inclusion body myopathy with Paget disease of bone and frontotemporal dementia https://www.malacards.org/card/inclusion_body_myopathy_with_paget_disease_of_bone_and_frontotemporal_dementia MONDO:0000507 MEDGEN:322251 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. SCTID:703544004 Orphanet:52430 DOID:0050881 inclusion body myopathy with early-onset Paget disease with or without frontotemporal dementia OMIMPS:167320 inclusion body myopathy with early-onset Paget disease and frontotemporal dementia icd11.foundation:1947548457 GARD:0010899 UMLS:C1833662 inclusion body myopathy/Paget disease/frontotemporal dementia pagetoid neuroskeletal syndrome limb-girdle muscular dystrophy with Paget disease of bone IBMPFD pagetoid amyotrophic lateral sclerosis syndromic disease hereditary inclusion-body myopathy frontotemporal dementia obsolete primary bone dysplasia with disorganized development of skeletal components