Prevalence and clinical features of HTLV neurologic disease in the HTLV Outcomes Study

Background: Almost 20 years after its discovery, the prevalence and clinical course of human T-lymphotropic virus type I ( HTLV-I) - associated myelopathy ( HAM, also known as tropical spastic paraparesis [TSP]) remain poorly defined. Whereas the causative association of HTLV-I and HAM/TSP is generally recognized, controversy still surrounds the relationship between HTLV-II and HAM/TSP. Methods: The HTLV Outcomes Study ( HOST - formerly Retrovirus Epidemiology Donor Study [ REDS]) is a prospective cohort study including 160 patients with HTLV-I, 405 patients with HTLV-II, and 799 uninfected controls who have been followed every 2 years since 1990 - 1992. Clinical outcomes are measured by health interviews and examinations, and blood samples are obtained. Results: Six cases of HTLV-I associated myelopathy (3.7%, 95% CI 1.4 to 8.0) and four cases of HTLV-II myelopathy (1.0%, 95% CI 0.3 to 2.5) have been diagnosed since the formation of the cohort. There have been no cases of HAM/TSP diagnosed among HTLV-negative subjects (0.0%, 95% CI 0.0 to 0.5). Clinical features of the cases include lower extremity hyperreflexia, variably associated with weakness, spasticity, and bladder dysfunction. Conclusions: Systematic screening of HTLV-infected blood donors reveals a high prevalence of HAM/TSP. The clinical course of HAM/TSP appears highly variable. HTLV-II - associated myelopathy generally presents with milder and more slowly progressive signs and symptoms.