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An eight-day-old male infant with galactosemia presented with signs of increased intracranial pressure and no evidence of intracranial infection or hemorrhage. Computed tomographic scans demonstrated the presence of diffuse cerebral edema. With treatment, the edema gradually resolved, although it persisted longer within the white matter and was associated with transient bilateral pyramidal tract signs.

CT or postmortem examination demonstrated calcification of the basal ganglia in eight infants and children with acquired immune deficiency syndrome. Serial CT studies documented progression of both bilateral symmetric calcium densities and cerebral atrophy. Clinical features included progressive encephalopathy with dementia, and pyramidal tract signs. Postmortem examination of four children revealed variable degrees of calcific vasopathy of the basal ganglia, involving predominantly the putamen and globus pallidus.

Neurological complications occurred in 6 children, aged 6 months to 5 years, with acquired immune deficiency syndrome who were followed for 14 months. The most frequent manifestations included encephalopathies, acquired microcephaly, and pyramidal tract signs. Computed tomographic examinations showed variable degrees of cortical atrophy with ventricular dilatation and calcification. Electrophysiological abnormalities were demonstrated. Two children had documented central nervous system infections. Neurological deterioration resulted in dementia in 3 children. Cognitive impairment and developmental delays were evident in the other 3. Postmortem examination of the 3 children who died showed subacute cytomegalovirus encephalitis in 1; nonspecific hemispheric white matter changes, calcific vasopathy of the basal ganglia, and striking bilateral corticospinal tract degeneration in the second; and extensive calcific vasopathy of the basal ganglia and frontal centrum semiovale, and bilateral attenuation of the frontopontine and corticospinal tracts in the third.

Children with acquired immune deficiency syndrome (AIDS) display two types of clinical picture: a full-blown AIDS characterized by the presence of opportunistic infections and/or Kaposi's sarcoma and a prodromal stage now identified as AIDS-related complex (ARC). Neurological complications have been identified in infants and children with the disease. This paper discusses the developmental abnormalities in 16 pediatric patients, seven with AIDS and nine with ARC, ranging in age from six months to six years. In all cases, the mothers of these children either had ARC, AIDS and/or used intravenous drugs. Developmental histories showed delayed acquisition of milestones in most children following the diagnosis of AIDS or ARC, with delayed motor milestones consistently noted in both groups. Several children with AIDS actually lost milestones as their illness progressed; this has not occurred in the ARC group. Psychometric testing revealed more severe cognitive dysfunction in the group with AIDS. Involvement of the central nervous system was documented clinically, radiologically, and/or electrophysiologically in all patients with AIDS. In the ARC group the course of the illness has shown greater variability. Medical and social factors that may contribute to the developmental abnormalities are discussed.

Three patients developed prominent neurologic symptoms and signs associated with Schoenlein-Henoch purpura. A 7 1/2-year-old boy was seen with status epilepticus after a 2-week history of generalized headaches, irritability, and intermittent colicky abdominal pain. A left hemiparesis and a left homonymous hemianopia with a right gaze preference that were present on initial examinations gradually resolved, but a mild left arm paresis persisted. Cutaneous, renal, and joint involvement followed initial CNS manifestations. The second patient, a 7-year-old girl, had a complex partial seizure with secondary generalization and a postictal hemiparesis seven days after presentation with classic signs of Schoenlein-Henoch purpura. Behavioral changes were noted during the acute phase of the illness. The third patient, a 13-year-old boy, developed signs of a left brachial plexopathy and transient weakness of his right leg during a complicated course of Schoenlein-Henoch purpura. Review of the world literature indicates that headaches and mental status changes are the most frequent neurologic complications of Schoenlein-Henoch purpura, followed by seizures, focal neurologic deficits, mononeuropathies, and polyradiculoneuropathies. The vasculitis of Schoenlein-Henoch purpura can involve the nervous system and may add significantly to the morbidity of the illness.