PEDIATRICS Vol. 91 No. 4 April 1993, pp. 742-746
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Effects of Human Immunodeficiency Virus and Immune Status on Magnetic Resonance Imaging of the Brain in Hemophilic Subjects: Results From the Hemophilia Growth and Development Study

Wendy G. Mitchell MD1, Marvin D. Nelson MD2, Charles F. Contant PhD3, James F. Bale Jr MD4, Don A. Wilson MD5, Timothy P. Bohan MD, PhD6, and Marc J. Fenstermacher MD7

1 From the Department of Neurology, University of Southern California School of Medicine and Childrens Hospital Los Angeles
2 From the Department of Radiology, University of Southern California School of Medicine and Childrens Hospital Los Angeles
3 From the Department of Neurosurgery, Baylor College of Medicine, Houston, TX
4 From the Department of Pediatrics, University of Iowa College of Medicine, Iowa City
5 From the Department of Radiology, University of Oklahoma, Oklahoma City
6 From the Departments of Neurology and Pediatrics, University of Texas, Houston
7 From the Department Radiology, University of Texas, Houston

To determine the effects of hemophilia and human immunodeficiency virus (HIV) infection on the nervous system, the authors examined the relationship of brain magnetic resonance imaging (MRI) findings to immunologic function and neurologic examination findings. Baseline examinations included physical and neurologic examination, immunologic and virologic testing, and MRI of the brain. On neurologic examination, muscle atrophy was considered to be related to hemophilia if adjacent joints had arthropathy due to bleeding. Muscle atrophy was considered non-hemophilia-related if unrelated to arthropathy or if muscle atrophy was diffuse. Subjects were boys aged 6 to 19 years, enrolled in a multicenter study of the effects of hemophilia and HIV infection on growth and development, all with congenital coagulopathies requiring factor infusions. Three hundred ten subjects had complete data including neurologic examination, T-cell subsets, HIV antibodies, and MRI. Subjects with HIV infection whose CD4+ counts were <200/µL were compared with subjects with HIV infection and CD4+ counts ge200/µL and with HIV-negative subjects, all of whom had CD4+ counts >200/µL. MRI studies were normal in 230. Abnormal MRI studies were more frequent in HIV-positive subjects with CD4+ counts <200 (29.4% abnormal compared with 17% in HIV-positive subjects with CD4+ counts ge200 and 15.3% in HIV-negative subjects). Diffuse atrophy accounted for most of the excess abnormalities in HIV-positive subjects with CD4+ counts <200 (77.3% of abnormal scans). Diffuse atrophy on MRI was associated with decreased muscle bulk on neurologic examination, but not with abnormal tendon reflexes. Four of six subjects with non-hemophilia-related diffuse muscle atrophy had cerebral atrophy on MRI. All 6 were HIV-positive and had CD4+ counts <200. Congenital abnormalities (primarily arachnoid cysts) were present in 12 subjects, not related to HIV or CD4+ status. Acquired focal abnormalities including both old hemorrhagic lesions (12 subjects) and nonhemorrhagic lesions (66 subjects) were equally frequent in HIV-positive and HIV-negative groups and did not differ by CD4+ count. Multifocal white-matter lesions, hyperintense on T2-weighted images, seen in both HIV-positive and HIV-negative subjects, are of uncertain significance. Of the multiple MRI abnormalities found, only diffuse cerebral atrophy appears to be associated with HIV infection, and only in subjects with compromised immunologic function.

Key Words: hemophilia • human immunodeficiency virus • acquired immunodeficiency syndrome • magnetic resonance imaging • cerebral atrophy

Submitted on June 30, 1992
Accepted on December 2, 1992




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