SEVERE BULBAR-PREDOMINANT DERMATOMYOSITIS WITH MI-2A ANTIBODIES PRESENTING AS PROGRESSIVE QUADRIPARESIS: A CASE REPORT

Abstract

Background: Idiopathic inflammatory myopathies (IIMs) are autoimmune disorders characterized by skeletal muscle inflammation and progressive weakness. Dermatomyositis is a major subtype distinguished by characteristic cutaneous manifestations in addition to muscle involvement. Dysphagia may occur during the course of disease; however, severe bulbar involvement requiring enteral feeding is relatively uncommon and may delay diagnosis. Case Presentation: We report a 45 - year - old male farmer who presented with a two-month history of progressive proximal limb weakness and severe dysphagia. The illness initially began with bilateral facial swelling and violaceous periorbital rash that resolved spontaneously after several weeks. Subsequently, the patient developed progressive proximal quadriparesis, hoarseness of voice, and severe oropharyngeal dysphagia requiring Ryles tube feeding. Neurological examination revealed pure motor proximal-predominant quadriparesis with bulbar involvement. Laboratory investigations demonstrated markedly elevated muscle enzymes.