has characteristic has characteristic has material basis in germline mutation in VCP Adult onset autosomal dominant distal myopathy adult-onset distal myopathy due to VCP mutation https://www.malacards.org/card/adult_onset_distal_myopathy_due_to_vcp_mutation MEDGEN:1660404 GARD:0021492 UMLS:C4749506 Orphanet:329478 MONDO:0018006 Adult-onset distal myopathy due to VCP mutation is a rare, genetic distal myopathy disorder characterized by middle age-onset of distal leg muscle weakness, atrophy in the anterior compartment resulting in foot drop, without proximal or scapular skeletal muscle weakness. Rapidly progressive dementia, Paget disease of bone and hand weakness have been reported. Muscle biopsy shows pronounced myopathic changes with rimmed vacuoles.