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In a prospective study of the growth and neuropsychologic function of hemophilic subjects, 333 boys, median age of 12.3 years, had baseline neurologic examinations. The study population included 207 individuals (62%) who were seropositive for human immunodeficiency virus type 1 (HIV-1). Overall results indicated that 11% had abnormalities of cranial nerve function, 17% had abnormal deep tendon reflexes, 23% had abnormal strength, 25% had abnormal coordination, and 31% had abnormal tone, bulk, or range of motion. By contrast, 2% or fewer displayed abnormal movements or had abnormal pain or vibratory sensation, or altered mental status. Abnormalities were more common in older hemophilic subjects (eg, 67 [38%] of 177 subjects ≥12 years of age had abnormal tone, bulk, or range of motion vs 36 [23%] of 156 subjects < 12 years of age). When compared with regard to HIV-1 status, HIV-seronegative and HIV-seropositive subjects did not differ with regard to head circumference or the frequency of abnormalities of cranial nerve function, sensation, muscle strength, or coordination. However, deep tendon reflexes and tone, bulk, or range of motion were more frequently abnormal in HIV-1-seropositive individuals. More HIV-1-positive subjects had at least one increased deep tendon reflex (13/207 [6.3%] vs 1/126 [0.8%] in seronegatives) and more had non-hemophilia-related decreases in muscle bulk (7/207 [3.4%] vs 0/126 in seronegatives). These results indicate that hemophilia causes substantial neurologic dysfunction and that certain findings, such as changes in muscle-stretch reflexes or muscle bulk, may also reflect the neurologic consequences of HIV infection.

Background. Boys and young men with hemophilia treated with factor infusions before 1985 had a substantial risk of acquiring the human immunodeficiency virus (HIV) and the acquired immunodeficiency syndrome. This study was designed to assess the effects of HIV and hemophilia per se on neurological function in a large cohort of subjects with hemophilia, 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 evaluated longitudinally in a multicenter, multidisciplinary study. Neurological history and examination were conducted at baseline and annually for 4 years. The relationship between neurological variables, HIV serostatus, CD4+ cell counts, and vital status at the conclusion of the study was examined using logistic regression models.Results. The risks of nonhemophilia-associated muscle atrophy, behavior change, and gait disturbance increased with time in immune compromised HIV-seropositive subjects compared with HIV seronegative or immunologically stable HIV-seropositive subjects. The risk of behavior change in immune compromised HIV-seropositive hemophiliacs, for example, rose to 60% by year 4 versus 10% to 17% for the other study groups. Forty-five subjects (13.5%), all of whom were HIV seropositive, died by year 4. Subjects who died had had increased risks of hyperreflexia, nonhemophilia-associated muscle atrophy, and behavior change.Conclusions. These results indicate that immune compromised, HIV-seropositive hemophiliacs have high rates of neurological abnormalities over time and that neurological abnormalities were common among subjects who later died. By contrast, immunologically stable HIV-seropositive subjects did not differ from the HIV-seronegative participants. Hemophilia per se was associated with progressive abnormalities of gait, coordination, and motor function.