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A 56-year-old woman presenting with type II respiratory failure was transferred to our hospital. She did not exhibit muscle weakness or elevated serum myogenic enzymes, but needle electromyography revealed myogenic changes in the limb muscles, and her blood tests were positive for anti-mitochondrial antibodies (AMA). Muscle histopathological findings included immune-mediated necrotizing myopathy, so she was diagnosed with inflammatory myopathy associated with AMA. After treatment with corticosteroids and noninvasive positive pressure ventilation, her symptoms improved. If a diagnosis of type II respiratory failure is difficult, inflammatory myopathy associated with AMA should be considered as a differential diagnosis.

We herein report a case of granulomatosis with polyangiitis in a 73-year-old man. He had experienced bilateral ptosis, redness of both eyes, right facial weakness, and hearing loss in the right ear for two months. Myeloperoxidase anti-neutrophil cytoplasmic antibody was positive. Corticosteroids and intravenous cyclophosphamide pulse therapy yielded a response. After the fourth pulse of cyclophosphamide, he developed headache, diplopia, restricted left eye movement, right facial palsy, and hearing loss in the right ear. A one-year remission period was achieved by increasing the steroid and rituximab doses. Rituximab was effective against the relapse of granulomatosis with polyangiitis and cranial neuropathy.

Reports on the long-term survival effect of edaravone, which was approved for the treatment of amyotrophic lateral sclerosis (ALS) in 2015 in Japan, are rare. Herein, we report our retrospective analysis of 45 consecutive patients with ALS who initially visited our hospital between 2013 and 2018. Of these, 22 patients were treated with edaravone for an average duration of 26.6 (range, 2–64) months, whereas the remaining patients were not treated with edaravone and comprised the control group. There were no differences in baseline demographics between the two groups. The primary endpoint was tracheostomy positive-pressure ventilation (TPPV) or death, and the follow-up period ended in December 2020. The survival rate was significantly better in the edaravone group than in the control group based on the Kaplan–Meier analysis, which revealed that the median survival durations were 49 (9–88) and 25 (8–41) months in the edaravone and control groups, respectively (p = 0.001, log-rank test). There were no serious edaravone-associated adverse effects during the study period. Overall, the findings of this single-center retrospective study suggest that edaravone might prolong survival in patients with ALS.

We herein report the case of myasthenic crisis occurring in a 51-year-old man. He had experienced ptosis, increased body weight with edema, and fatigue with dyspnea. He presented at our emergency department with disturbed consciousness. He was originally diagnosed with myxedema coma, and he required artificial respiration. Because his weakness persisted and he was positive for anti-acetylcholine receptor antibodies and anti-muscle-specific tyrosine kinase antibodies, we diagnosed myasthenic crisis after various examinations. His clinical response to treatment was good and he was discharged in an ambulatory status 3 months after admission. This case demonstrates that myasthenic crisis may occur in association with myxedema.

Objective:To clarify the prevalence and clinical characteristics of neuromyelitis optica spectrum disorders (NMOSD) in Japan and compare them with those in other ethnic populations.Methods:Data processing sheets were sent to all related institutions in northern Japan and were collected from April to May 2016. Prevalence was determined on March 31, 2016, using the 2015 International Panel for NMO Diagnosis criteria.Results:The crude prevalence was 4.1/100,000 (95% confidence interval 2.2–6.9) for NMOSD in northern Japan, with a significantly higher number of female than male patients (female: male 12:2). The positivity for anti-aquaporin-4 antibody was 78.6%, and the mean age at onset was 45.2 years. All patients were subjected to preventive therapy in the form of treatment with steroids or immunosuppressive agents.Conclusions:Our results showed that the prevalence of NMOSD in the Japanese population is similar to that in Caucasians.

