Brachio-cervical inflammatory myopathy with associated scleroderma phenotype and lupus serology

Brachio-cervical inflammatory myopathy (BCIM) is a unique clinicopathologic entity characterized by neck and upper extremity weakness with relative sparing of lower extremities and commonly associated with connective tissue diseases or myasthenia gravis and serum autoantibodies (e.g., antinuclear antibody [ANA], anti–double stranded DNA [dsDNA], and anti–acetylcholine receptor).1 Muscle pathology is distinctive, with prominent B-cell infiltrates and endomysial membrane attack complex (MAC; C5b-9) deposition. Despite the detailed original series, there have been no subsequent reports (besides abstracts2,3) demonstrating the full clinicopathologic features of BCIM.

http://nn.neurology.org/cgi/content/short/5/1/e410?rss=1

Source: Neurology Neuroimmunology and Neuroinflammation - October 27, 2017 Category: Neurology Authors: Gao, A. F., Saleh, P. A., Kassardjian, C. D., Vinik, O., Munoz, D. G. Tags: Clinical/Scientific Notes Source Type: research