Inflammatory demyelinating peripheral neuropathies associated with human T-cell lymphotropic virus type III infection.
Nine patients with inflammatory demyelinating polyneuropathies (IDP) were found to have human T-cell lymphotropic virus type III (HTLV-III) infection. The 8 men, 6 of whom were homosexual, and 1 woman, a former intravenous drug user, presented with progressive weakness. Two had lymphadenopathy but all were otherwise asymptomatic. Six had chronic IDP and 3 had Guillain-Barré syndrome. In addition to an elevated cerebrospinal fluid (CSF) protein level (mean, 193 mg/dl), most patients had cerebrospinal fluid pleocytosis (mean, 23 cells/mm3), a distinctive feature. All had reduced T4:T8 T-cell ratios. Results of nerve conduction studies were characteristic of demyelination. Nerve biopsies revealed intense inflammatory cell infiltrates and macrophage-mediated demyelination. The patients recovered either spontaneously or following treatment with corticosteroids or plasmapheresis. During a mean interval of 20 months after presentation, only 1 patient had developed acquired immune deficiency syndrome. Patients with HTLV-III infection have disordered immune function, and the mechanism of the development of the IDP is likely to be immunopathogenic. As a result of our experience, we suggest that all patients with IDP be tested for evidence of HTLV-III infection. We also found, although in uncontrolled trials, that treatment with either prednisone or plasmapheresis was followed by clinical improvement; since plasmapheresis is not likely to further depress cell-mediated immunity, we suggest that it be the initial therapy.
D000163:Acquired Immunodeficiency Syndrome / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D000293:Adolescent; D000328:Adult; D001706:Biopsy; D004568:Electrodiagnosis; D005260:Female; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D010525:Peripheral Nerves / Q000473:pathology*; D010523:Peripheral Nervous System Diseases / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy