateral sclerosis (ALS). Treatment of ALS symptoms is an important component of palliative care, and it is important that neurologists and home care physicians familiarize themselves with palliative care for ALS in consultation with palliative care specialists. Notably, the use of opioids at the end of life differs from that of pain relief for cancer.

Clinical practice guidelines for multiple sclerosis, neuromyelitis optica spectrum disorder, and myelin oligodendrocyte glycoprotein antibody-associated disease 2023 in Japan.

Background: The previous Japanese clinical practice guidelines for multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) were published in 2017. Recently, for the first time in 6 years, the MS and NMOSD guideline development committee revised the Japanese guidelines for MS, NMOSD, and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).

Methods: The committee utilized the Grading of Recommendations Assessment, Development, and Evaluation system based on the "Minds Handbook for Clinical Practice Guideline Development 2020 Ver.

Deterioration of Cough, Respiratory, and Vocal Cord Functions in Patients with Multiple System Atrophy.

The purpose of this study was to clarify changes in cough function in patients with multiple system atrophy (MSA). Seventeen probable patients with MSA were studied. Peak cough flow (PCF), respiratory function (percentage of vital capacity, percentage of forced vital capacity, and percentage of predicted forced expiratory volume in one second), respiratory muscle strength (percentage of maximal inspiratory mouth pressure and percentage of maximal expiratory mouth pressure), and maximum phonation time (MPT) were assessed.

Effects of Rehabilitative Intervention for Augmenting Cough Function in Patients with Multiple System Atrophy.

Objectives: One of the causes of death in patients with multiple system atrophy (MSA) is aspiration pneumonia caused by cough dysfunction. This study aimed to identify an effective approach to improve coughing and to explore the establishment of criteria for the use of gastrostomy based on cough and respiratory dysfunctions.

Methods: Eighteen probable MSA patients participated in the study.

[Drugs for Amyotrophic Lateral Sclerosis].

Riluzole and edaravone for the treatment of amyotrophic lateral sclerosis (ALS) are currently covered by insurance in Japan. Both have been shown to prolong survival and/or inhibit progression, but neither is a cure-all treatment, and the effects are difficult to realize. The data presented in clinical trials are not applicable to all patients with ALS; the risks and benefits should be explained carefully before use.

[Video Works on Amyotrophic Lateral Sclerosis].

Amyotrophic lateral sclerosis (ALS) is known to many as a disease that is infrequent but causes progressive disability and death within three to five years if a tracheostomy ventilator are not used. The patient must choose whether to live with a crippled body or die, and is faced with the question of what kind of condition is considered "living". By watching a video that addresses this theme, we can grasp this question even more deeply.

Culturally Adapted Consensus Definition and Action Guideline: Japan's Advance Care Planning.

Context: A conceptual framework for advance care planning is lacking in societies like Japan's valuing family-centered decision-making.

Objectives: A consensus definition of advance care planning with action guideline adapted to Japanese society.

Methods: We conducted a multidisciplinary modified Delphi study 2020-2022.

Teaching "medical interview and physical examination" from the very beginning of medical school and using "escape rooms" during the final assessment: achievements and educational impact in Japan.

Background: There is no consensus regarding the best time to teach two fundamental pillars of clinical medicine: medical interview and physical examination. We investigated the impacts of teaching the course "Medical Interview and Physical Examination" in Japan from the very beginning of medical school. In addition, we also evaluated the educational value of using "Escape Rooms", a series of timed, game-based scenarios using simulators, as a part of the final assessment of the course.

Prescription pattern of anti-Parkinson's disease drugs in Japan based on a nationwide medical claims database.

Introduction: Parkinson's disease (PD) treatment should follow guidelines and be tailored to each patient. Large database analyses can provide insights into prescribing patterns.

Methods: Retrospective, cross-sectional study of patients (≥30 years) with PD diagnosis (ICD-10; schizophrenia/cerebrovascular disease excluded) using health insurance claims data (April 2008-December 2016) from the Japan Medical Data Vision database.

The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) for Japanese ALS and FTD patients.

Objective: Amyotrophic lateral sclerosis (ALS) patients might present with cognitive and behavioural abnormalities resembling frontotemporal dementia (FTD). The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) was developed as an easy to administer cognitive screen for detecting these symptoms. The aim of the present study was to develop and validate a Japanese version of the ECAS.

[Basics of Clinical Ethics].

Neurologists encounter patients who develop diseases that suddenly change their life one day, such as stroke, and diseases that are difficult to cure and progressive, such as intractable neurological diseases. There are many types of ethical issues, including the choice of medical intervention, caregiver conflict, genetic disease issues, end-of-life issues, etc. In this article, I will describe the matters advocated in general clinical ethics and discuss the interpretation and application in the field of neurology based on my own experience.

[Home Care for Cancer Patients and Outreach from the Hospital].

Japan is entering a multi-dying era. In this situation palliative care is increasingly important. More than half of Japanese state that they want to stay at home even when facing the end of life, especially in cancer area.

