Inclusion body myopathy with Paget disease of bone and frontotemporal dementia (IBMPFD) is a multisystem degenerative genetic disorder characterized by adult-onset proximal and distal muscle weakness (clinically resembling limb-girdle muscular dystrophy); early-onset Paget disease of bone, manifesting with bone pain, deformity and enlargement of the long-bones; and premature frontotemporal dementia, manifesting first with dysnomia, dyscalculia and comprehension deficits followed by progressive aphasia, alexia, and agraphia. As the disease progresses, muscle weakness begins to affect the other limbs and respiratory muscles, ultimately resulting in respiratory or cardiac failure. The disease is Monarch Disease Ontology id MONDO_0000507 (inclusion body myopathy with Paget disease of bone and frontotemporal dementia). Also known as: IBMPFD, inclusion body myopathy with Paget's disease of bone and frontotemporal dementia, inclusion body myopathy with early-onset Paget disease with or without frontotemporal dementia, inclusion body myopathy/Paget disease/frontotemporal dementia, limb-girdle muscular dystrophy with Paget disease of bone, pagetoid amyotrophic lateral sclerosis, pagetoid neuroskeletal syndrome.