PMID: 9527159Apr 4, 1998Paper

Absence of characteristic features in two patients with inclusion body myositis

Journal of Neurology, Neurosurgery, and Psychiatry
M F van der MeulenJ H Wokke

Abstract

According to recently published criteria a diagnosis of definite sporadic inclusion body myositis is made if the typical histopathological abnormalities (rimmed vacuoles and abnormal accumulations of proteins, in addition to mononuclear cell infiltrates) are present. The two women described here presented with myositis which was unresponsive to treatment. Patient 1 had features of non-progressive sporadic inclusion body myositis clinically, whereas patient 2 had a very slowly progressive limb girdle syndrome. The cryostat sections of the first biopsies did not show rimmed vacuoles, even in retrospect. Only a repeated biopsy, 12 years after presentation in one patient and 18 years after presentation in the other, disclosed the typical features of sporadic inclusion body myositis. The initial absence of abnormal fibres probably represents a real absence or scarcity rather then a sampling error due to a multifocal nature of the histological abnormalities. It is of importance for the clinician to realise that some patients with myositis unresponsive to treatment, even if both clinical and histological features do not suggest sporadic inclusion body myositis, may prove to have the disease on repeated histopathological examination.

Citations

Jun 19, 2001·Neuromuscular Disorders : NMD·M F van der MeulenJ H Wokke
Dec 3, 1999·The British Journal of Dermatology·N Y TalaninR Friedman-Musicante
Apr 12, 2006·Annals of the Rheumatic Diseases·I M BronnerJ E Hoogendijk
Dec 18, 2004·BMJ : British Medical Journal·Gerald J D Hengstman, Baziel G M van Engelen
Jul 23, 2009·BMJ : British Medical Journal·Steven A Greenberg
Jul 19, 2013·Journal of Neurology, Neurosurgery, and Psychiatry·Stefen BradyDavid Hilton-Jones
Mar 30, 2011·Rheumatic Diseases Clinics of North America·Brent T Harris, Carrie A Mohila
Dec 27, 2015·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·H NoderaR Kaji

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