Faculty Bibliography

A 6-year-old boy with acquired immunodeficiency syndrome (AIDS) developed aphasia and quadriplegia 3 months before his death. Cerebral vascular ectasia and multiple cerebral infarcts were noted on premortem radiological studies. Postmortem evaluation revealed diffuse aneurysmal dilatation of the circle of Willis associated with fresh and organizing thrombi, destruction of the elastic lamina, and marked intimal fibroplasia. Multiple cerebral infarcts and subacute AIDS encephalitis with basal ganglia calcification were also present. Immunohistochemistry with a monoclonal antibody (anti-gp41) to human immunodeficiency virus (HIV) demonstrated positively stained cells in the arterial wall of the circle of Willis and in the cerebral parenchyma. Double immunostaining demonstrated that gp41-positive cells in the circle of Willis were also positive for a macrophage marker or leukocyte-common antigen, but not with an endothelial marker. Some macrophages or microglia in the cerebrum were also colabeled with anti-gp41. These results suggest that HIV may be directly involved in vascular pathology associated with pediatric AIDS.

In a retrospective study involving several medical centers we identified 52 patients under age 5 years who met the adult clinical criteria for brain death and had at least one EEG with electrocerebral silence. Of the 52 patients, 31 died spontaneously and 21 were disconnected from the respirator. Repeat EEGs were obtained in 28 patients, and in all electrocerebral silence persisted. The study suggests that clinical criteria similar to those used for adults in the determination of brain death can also be applied to children above age 3 months and that a single EEG with electrocerebral silence is sufficient to confirm brain death in this age group.

Central nervous system (CNS) dysfunction was documented in 61 of 68 infants and children with symptomatic human immunodeficiency virus infection. The most frequent manifestations included acquired microcephaly, cognitive deficits, and bilateral pyramidal tract signs. Lymphoma of the CNS, cerebrovascular accidents, and CNS infection caused by conventional pathogens were documented in only ten children (15%). Neurologic deterioration in 11 children was subacute but steadily progressive; in 31 the course was more indolent and began with a plateau. Of these 31 children, 13 had further neurologic deterioration and the conditions of three improved. Seventeen children had a static course with cognitive deficits (seven children) or cognitive plus neurologic impairment (ten children). Neuroradiologic studies in the children with a subacute progressive or plateau course disclosed cerebral atrophy, white matter abnormalities, and calcification of the basal ganglia. Postmortem findings included variable degrees of inflammatory response, multinucleated cells, calcific vasculopathy, and pyramidal tract degeneration. Computed tomographic studies of the children with a static course were normal or showed mild atrophy, but poor brain growth was documented by serial head circumference measurements.

Developmental abnormalities in 16 pediatric patients with AIDS or AIDS-Related Complex (ARC) were previously described. Neurological deterioration was in evidence on follow-up in 9 of the children, 5 died since the original assessments were performed. Ten patients were reevaluated 14 months later by cognitive testing. Two showed greater progress than expected on the basis of earlier test results; 6 showed the expected level of developmental progress; and the remaining 2 showed regression in cognitive functioning. All patients who exhibited regression in their developmental course showed deterioration in their neurological examinations. Developmental progression was noted in some children who on follow-up serial examinations exhibited a clinically deteriorating neurological picture. Pediatric AIDS patients manifest variable neurodevelopmental courses. As a result, rehabilitative intervention services must be tailored to meet individual needs.

The cognitive status of 12 clinically stable children with congenital HIV infection, nine of whom were neurologically impaired, age three to nine years, was assessed using the Kaufman ABC test. Seven of the children had ARC; five were diagnosed as having AIDS. The same children were evaluated by standard neurologic examinations with Characterization of tone and fine motor functioning. Two were diagnosed as being mildly retarded; six were borderline; and four tested as being of average intelligence. Visual-spatial perceptual based functioning was found to be more impaired than were abstract reasoning and verbally mediated skills in six (50%) of the patients. This pattern of impaired information processing was found irrespective of overall cognitive status. On neurological and physiatric examination abnormal developmental histories were obtained, or poor fine motor coordination, abnormal tone and gait, and impaired rapidly alternating movements were found in 9 of the 12 subjects. These findings suggest selective impairment in distinct areas of neurologic and neuropsychological functioning during stable phases of HIV infection in a select group of children. These patterns appear to persist over time. They differ from the clustering of impaired skills seen in children of comparable socio-cultural backgrounds without HIV infection. Similarities in functioning are noted between this subgroup of children with AIDS and ARC and comparable groups with cerebral palsy.