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π CustomizeMedical Overview of Inclusion Body Myopathy With Paget Disease Of Bone And Frontotemporal Dementia
Sources citedInclusion 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.
Classification & codes: GARD 0010899 · Orphanet ORPHA:52430 · OMIM 167320 · ICD-10 G71.8
Sources: GARD (NIH/NCATS), Orphanet Β· last reviewed 2026. Educational only β not medical advice.
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Disclosure, accommodations, insurance transitions and workplace planning for teens and young adults with IBMPFD.
Sources: GARD (NIH/NCATS), Orphanet Β· last reviewed 2026. Educational only β not medical advice.