Adherence to treatment guideline recommendations for Parkinson's disease in Japan: A longitudinal analysis of a nationwide medical claims database between 2008 and 2016.

Background: Adherence to the 2011 Japanese guidelines for treatment of Parkinson's disease (PD) in real-life practice is unknown.

Methods: In this retrospective longitudinal observational study, we examined patterns and trends in anti-PD drug prescriptions in 20,936 patients (≥30 years of age with newly diagnosed PD [International Classification of Diseases-Tenth code G20 or PD Hoehn and Yahr scale 1-5] and one or more prescriptions) using nationwide registry data between 2008 and 2016. Data are presented as descriptive statistics.

Cognitive and behavioral status in Japanese ALS patients: a multicenter study.

Objectives: Amyotrophic lateral sclerosis (ALS) patients may present with cognitive and behavioral abnormalities similar to frontotemporal dementia (FTD). In this multicenter study we examined Japanese ALS patients with and without FTD in order to characterize the full extent of cognitive and behavioral abnormalities, including associations with functional motor status, anxiety and depression.

Methods: Patients were evaluated using the Montreal Cognitive Assessment (MoCA), Frontal Assessment Battery (FAB), Hospital Anxiety and Depression Scale, ALS Functional Rating Scale-Revised, spirometry, and verbal fluency tests.

Recent transition of medical cost and relapse rate of multiple sclerosis in Japan based on analysis of a health insurance claims database.

Background: In this study, we aimed to understand the trends in total and itemized medical expenses, especially of disease-modifying therapy (DMT), for multiple sclerosis (MS) in Japan through an analysis of health insurance claims data.

Methods: We analyzed a database containing health insurance claims data from hospitals that have adopted the Diagnosis Procedure Combination/Per-Diem Payment System in Japan. According to an algorithm based on diagnosis codes, data for all patients diagnosed with MS from April 2008 to July 2016 were extracted.

Treatment and comorbidities of multiple sclerosis in an employed population in Japan: analysis of health claims data.

Aim: Use real-world data to investigate the treatment, comorbidities and prevalence of multiple sclerosis (MS) in an employed Japanese population.

Methods: Data from the Japanese Medical Data Center health insurance claims database were analyzed (January 2005-November 2014). Patients with claims associated with an MS diagnosis were identified (n = 494) and comorbidities and prescribed treatments were investigated.

Prevalence, treatments and medical cost of multiple sclerosis in Japan based on analysis of a health insurance claims database.

Objective: To understand, through an analysis of health insurance claims data, the current treatment status and medical cost of multiple sclerosis (MS) in Japan.

Methods: We analyzed claims data (January 2005-January 2016) from the Japan Medical Data Center Co., Ltd.

Current treatment status and medical cost for multiple sclerosis based on analysis of a Japanese claims database.

Objective: To assist policymakers as they reflect on treatment protocols and approaches for the efficient delivery of medical care for multiple sclerosis (MS) patients in Japan.

Methods: We analyzed data from a large Japanese health insurance claims database. Using an algorithm based on diagnosis codes, all patients with a diagnosis of MS were identified; patients having a non-MS demyelinating disease were excluded from the population.

Japanese version of the ALS-FTD-Questionnaire (ALS-FTD-Q-J).

Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) share common clinical, genetic and neuropathological features. Some ALS patients have behavioral/personality changes, which could result in significant obstacles in the care provided by family members and caregivers. An easy screening tool would contribute greatly to the evaluation of these symptoms.

Bromocriptine Mesylate Attenuates Amyotrophic Lateral Sclerosis: A Phase 2a, Randomized, Double-Blind, Placebo-Controlled Research in Japanese Patients.

Objective: Bromocriptine mesylate (BRC), a dopamine D2 receptor agonist has been shown to confer neuroprotection, sustained motor function and slowed disease progression in mouse models of amyotrophic lateral sclerosis (ALS) Here we report a first in human trial in ALS.

Design: A multicenter, Riluzole add-on, randomized, double-blind, placebo controlled 102-week extension BRC clinical trial.

Methods: The trial was conducted between January 2009 and March 2012 on 36 Japanese ALS patients.

Cognitive impairment of Japanese multiple sclerosis patients: Follow-up study using BRB-N assessment tool.

Objectives: The purpose of this study was to evaluate cognitive function in patients with multiple sclerosis (MS), compared with control subjects, and to establish whether decline of cognitive function continues in the patients during remission.

Methods: The Japanese version of the Brief Repeatable Battery of Neuropsychological tests (BRB-N), which includes the selective reminding test (SRT), spatial recall test (SPART), symbol digit modalities test (SDMT), paced auditory serial addition test (PASAT), and the word list generation test (WLG), was performed in 34 Japanese patients with MS (8 males, 26 females; mean age, 42 years) and in 37 age- and education-matched healthy controls (20 males, 17 females; mean age, 36 years). BRB-N was conducted at intervals of two years for MS patients who remained in remission, and the cognitive changes were evaluated by comparing the results with those of the initial examination at entry into the study.