Severe myopathy in a patient with chronic neurological disease – diagnostic challenges

Abstract

Polymyositis is a rare disease that belongs to the idiopathic inflammatory myopathies (IIMs) group, characterized by chronic muscle inflammation, and in rare cases a life-threatening condition due to extra-muscular involvement. Even though steroids constitute the building block of treatment of this disease, in severe cases, escalation treatment should be considered in order to obtain good clinical outcomes. We report a clinical case of a 22-year-old female who developed progressive severe systemic muscular weakness, dysphagia and dysphonia, accompanied by elevated serum muscle enzymes, positive myositis-specific antibodies, and muscle biopsy suggestive of inflammatory myopathy. The clinical features and laboratory results led us to the diagnosis of polymyositis. On additional laboratory tests the patient tested positive for Borrelia burgdorferi (Borrelia b) specific antibodies. Due to life-threatening organ involvement the immunosuppressive treatment, immunoglobulin intravenous infusion and pulse therapy with methylprednisolone were initiated and she needed nasogastric tube in order to be fed. Furthermore antibiotic treatment was administrated. The patient improved almost completely after 3 months of treatment.