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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.

Myasthenia gravis (MG) is an immune-related adverse event (irAE), and as an irAE, MG (irAE-MG) generally has a monophasic course, with only a few case reports of irAE-MG flare-ups during the course of the disease. We herein report a case of pembrolizumab-induced MG with relapsing symptoms. irAE-MG is a rare disease that has not yet been fully characterized, and our case shows that MG symptoms may relapse. Therefore, regular follow-up is necessary, even after the symptoms improve with immunosuppressive therapy.

In 2012, a large number of myositis cases with anti-mitochondrial M2 (AMA-M2) antibody, which had well been known as the serological hallmark for primary biliary cholangitis (PBC), were reported in Japan. Recently, some case series from Japan, France, America, China and India have shown that approximately 2.5% to 19.5% of patients with myositis have AMA-M2 antibody. The objective of this study was to clarify the prevalence, clinical features, treatment outcome, and severity determinants of AMA-M2 positive myositis.This study was a multicenter observational study. We enrolled patients who were diagnosed with myositis during a ten-year period between 2012 and 2021.Of the total of 185 patients with inflammatory myopathy, 17 patients were positive for AMA-M2 antibody. The typical symptoms were weakness mainly involving paravertebral muscles, weight loss, respiratory failure, and cardiac complications. Thirteen of the 17 patients had cardiac complications. A strong correlation was found between respiratory failure and modified Rankin Scale (mRS) score. A strong correlation was also found between respiratory failure and body weight, indicating that weight loss can be an indicator of potential progression of respiratory failure. Six of the 17 patients were complicated by malignancy.This study showed significant correlations between % vital capacity (VC), body mass index (BMI), and mRS score in patients with AMA-M2-positive myositis. Immunotherapy often improved CK level and respiratory dysfunction. We therefore propose that %VC and BMI should be monitored as disease indicators in treatment of AMA-M2-positive myositis.

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.

We report a 60-year-old woman with toxoplasma encephalopathy. She was being treated with prednisolone and methotrexate for rheumatoid arthritis that was diagnosed at the age of 40. In a preoperative examination of her left fifth finger ganglion, pericardial effusions, cardiomegaly, and a right atrial mass were detected. In addition, brain MRI showed nodular shadows in the right thalamus, bilateral globus pallidus, and left dentate nucleus of the cerebellum. T1 and T2 weighted images showed high intensities within those shadows; however, a T1 gadolinium enhancement image showed no contrast enhancement in the lesions. There were no positive neurological findings. Examination of the cerebrospinal fluid and cultivation tests showed nothing particular. The right atrial mass was subsequently diagnosed as malignant lymphoma and treated with radiation therapy. Toxoplasma gondii antibody titers were increased in both serum and cerebrospinal fluid. Based on IgG avidity index and nested PCR, we diagnosed toxoplasma encephalopathy with chronic T. gondii infection. The T. gondii gene product was also detected in cerebrospinal fluid by nested PCR. We consider that IgG avidity index and nested PCR were useful for the diagnosis of toxoplasma encephalopathy.

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.

We report a case of tetanus in a 77-year-old man with type 2 diabetes. His diabetes was being managed with insulin injections and self-monitoring of blood glucose. He presented to our emergency department with a 2-week history of worsening myalgia. On examination, he had trismus, severe spasms and opisthotonus. We diagnosed tetanus and started treatment with human tetanus immunoglobulin, ampicillin, and magnesium, but he required an artificial respirator. The muscle spasms continued for a long term, but gradually improved. He was weaned from the respirator after 6 weeks. The patient was discharged in an ambulatory status 3 months after admission. On review, the he reported gardening barehanded after carrying out needle puncture of a finger to monitor the blood glucose. We speculate that this needle puncture could have resulted in tetanus infection. The present case shows that tetanus infection can occur after needle puncture for self-monitoring of blood glucose.

Cyclosporine A (CYA) treatment has been reported to be probably useful for patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) that is resistant to conventional treatment. Although several studies have shown that appropriate area under the concentration-time curve (AUC) monitoring of CYA levels results in improved outcomes for refractory nephrotic syndrome patients, the importance of using AUC analysis for CIDP remains unclear. In this study, we measured both trough and AUC from 0 to 4 h (AUC(0-4 h)) levels of CYA in 2 patients with CIDP and compared the findings for the clinical parameters. On the basis of the CYA dosing recommendations for patients with nephrotic syndrome, we used a CYA concentration of 150 ng/ml for the trough level and an AUC(0-4 h) value of 2,500 ng/(ml·h). Patient 1 showed a significant increase in grip strength and a prolonged remission period. Patient 2 showed improvement in the modified Rankin scale and manual muscle test (MMT) scores. Monitoring both AUC(0-4 h) and trough levels of CYA seems to be a better option than monitoring the trough level alone because it leads to improved estimation of the efficacy and safety of CYA treatment in the case of CIDP patients.

We report the case of a 34-year-old woman with cerebral and pulmonary cryptococcosis. After surgery for uterine cervical cancer, chest CT scan indicated a solitary tumor. Cryptococcosis was detected by transbronchial lung biopsy, and brain MRI showed multiple tumors. We diagnosed the patient with cerebral and pulmonary cryptococcosis. Oral and intravenous antifungal treatments were not effective, and a disturbance of consciousness appeared. We began intraventricular antifungal treatment, and the symptoms improved, with a reduction in the size of multiple lesions. However, the size of the brain lesions increased, and we diagnosed late deterioration of cryptococcosis and corticosteroid response. Because of the refractory clinical course, we examined the Cryptococcus strains from the surgical resected pulmonary lesion and identified Cryptococcus gattii(VG I type). C. gattii occurs predominantly in apparently healthy hosts. An intracranial C. gattii infection is associated with neurological complications and delayed therapeutic response. If cerebral cryptococcosis responds slowly and relatively poorly to antifungal therapy, C. gattii should be considered. Aggressive therapy, including intraventricular therapy and corticosteroids therapy for cryptococcoma, is required.

Nephrotoxic potential of cefpirome sulfate (CPR) was examined by single and multiple intravenous administrations to Japanese white male rabbits. In single administration studies, no nephrotoxic symptoms were observed at the dose level of 320 mg/kg of CPR or less. At the dose level of 500 mg/kg or more, nephrotoxic findings were noted in both CPR and cefazolin sodium(CEZ) groups, such as proteinuria, glucosuria, increase in serum level of urea nitrogen, creatinine and uric acid, and necrosis and calcification in the proximal tubular epithelium of kidney. Renal phenolsulfonphthalein(PSP) excretion was suppressed at the dose level of 500 mg/kg of CPR or more, and 2000 mg/kg of CEZ. In 14 and 21 days repeated administration studies, no nephrotoxic symptoms were observed at the dose level of 100 mg/kg of CPR and CEZ or less. At the dose level of 200 mg/kg of CPR or more, urinary and serum biochemical findings mentioned above were observed, and histopathological changes in the kidney described above were added in 400 mg/kg group. The similar nephrotoxic symptoms including histopathological changes of the kidney were observed in the groups of 100 mg/kg of cefaloridine(CER) and 200 mg/kg of CEZ. In addition, renal PSP excretion was suppressed in the group of 200 mg/kg of CEZ. The results would suggest that CPR is less nephrotoxic than CER and that the nephrotoxic potential of CPR is comparable to CEZ because CPR caused more severe renal failures in single administration study and less severe renal failures in multiple administration study than CEZ.