CLINICAL CORRELATES OF GRANULOMAS IN MUSCLE

Citation
T. Mozaffar et al., CLINICAL CORRELATES OF GRANULOMAS IN MUSCLE, Journal of neurology, 245(8), 1998, pp. 519-524
Citations number
28
Categorie Soggetti
Clinical Neurology
Journal title
ISSN journal
03405354
Volume
245
Issue
8
Year of publication
1998
Pages
519 - 524
Database
ISI
SICI code
0340-5354(1998)245:8<519:CCOGIM>2.0.ZU;2-M
Abstract
We evaluated the clinical and myopathological features of all patients with granulomas in muscle biopsy specimens identified over a 5-year p eriod (1992-1996) at the Washington University Medical Center. Ten pat ients were found to have granulomas in their muscle biopsy specimens. Of these, eight patients had myopathic changes. Seven had dysphagia as a major functional difficulty during the course of their disease. Non e had elevated levels of serum creatine kinase (CK). Four of the patie nts with myopathy had systemic sarcoidosis and relatively severe proxi mal weakness with functional disability. Treatment with corticosteroid s was followed by marked improvement in strength and functional disabi lity. The four other patients with myopathy had no systemic signs of s arcoidosis. Weakness was especially prominent distally in three of the se patients. The two patients in this group treated with corticosteroi ds did not improve. The final two patients, who had granulomas in musc le but no myopathic changes, had clinical syndromes of mononeuritis mu ltiplex and eosinophilic fasciitis (Shulman syndrome). We conclude tha t granulomatous myopathy, in the presence or absence of systemic sarco idosis, is commonly associated with dysphagia (87%) and a normal serum CK. Clinical features in patients with sarcoidosis included severe pr oximal weakness with functional disability that often responded to cor ticosteroid treatment. Granulomatous myopathy without systemic sarcoid osis was associated with milder, but more predominantly distal weaknes s.