LONGITUDINAL NEUROLOGICAL FOLLOW-UP OF A GROUP OF HIV-SEROPOSITIVE AND HIV-SERONEGATIVE HEMOPHILIACS - RESULTS FROM THE HEMOPHILIA GROWTH AND DEVELOPMENT STUDY

Background. Boys and young men with hemophilia treated with factor inf usions before 1985 had a substantial risk of acquiring the human immun odeficiency virus (HIV) and the acquired immunodeficiency syndrome. Th is study was designed to assess the effects of HIV and hemophilia per se on neurological function in a large cohort of subjects with hemophi lia, and to investigate the relationships between neurological disease and death during follow-up. Methods. Three hundred thirty-three boys and young men (207 HIV seropositive and 126 HIV seronegative) were eva luated longitudinally in a multicenter, multidisciplinary study. Neuro logical history and examination were conducted at baseline and annuall y for 4 years. The relationship between neurological variables, HIV se rostatus, CD4+ cell counts, and vital status at the conclusion of the study was examined using logistic regression models. Results. The risk s of nonhemophilia-associated muscle atrophy, behavior change, and gai t disturbance increased with time in immune compromised HIV-seropositi ve subjects compared with HIV seronegative or immunologically stable H IV-seropositive subjects. The risk of behavior change in immune compro mised HIV-seropositive hemophiliacs, for example, rose to 60% by year 4 versus 10% to 17% for the other study groups. Forty-five subjects (1 3.5%), all of whom were HIV seropositive, died by year 4. Subjects who died had had increased risks of hyperreflexia, nonhemophilia-associat ed muscle atrophy, and behavior change. Conclusions. These results ind icate that immune compromised, HIV-seropositive hemophiliacs have high rates of neurological abnormalities over time and that neurological a bnormalities were common among subjects who later died. By contrast, i mmunologically stable HIV-seropositive subjects did not differ from th e HIV-seronegative participants. Hemophilia per se was associated with progressive abnormalities of gait, coordination, and motor function.