No abstract

Eosinophilia-myalgia syndrome (EMS) is a newly recognized disorder, characterized by myalgia, weakness, scleroderma-like changes, and eosinophilia. EMS is associated with lots of L-tryptophan allegedly contaminated with byproducts of the manufacturing process. We describe 3 patients with EMS who presented with a severe demyelinating sensorimotor polyneuropathy. Electrodiagnostic studies revealed multifocal conduction block, slowing and temporal dispersion of motor responses, and prolonged or absent F-responses. Despite plasmapheresis; corticosteroids; and, in 1 patient, cyclophosphamide, 2 patients died and the remaining patient experienced minimal recovery. Pathology revealed patchy perivascular infiltrates and fibrosis in the connective tissue of muscle and nerve. Autopsy of the central nervous system in 2 patients did not reveal changes unique to EMS. In addition to other organ involvement, EMS may manifest as a potentially fatal polyneuropathy, which initially appears to have prominent demyelinating features.

Dermatomyositis (DM) is an inflammatory myopathy of skeletal muscle with characteristic cutaneous findings. It is a rare disorder with a bimodal age distribution that affects almost twice as many women as men. One category of DM, normal-enzyme DM, is characterized by cutaneous changes only at baseline, normal serum muscle enzyme levels and myositis demonstrated by electromyography (EMG) and/or muscle biopsy specimens. Typically, patients with normal-enzyme DM progress to severe muscle involvement and require systemic corticosteroid therapy. The patient we report has normal-enzyme DM confirmed by serial serum enzymes, EMG, and skin and muscle biopsies but is unique in that she never experienced progression of muscle weakness although muscle involvement was documented histologically and by EMG. Follow-up examination after 1 year revealed near-complete resolution of cutaneous involvement after topical therapy and no evidence of muscle weakness.