Abnormal TDP-43-immunoreactive inclusions occur in muscle cells in inclusion body myositis (81) and myofibrillar myopathies (82), which are considered paradigms of degenerative muscular diseases with abnormal protein aggregates, and inclusion body myopathy, Paget disease, and frontotemporal dementia (IBMPFD), which can present as a spectrum of ALS, FTLD, and myopathy with abnormal TDP-43 inclusions (83). Here, TARDBP is linked to inclusion body myositis.