Poster 41: Distal weakness in a missionary with a history of asthma1

Abstract Setting: Tertiary care hospital. Patient: A 59-year-old male missionary in South Africa with adult-onset asthma. Case Description: The patient developed a small spot on his right foot. 2 days later, he had a fever, multiple joint pains, and pain and swelling of both legs. He presented to a Cote d'Ivoire hospital, was diagnosed with "insect bite" and treated with steroids. This progressed to severe neuropathic pain involving the upper extremities and weakness of hands and foot drop bilaterally. He returned to the United States for further workup. Assessment/Results: Extensive work-up was done to exclude infectious causes, including malaria, Lyme titre, venereal disease, blood cultures, human immunodeficiency virus, and lumbar puncture; all were negative. White blood count was 29.1 with 27% eosinophils. Lead, arsenic, mercury, folate, and B 12 levels were normal. Total complement levels were reduced. Immunoglobulin E was high, creatine kinase was high, and antineutrophil cytoplasmic antibody (ANCA) was persistent with pANCA (myeloperoxidase). A chest radiograph showed calcified granuloma in the left midlung. A muscle and nerve biopsy revealed prominent perivascular inflammatory infiltrate (T-cells) with eosinophils. Extensive perivascular and endoneural macrophages were seen. Electromyography was consistent with severe multifocal axonal sensorimotor polyneuropathy. Sensory nerve action potentials of the bilateral sural, right ulnar, and median nerves were unobtainable. Compound muscle action potential (CMAP) of the bilateral peroneal, left tibial, right median, radial, and ulnar nerves were unobtainable. CMAP of the right tibial, musculocutaneous, and left axillary were attenuated. Electromyography demonstrated abnormal spontaneous activity in all muscles studied. Discussion: This is a rare cause of peripheral neuropathy Churg-Strauss syndrome. Diagnostic criteria are peak eosinophils >1500/mm 3 , adult-onset asthma, systemic vasculitis involving 2 or more organs, with peripheral neuropathy dominating the clinical picture. Conclusion: It is important to familiarize physiatrists with this characteristic albeit rare cause of peripheral neuropathy. Early recognition and treatment has a good overall prognosis. When consulted, the patient was doing poorly in therapies; the cause was poor management of neuropathic pain. Once pain was treated, the patient's FIM™ instrument scores improved dramatically.