{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,4,21]],"date-time":"2023-04-21T05:18:29Z","timestamp":1682054309840},"reference-count":19,"publisher":"Oxford University Press (OUP)","issue":"683","license":[{"start":{"date-parts":[[1982,9,1]],"date-time":"1982-09-01T00:00:00Z","timestamp":399686400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1982,9,1]]},"abstract":"<jats:title>Summary<\/jats:title>\n               <jats:p>A 14-year-old boy with a long history of distal muscle weakness affecting primarily and predominantly the upper limbs is described. There is a family history of pes cavus and congenital dislocation of the hip. Electromyography and histopathological studies of skeletal muscle showed conclusive evidence of a neurogenic muscular disorder, and excluded primary muscle disease. The muscle biopsy showed group atrophy. As many target fibres which are identical to structured cores were a prominent feature of the biopsy, central core disease was considered. However, it was concluded on clinical, neurophysiological and histological evidence that the patient was suffering from distal spinal muscular atrophy of an intermediate type designated by previous authors.<\/jats:p>\n               <jats:p>A review of the current concepts of distal muscle weakness is included.<\/jats:p>","DOI":"10.1136\/pgmj.58.683.554","type":"journal-article","created":{"date-parts":[[2008,12,29]],"date-time":"2008-12-29T20:43:59Z","timestamp":1230583439000},"page":"554-557","source":"Crossref","is-referenced-by-count":0,"title":["The distal form of spinal muscular atrophy: an unusual case demonstrating the intermediate variety"],"prefix":"10.1093","volume":"58","author":[{"given":"D A","family":"Isenberg","sequence":"first","affiliation":[{"name":"Department ofRheumatology, University College Hospital , London WC1"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pauline A","family":"Kahn","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Whittington Hospital , London N19"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[1982,9,1]]},"reference":[{"issue":"68","key":"ref_1","first-page":"1","article-title":"Clinical and electro-diagnostic features of Charcot-Marie-Tooth syndrome","volume":"8","author":"BRUST, J.C.M.; LOVELACE, R.E.; DEVI, S.","year":"1978","unstructured":"BRUST, J.C.M., LOVELACE, R.E. & DEVI, S. (1978) Clinical and electro-diagnostic features of Charcot-Marie-Tooth syndrome. Acta Neurologica Scandinavia, S8, Suppl. 68, 1.","journal-title":"Acta Neurologica Scandinavia"},{"key":"ref_2","first-page":"97","article-title":"Sur une forme particuliere d'atrophie musculaire progressive, souvent familiale, debutant par les pieds et les jambes, et atteignant plus tard les mains","volume":"6","author":"CHARCOT, J.M.; MARIE, P.","year":"1886","unstructured":"CHARCOT, J.M. & MARIE, P. (1886) Sur une forme particuliere d'atrophie musculaire progressive, souvent familiale, debutant par les pieds et les jambes, et atteignant plus tard les mains. Revue de Mddecine (Paris), 6, 97.","journal-title":"Revue de Mddecine (Paris)"},{"key":"ref_3","first-page":"254","article-title":"Muscle Biopsy: A Modern Approach","author":"DuBowITz, V.; BROOKE, M.H.","year":"1973","unstructured":"DuBowITz, V. & BROOKE, M.H. (1973) Muscle Biopsy: A Modern Approach, p. 254. W. B. 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