We herein report the case of stiff-person syndrome in a 73-year-old woman. She experienced episodes of painful muscle spasms and was admitted to another hospital. She was diagnosed with Waldenstrom macroglobulinemia. She showed improvement in muscle spasms post-chemotherapy, which was discontinued due to pancytopenia. Six months later, she was admitted to our hospital for repeated whole-body muscle spasms, at which point she was diagnosed with stiff-person syndrome. An anti-glutamic acid decarboxylase antibody text was negative. Her muscle spasms disappeared after the administration of corticosteroids and rituximab. Stiff-person syndrome may develop with Waldenstrom macroglobulinemia. In the present case, corticosteroids and rituximab provided effective treatment.

Background Multiple system atrophy (MSA) is a rare progressive neurodegenerative disorder for which brief yet sensitive scale is required in order for use in clinical trials and general screening. We previously compared several scales for the assessment of MSA symptoms and devised an eight-item pilot scale with large standardized response mean [handwriting, finger taps, transfers, standing with feet together, turning trunk, turning 360°, gait, body sway]. The aim of the present study is to investigate the validity and reliability of a simple pilot scale for assessment of multiple system atrophy symptoms. Methods Thirty-two patients with MSA (15 male/17 female; 20 cerebellar subtype [MSA-C]/12 parkinsonian subtype [MSA-P]) were prospectively registered between January 1, 2014 and February 28, 2015. Patients were evaluated by two independent raters using the Unified MSA Rating Scale (UMSARS), Scale for Assessment and Rating of Ataxia (SARA), and the pilot scale. Correlations between UMSARS, SARA, pilot scale scores, intraclass correlation coefficients (ICCs), and Cronbach’s alpha coefficients were calculated. Results Pilot scale scores significantly correlated with scores for UMSARS Parts I, II, and IV as well as with SARA scores. Intra-rater and inter-rater ICCs and Cronbach’s alpha coefficients remained high (> 0.94) for all measures. Conclusion The results of the present study indicate the validity and reliability of the eight-item pilot scale, particularly for the assessment of symptoms in patients with early state multiple system atrophy.

Background and purpose The prevalence of multiple sclerosis (MS) is considered to be lower in East Asia than in Western countries. An increasing trend has been globally reported for the prevalence of MS. We investigated the changes in the prevalence and clinical characteristics of MS in Tokachi province of Hokkaido, northern Japan, from 2001 to 2016. Methods Prevalence was determined on March 31, 2016. Data processing sheets were collected from all MS-related institutions in Tokachi province. Herein, we applied the Poser's diagnostic criteria for MS as that used in our previous three studies. Cases of neuromyelitis optica spectrum disorders were excluded. Results In 2016, the crude MS prevalence was 18.6/100,000 (95% confidence interval, 14.3–23.8) in northern Japan. Over the last 15 years, the prevalence of MS in the same area was 8.1, 12.6, and 16.2, in 2001, 2006, and 2011, respectively. The female/male ratio was 3.57, which increased from 2.63 in 2001. The ratios of primary-progressive, relapsing-remitting, and secondary-progressive MS types were 2%, 84%, and 14%, respectively. Conclusion Our results demonstrated consistent increase in the MS prevalence among the northern Japanese, particularly in females, and the relatively lower rates of progressive MS in northern Japan than in Western countries. This article is protected by copyright. All rights reserved.

Myasthenia gravis is an autoimmune disorder predominantly affecting women of reproductive age; its course during pregnancy is unpredictable. Here we report a case of a 34-year-old woman who developed myasthenia gravis during pregnancy. At 18 weeks of pregnancy, she started experiencing general fatigue and weakness when working. After examination, we diagnosed her with seronegative myasthenia gravis. Treatment included pyridostigmine (60 mg/day), oral prednisolone (5 mg, alternate day), intravenous immunoglobulin (0.4 mg/kg/day, 5 days) and methylprednisolone pulse therapy (500 mg/day, 3 days); her clinical response was good. An uneventful vaginal delivery took place and the infant showed no signs of muscular weakness. Myasthenia gravis is a high-risk disease, particularly when associated with pregnancy. We suggest that early treatment with intravenous immunoglobulin is safe and effective for myasthenia gravis with pregnancy.