title: [Appearance of numerous lobulated fibers after a protracted course of 18 years in a case of dermatomyositis].
abstract: A woman aged 47 first noticed weakness in her proximal muscles of all four limbs at the age of 29. After the initial investigation at our hospital, she was diagnosed as having dermatomyositis associated with primary biliary cirrhosis. In the following 18 years, she was kept on steroids. Although she responded to this treatment, weakness progressed slowly necessitating re-investigation including a second muscle biopsy. It was remarkable that in her first muscle biopsy, no lobulated fiber had been, while they were numerous in the second one. As a result of these studies, possibilities of limb-girdle muscle dystrophy (LGMD) and other myopathies remained but were ultimately ruled out after immunohistochemical/and genetic studies, and the original diagnosis was confirmed. Her weakness responded to an increased dose of steroids. Lobulated fibers were originally described in LGMD, and were reported also in the facioscapulohumeral muscular dytrophy and other hereditary myopathies. They have been rarely described in the inflammatory myopathies. Based on the observation of the present case, we concluded that lobulated fibers have little disease specificity and can newly appear in the course of any chronic myopathies including acquired myopathies like dermatomyositis.
authors.affiliation: Department of Neurology, Tokyo Metropolitan Neurological Hospital, Tokyo.
authors.forename: Naoki
authors.initials: N
authors.lastname: Onodera
authors.forename: Kazuhito
authors.initials: K
authors.lastname: Miyamoto
authors.forename: Shiro
authors.initials: S
authors.lastname: Matsubara
authors.forename: Hideaki
authors.initials: H
authors.lastname: Hayashi
authors.forename: Narihiro
authors.initials: N
authors.lastname: Minami
authors.forename: Ichizo
authors.initials: I
authors.lastname: Nishino
mesh_terms: D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D002908:Chronic Disease; D003882:Dermatomyositis / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D005260:Female; D006801:Humans; D008875:Middle Aged; D018485:Muscle Fibers, Skeletal / Q000473:pathology*; D011239:Prednisolone / Q000008:administration & dosage
chemical_list: D000893:Anti-Inflammatory Agents; D011239:Prednisolone