Objective:To show cancer association is a risk factor other than statin exposure for anti-3-hydroxy-3-methylglutaryl coenzyme A reductase autoantibody-positive (anti-HMGCR Ab+) myopathy.Methods:We analyzed the clinical features and courses of 33 patients (23 female and 10 male) with anti-HMGCR Ab+ myopathy among 621 consecutive patients with idiopathic inflammatory myopathies.Results:Among the 33 patients, 7 (21%) were statin-exposed and 26 were statin-naive. In relation with cancer, there were 12 patients (statin-exposed, n = 4) with cancers detected within 3 years of myopathy diagnosis (cancer association), 3 patients (all statin-naive) with cancers detected more than 3 years before myopathy diagnosis (cancer history), 10 cancer-free patients followed up for more than 3 years (all statin-naive), and 8 patients without cancer detection but followed up for less than 3 years (statin-exposed, n = 3). Therefore, 12 patients with cancer association (36%) formed a larger group than that of 7 statin-exposed patients (21%). Among 12 patients with cancer association, 92% had cancer detection within 1 year of myopathy diagnosis (after 1.3 years in the remaining patient), 83% had advanced cancers, and 75% died of cancers within 2.7 years. Of interest, 1 patient with cancer history had sustained increase in creatine kinase level over 12 years from cancer removal to the development of weakness.Conclusions:Patients with cancer association formed a large group with poor prognosis in our series of patients with anti-HMGCR Ab+ myopathy. The close synchronous occurrence of cancers and myopathies suggested that cancer association is one of the risk factors for developing anti-HMGCR Ab+ myopathy.

CD8-MHC-1 complex is present in patients with PM, IBM, or unclassifiable group. The data may serve as an argument for a trial of immunosuppressive treatment in p62-immunonegative patients with unclassifiable myositis.

Cardiac iodine-123-labeled-metaiodobenzylguanidine uptake is reduced in early-stage Parkinson's disease, suggesting sympathetic nerve degeneration. The scintigraphic findings in patients with Parkinson's disease with different clinical features have, however, not been established. Iodine-123-labeled-metaiodobenzylguanidine myocardial scintigraphy was performed in 143 patients with Parkinson's disease. The early and delayed heart to mediastinum ratios were analyzed according to the dominant motor deficit (tremor, bradykinesia, rigidity, and postural instability), age, sex, age at onset, disease duration, and Hoehn and Yahr stage. Both ratios correlated with bradykinesia, age at disease onset, and disease duration; but not with sex, Hoehn and Yahr stage, tremor, rigidity, and postural instability. Our results suggest a close link between myocardial sympathetic degeneration and bradykinesia, age at onset and disease duration.

Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disease with marked variety in its clinical manifestations. While characteristic neuroimaging and skin biopsy findings are important clues to the diagnosis, autopsy studies are still important for confirming the exact disease features. We herein report the case of a patient who received an antemortem diagnosis of familial NIID with dementia-dominant phenotype that was later confirmed by an autopsy. Our report is the first to document a case of autopsy-confirmed NIID involving both cognitive impairment and sensorimotor neuropathy.

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With the expanding use of immune checkpoint blockers typified by anti-programmed death-1 (PD-1) and anti-cytotoxic T-lymphocyte-associated protein 4 monoclonal antibodies (Abs) for antitumor therapy, the number of patients showing immune-related adverse events (irAEs) is increasing. Skeletal muscle is one of the target tissues of irAEs and several features of myopathy as irAEs have been reported: myasthenia gravis (MG) overlap, cardiac involvement, necrotizing myopathy, and inflammatory myopathy with T-cell and B-cell infiltration. [1][2][3][4] However, the immunopathogenesis of muscle destruction remains unclear. Here, we report 2 cases of granulomatous myositis after anti-PD-1 therapy. Case reportsCase 1 A 79-year-old woman with stage IV lung adenocarcinoma developed nonfatigable mild proximal limb weakness (Medical Research Council [MRC] grade 4) 14 days after the fourth cycle of nivolumab. She presented no skin rash and showed no ocular, bulbar, truncal, or respiratory symptoms. She had well-controlled concurrent breast cancer. Laboratory test results showed an elevated serum creatine kinase (CK) level of 1,638 IU/L. Myositis-specific autoantibodies (MSAs), anti-acetylcholine receptor (AChR) Ab, and anti-titin Ab were negative. Antistriated muscle Abs were not measured. Electrocardiography findings were normal. EMG showed spontaneous activity. Chest CT showed no lesions except the cancers.Biopsy of the biceps brachii muscle showed patchy mononuclear cell infiltrates and granuloma formation in muscle fascicles ( figure 1A). In the granulomas, CD11c + M1 and CD163 + M2 macrophages were abundant (figure e-1, links.lww.com/NXI/A46), and CD11c + M1 macrophages frequently invaded non-necrotic fibers and formed granulomatous collection inside the basal lamina of muscle fibers (figure 1, B-D). CD8 + T cells invading non-necrotic fibers were also observed ( figure 1E). PD-1 + cells were scattered in granulomas (figure 1F), and programmed death-ligand 1 (PD-L1) was upregulated on the non-necrotic fibers around granulomas ( figure 1G). Electron microscopy confirmed the macrophage invasion of non-necrotic fibers (figure e-2, links.lww.com/NXI/A46). Nivolumab was discontinued, and oral prednisolone 50 mg was started. Her symptoms improved dramatically with complete resolution, and prednisolone was tapered off within 3 weeks. During 20 months of follow-up, she showed no neurologic recurrence and received conventional chemotherapy. Case 2A 70-year-old man who received pembrolizumab (anti-PD-1 Ab) and axitinib (tyrosine kinase inhibitor) for treatment of renal cell carcinoma developed left ptosis, diplopia, and weakness with myalgia in his neck and left shoulder (MRC grade 4) without bulbar, respiratory, or cardiac Biopsy of the left deltoid muscle showed mononuclear cell infiltration and giant-cell granulomas ( figure 1H). CD11c + M1 macrophages frequently invaded non-necrotic fibers (figure 1, I and J). Small granulomas replacing muscle fibers always contained both CD11c + M1 and CD163 + M2 macrophages (figure 1, K and L...