{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T05:51:52Z","timestamp":1791179512107,"version":"4.1.0"},"reference-count":30,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2015,12,29]],"date-time":"2015-12-29T00:00:00Z","timestamp":1451347200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2015,12,29]],"date-time":"2015-12-29T00:00:00Z","timestamp":1451347200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2016,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:label\/>\n                    <jats:p>\n                      <jats:italic>Introduction<\/jats:italic>\n                      : Valosin containing protein (\n                      <jats:italic>VCP<\/jats:italic>\n                      ) mutations cause a rare disorder characterized by hereditary inclusion body myopathy, Paget disease of bone (PDB), and frontotemporal dementia (FTD) with variable penetrance.\n                      <jats:italic>VCP<\/jats:italic>\n                      mutations have also been linked to amyotrophic lateral sclerosis and Charcot\u2010Marie\u2010Tooth disease type 2.\n                      <jats:italic>Methods<\/jats:italic>\n                      : Review of clinical, serological, electrophysiological, and myopathological findings of 6\n                      <jats:italic>VCP<\/jats:italic>\n                      \u2010opathy patients from 4 unrelated families.\n                      <jats:italic>Results<\/jats:italic>\n                      : Patients manifested muscle weakness between ages 40 and 53 years and developed predominant asymmetric limb girdle weakness. One patient had distal weakness at onset and co\u2010existing peripheral neuropathy. Another patient had PDB, 1 had mild cognitive deficits, and 1 had FTD. All patients had myopathic and neurogenic electromyographic findings with predominant neurogenic changes in 2. Rimmed vacuoles were infrequent, while neurogenic changes were prominent in muscle biopsies.\n                      <jats:italic>Conclusions<\/jats:italic>\n                      :\n                      <jats:italic>VCP<\/jats:italic>\n                      \u2010opathy is a multifaceted disorder in which myopathy and peripheral neuropathy can coexist. The electrophysiological and pathological neurogenic changes raise the possibility of coexisting motor neuron involvement.\n                      <jats:italic>Muscle Nerve<\/jats:italic>\n                      , 2015\n                      <jats:italic>Muscle Nerve<\/jats:italic>\n                      <jats:bold>54<\/jats:bold>\n                      : 94\u201399, 2016\n                      <jats:italic>Muscle Nerve<\/jats:italic>\n                      <jats:bold>54<\/jats:bold>\n                      : 94\u201399, 2016\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/mus.24980","type":"journal-article","created":{"date-parts":[[2015,11,17]],"date-time":"2015-11-17T18:28:52Z","timestamp":1447784932000},"page":"94-99","source":"Crossref","is-referenced-by-count":30,"title":["Clinical spectrum of valosin containing protein (\n                    <i>VCP<\/i>\n                    )\u2010opathy"],"prefix":"10.1002","volume":"54","author":[{"given":"Mohamed","family":"Kazamel","sequence":"first","affiliation":[{"name":"Department of Neurology Mayo Clinic Rochester Minnesota 55905 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric J.","family":"Sorenson","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic Rochester Minnesota 55905 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathleen M.","family":"McEvoy","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic Rochester Minnesota 55905 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"Jr.","given":"Lyell K.","family":"Jones","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic Rochester Minnesota 55905 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea 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