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Ninety percent of patients with VCP\u2010associated MSP have myopathy, but there is no consensus\u2010based guideline. The goal of this working group was to develop a best practice set of provisional recommendations for VCP myopathy which can be easily implemented across the globe. As an initiative by Cure VCP Disease Inc., a patient advocacy organization, an online survey was initially conducted to identify the practice gaps in VCP myopathy. All prior published literature on VCP myopathy was reviewed to better understand the different aspects of management of VCP myopathy, and several working group sessions were conducted involving international experts to develop this provisional recommendation. VCP myopathy has a heterogeneous clinical phenotype and should be considered in patients with limb\u2010girdle muscular dystrophy phenotype, or any myopathy with an autosomal dominant pattern of inheritance. Genetic testing is the only definitive way to diagnose VCP myopathy, and single\u2010variant testing in the case of a known familial VCP variant, or multi\u2010gene panel sequencing in undifferentiated cases can be considered. Muscle biopsy is important in cases of diagnostic uncertainty or lack of a definitive pathogenic genetic variant since rimmed vacuoles (present in ~40% cases) are considered a hallmark of VCP myopathy. Electrodiagnostic studies and magnetic resonance imaging can also help rule out disease mimics. Standardized management of VCP myopathy will optimize patient care and help future research initiatives.<\/jats:p>","DOI":"10.1002\/acn3.51760","type":"journal-article","created":{"date-parts":[[2023,4,7]],"date-time":"2023-04-07T12:05:22Z","timestamp":1680869122000},"page":"686-695","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Provisional practice recommendation for the management of myopathy in <scp>VCP<\/scp>\u2010associated multisystem proteinopathy"],"prefix":"10.1002","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3604-6310","authenticated-orcid":false,"given":"Bhaskar","family":"Roy","sequence":"first","affiliation":[{"name":"Department of Neurology Yale School of Medicine  New Haven Connecticut USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Allison","family":"Peck","sequence":"additional","affiliation":[{"name":"Cure VCP Disease  Americus Georgia 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