PEDIATRICS Vol. 91 No. 4 April 1993, pp. 736-741
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Neurologic History and Examination Results and Their Relationship to Human Immunodeficiency Virus Type 1 Serostatus in Hemophilic Subjects: Results From the Hemophilia Growth and Development Study

James F. Bale Jr MD1, Charles F. Contant PhD2, Bhuwan Garg MD3, Ann Tilton MD4, David M. Kaufman MD5, and Warren Wasiewski MD6

1 From The University of Iowa College of Medicine, Iowa City
2 From the Baylor College of Medicine, Houston, TX
3 From the Indiana University College of Medicine, Indianapolis
4 From the Tulane University School of Medicine, New Orleans, LA
5 The Mount Sinai Medical Center, New York, NY
6 From the Lancaster General Hospital, Lancaster, PA.

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 ge12 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.

Key Words: hemophilia • growth • neurologic function • development • human immunodeficiency virus

Submitted on June 30, 1992
Accepted on November 12, 1992




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