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Plasmapheresis in a Case of Eosinophilia-Myalgia Syndrome with Ascending Polyneuropathy

1991; Elsevier BV; Volume: 100; Issue: 2 Linguagem: Inglês

10.1378/chest.100.2.584

1931-3543

Emily Nolfo, Vance Wright-Browne, Michael L. Therrien, Anthony Ardolino, Zachary Macinski,

Cardiovascular Effects of Exercise

Eosinophilia-myalgia syndrome complicated by ascending polyneuropathy in a 40-year-old woman is described. High-dose intravenous steroids had no beneficial effect on the clinical course. Dramatic and rapid clinical improvement occurred with the use of plasmapheresis. The use of this therapeutic modality should be considered in patients with a similar clinical presentation.(Chest 1991; 100:584) Eosinophilia-myalgia syndrome complicated by ascending polyneuropathy in a 40-year-old woman is described. High-dose intravenous steroids had no beneficial effect on the clinical course. Dramatic and rapid clinical improvement occurred with the use of plasmapheresis. The use of this therapeutic modality should be considered in patients with a similar clinical presentation. (Chest 1991; 100:584) The eosinophilia-myalgia syndrome is a newly described condition associated with the ingestion of L-tryptophan.1Kilboume EM Swygert LA Philen RM et al.Interim guidance on the eosinophilia-myalgia syndrome..Ann Intern Med. 1990; 112: 85-87Crossref Scopus (66) Google Scholar We describe a patient with eosinophilia-myalgia syndrome and ascending polyneuropathy who showed rapid clinical recovery following treatment with plasmapheresis. The patient was a 40-year-old black woman with a history of mental retardation. Three weeks prior to admission she was seen for complaints of dyspnea and malaise. She was thought to have an upper respiratory tract infection, and a course of antibiotics was prescribed. Several days later she presented with no improvement in her symptoms. Physical examination was unremarkable except for a rectal temperature of 39.1°C. The chest x-ray film showed no abnormalities. The leukocyte count was 22.0 × 103Medsger TA Tryptophan induced eosinophilia-myalgia syndrome..N Engl J Med. 1990; 322: 926-928Crossref PubMed Scopus (45) Google Scholar cells/L. The differential cell count revealed 52 percent eosinophils. On August 16, 1989 (three months before the onset of symptoms), L-tryptophan at a dose of 1 g three times a day had been started for treatment of combative premenstrual behavior. In view of the recent institution of L-tryptophan therapy, eosinophilia-myalgia syndrome was suspected. She was admitted to the hospital on November 8, 1989, and the L-tryptophan was discontinued. Hydrocortisone, 50 mg, administered intravenously every 4 h was begun. Over the next several days, she developed an ascending flaccid paralysis and became ventilator-dependent. An electromyogram and nerve conduction studies showed widespread denervation polyneuropathy and low compound action potentials, consistent with Guillain-Barré syndrome. Three serial lumbar punctures done at one-week intervals showed normal cerebrospinal fluid protein. An empiric trial of plasmapheresis three times a week was begun on the tenth hospital day. On the 17th hospital day, the first signs of neurologic improvement were noted. The patient was soon extubated. On the 26th hospital day, she had grade 4/5 strength in the upper extremities and grade 3/5 strength in the lower extremities. Plasmapheresis was discontinued on the 33rd hospital day. She had a bilateral foot drop. She was discharged 104 days after admission and is now doing well. The etiology of eosinophilia-myalgia syndrome remains controversial. Initial epidemiologic investigation focused on the identification of a contaminant.2Hertzman PA Blevins WL Mayer J Greenfield B Ting M Gleich GJ Association of the eosinophilia-myalgia syndrome with the ingestion of L-tryptophan..N Engl J Med. 1990; 322: 869-873Crossref PubMed Scopus (295) Google Scholar,3Medsger TA Tryptophan induced eosinophilia-myalgia syndrome..N Engl J Med. 1990; 322: 926-928Crossref PubMed Scopus (45) Google Scholar Retrospective epidemiologic studies have identified a significant association of the syndrome with a specific retail lot of L-tryptophan from a single Japanese manufacturer.4Belongia EA Hedberg CW Gleich GJ et al.An investigation of the cause of the eosinophilia-myalgia syndrome associated with tryptophan use..N Engl J Med. 1990; 323: 357-365Crossref PubMed Scopus (309) Google Scholar,5Slutsker L Hoesly TC Miller L et al.Eosinophilia-myalgia syndrome associated with exposure to tryptophan from a single manufacturer..JAMA. 1990; 264: 213-217Crossref PubMed Scopus (135) Google Scholar Ascending polyneuropathy complicating the syndrome previously has been described.6Centers for Disease Control Eosinophilic-myalgia syndrome and L-tryptophan containing products—New Mexico, Minnesota, Oregon and New York, 1989..MMWR. 1989; 38: 785-788Google Scholar One such patient was treated with plasmapheresis and relapsed after it was discontinued. She did not respond to a second course of therapy.6Centers for Disease Control Eosinophilic-myalgia syndrome and L-tryptophan containing products—New Mexico, Minnesota, Oregon and New York, 1989..MMWR. 1989; 38: 785-788Google Scholar The precise clinical parameters prompting initiation and cessation of plasmapheresis were not discussed in this case. We can only postulate that the successful outcome in our case was due to early initiation of therapy and continued treatment for a prolonged period of time. All reported deaths from eosinophilia-myalgia syndrome have been associated with ascending polyneuropathy. Plasmapheresis may either replace a depleted plasma constituent or remove an abnormal one.7Shumak KH Rock GA Therapeutic plasma exchange..N Engl J Med. 1984; 310: 762-771Crossref PubMed Scopus (202) Google Scholar It is likely that the removal of an abnormal constituent (contaminant, toxic metabolites, immune complexes, or mediators of inflammation) was the reason for the patients improvement. The 1-week lag period separating the start of plasmapheresis and the improvement of clinical symptoms could be due to the gradual removal of substances deposited in tissues. We suggest that plasmapheresis be considered in patients with eosinophilia-myalgia syndrome complicated by ascending polyneuropathy. We also urge that further investigation of this treatment modality be pursued.