The relationships between viral load in plasma and cerebrospinal fluid (CSF) and computed tomography (CT) brain scan abnormalities were studied in 39 children between 0.5 and 13 years of age with symptomatic HIV-1 disease. Quantitative RNA PCR was used to determine HIV-1 RNA levels and a semiquantitative analog rating technique was used to evaluate non-contrast CT brain scans. CSF HIV-1 RNA copy number correlated significantly with CT brain scan ratings for severity of cortical atrophy (r = 0.36; P < 0.05) but not with ratings of intracerebral calcifications (r = -12; NS). The difference between these two correlations was significant (P < 0.05). Plasma HIV-1 RNA copy number did not correlate significantly with any CT brain scan ratings or with CSF viral load (r = 0.05; NS). Severity of cortical atrophy appeared to reflect the level of viral load in the CSF, supporting the notion that active HIV-1 replication in the CNS is at least in part responsible for such brain abnormalities in children. The lack of correlation of intracerebral calcifications with other CT brain scan abnormalities as well as with CSF viral load suggests that this lesion is relatively independent and may reflect a different neuropathologic process.

Objectives:To longitudinally assess the receptive and expressive language functioning of children with symptomatic HIV disease and to explore the relationship between immune status, computed tomography (CT) brain scan abnormalities, and language dysfunction over time. Methods:Children with symptomatic HIV infection were administered an age-appropriate standardized comprehensive language test and general cognitive measure prior to starting antiretroviral therapy (n = 44) and again after 6 months (n = 29) and 24 months (n = 17). CD4 percentage and CT brain scans were also obtained at each evaluation. Results:Expressive language was significantly more impaired than receptive language at the baseline, 6- and 24-month evaluations. No significant changes over time were found in receptive or expressive language from baseline to after 6 months of antiretroviral therapy, but despite treatment, language scores declined significantly between 6 and 24 months. Overall cognitive function, however, remained stable from baseline to 24 months. Age-adjusted CD4 percentage increased significantly over the initial 6 months, then remained stable. Overall CT brain scan severity ratings did not change significantly over 24 months. Conclusion:Expressive language was consistently more impaired than receptive language over 24 months, further supporting an earlier finding that expressive language was differentially affected by HIV in children with symptomatic disease. Both receptive and expressive language declined significantly after 24 months despite antiretroviral therapy, although overall cognitive function remained stable. Thus, functioning in some domains may be more vulnerable to the effects of HIV and global measures of cognitive ability may mask such differential changes in specific brain functions.

Peripheral neuropathy is infrequently reported in children with HIV infection, but may be underrecognized. To provide a better understanding of the patterns of peripheral neuropathy in these children, we surveyed the charts of 50 children with HIV infection referred to the EMG laboratory at the National Institutes of Health for evaluation of suspected peripheral neuropathy. Twelve children had an abnormal nerve conduction study. The findings suggested a distal sensory or sensorimotor axonal neuropathy in seven children, median nerve compression at the carpal tunnel in three, a demyelinating neuropathy in one child, and a lumbosacral polyradiculopathy in one adolescent. Distal symmetric polyneuropathy occurred mostly in older-aged children.

The purpose of this study was to correlate the relative risk for neurodevelopmental handicap at age 5 with the severity of neonatal neuroimaging abnormality; and determine the relationship between neonatal neuroimaging and measures of intellectual status, academic skills, neuropsychologic deficits, and neuromotor dysfunction. The study population consisted of 152 ECMO-treated neonates who had had routine cranial ultrasonography with followup CT scans and had undergone comprehensive neurodevelopmental evaluation at age five. The cohort was divided into four groups based on an independently derived neuroimaging score: No-Lesion: N=88; Mild: N=38; Moderate: N=12; Severe: N=14). Standardized testing at age five included a complete neuropsychological assessment, a neurological evaluation, and assessment of gross motor and fine motor function. The occurrence rate of handicap by severity of neuroimaging was: No-Les=12%; Mild=13%; Moderate=32%; Severe=58%. Severity of neonatal neuroimaging was significantly associated in a stepwise function with intellectual status (FSIQ, VIQ, PIQ), academic skills(WRAT-R arithmetic subtest), neuropsychologic deficit (receptive language, expressive language, verbal memory, visual memory, visual/spatial, visual motor, and attention/executive function), and psychometric measures of neuromotor function (grip strength and manual dexterity). Comprehensive evaluation in childhood following presumptive neonatal brain injury provides an opportunity to study neurologic compensation and recovery and provides important clinical information which can impact patient care.

Objective: To evaluate the clinical significance of computed tomographic brain scan abnormalities observed in children with symptomatic human immunodeficiency virus disease.Patients: Eighty-seven previously untreated children with symptomatic human immunodeficiency virus type 1 disease.Methods: General levels of cognitive functioning, obtained from age-appropriate intelligence tests, and social-emotional behavior were correlated with computed tomographic brain scan abnormality ratings.Results: A significant relation between computed tomographic brain scan abnormalities and cognitive dysfunction as well as aberrant behavior was found, which appeared stronger in (younger) vertically infected children compared with transfusion-infected patients. Calcifications, independent from the degree of brain atrophy, were associated with significantly greater delays in neurocognitive development.Conclusion: Computed tomographic brain scan abnormalities, even when mild, were of clinical significance, suggesting that human immunodeficiency virus-associated central nervous system compromise is a continuous process and that scans may be helpful at baseline in defining patients at risk and for monitoring them during therapy.

A single case of Wilson's hepatolenticular degeneration was imaged by computed tomography and magnetic resonance (1.5 Tesla). Findings included generalized cerebral atrophy seen both on computed tomography and magnetic resonance images. T1-weighted images revealed a slight symmetric hypointensity in the lentiform nuclei and thalamus. T2-weighted images demonstrated marked symmetric hypointensity in the lentiform nuclei. These hypointensities are ascribed to the deposition of intracellular hemosiderin (and perhaps copper), which produce heterogeneous magnetic susceptibility and preferential T2-proton relaxation. Areas of increased signal activity on T2-weighted images were also seen in areas of presumed demyelination known to occur in Wilson's disease.