Acute manifestations and neurologic sequelae of Epstein-Barr virus encephalitis in children.

BACKGROUND: Complications of Epstein-Barr virus (EBV) infection are diverse and include a number of neurologic manifestations such as meningitis, meningoencephalitis, cerebellitis, cranial neuritis and others. In general encephalitis caused by EBV in pediatric patients has been considered a self-limited illness with few or no sequelae. METHODS: Charts were reviewed from all patients < 18 years of age admitted to or discharged from the State University of New York Health Science Center at Syracuse between 1982 and 1992 with a diagnosis of encephalitis or meningo-encephalitis. Eleven cases of EBV encephalitis diagnosed during a 10-year period were reviewed to characterize the clinical and laboratory findings in the acute setting and the extent of neurologic sequelae on follow-up. RESULTS: Acute neurologic manifestations were diverse and included combative behavior (55%), seizures (36%), headache (36%) and evidence of focal involvement (27%). Classic findings of infectious mononucleosis were noted infrequently; 18% each had pharyngitis, adenopathy, positive heterophile antibody tests or atypical lymphocytosis. Two patients (18%) had abnormal neuroimaging studies, one in the acute stage and the other at the time of follow-up. Seven patients (64%) had abnormal electroencephalograms (EEGs) in the acute setting; of these three had persistent abnormalities on follow-up. Forty percent developed persistent neurologic abnormalities including global impairment, perseverative autistic-like behavior and persistent left upper extremity paresis. CONCLUSIONS: Classic signs, symptoms and laboratory findings in infectious mononucleosis may be absent in Epstein-Barr virus encephalitis. Neurologic sequelae occur in a substantial number of patients.

Demyelinating and gliotic cerebellar lesions in Langerhans cell histiocytosis.

PURPOSE: To describe the involvement of the cerebellum by a gliotic and demyelinating process in Langerhans cell histiocytosis. METHODS: A retrospective analysis of all (N = 30) cases of Langerhans cell histiocytosis followed at our institution since 1975 yielded four patients with CT and/or MR evidence of cerebellar abnormalities. RESULTS: Four patients manifested strikingly similar findings of symmetric nonenhancing hypodensities in the dentate nuclei region of the cerebellum, which were hypointense on short-repetition-time/short-echo-time MR and hyperintense on long-repetition-time/long-echo-time MR. Biopsy in one patient yielded areas of demyelination, cell loss, and gliosis without histiocytic infiltration. CONCLUSION: Langerhans cell histiocytosis involves the cerebellum in a specific and poorly understood manner. Lesions on imaging may precede clinical findings by years. Lesions may occur in patients who have never experienced radiation therapy and may act as a marker for eventual central nervous system deterioration.

Prenatal symmetrical thalamic degeneration with flexion spasticity at birth.

The unusual occurrence of spasticity at birth with symmetrical thalamic damage was found in a male infant delivered at 36 weeks' gestation following an episode of traumatically induced premature labor at 32 weeks. The infant was found to be spastic in flexion with increased stretch reflexes, depressed primitive reflexes, and moderate flexion contractures. Computerized tomographic scans showed bilateral nonenhancing thalamic densities. Neuropathologically, the lateral thalamic nuclei and the red nucleus showed neuron loss, astrocytosis, and, as confirmed by electron microprobe analysis, calcified neurons. The striatum was uninvolved. These findings closely resemble those reported as "symmetrical thalamic degeneration in infancy" and are strongly reminiscent of the pattern of thalamic involvement frequently seen in status marmoratus. It would appear that there is a period during perinatal life in which the lateral thalamus can be rendered vulnerable to hypoxic-ischemic injury, and that the thirty-second week of gestation must be included within this period.

Cerebral capillary telangiectasis in an infant. Coincidental Association with hypsarrhythmia.

We studied a case of cerebral capillary telangiectasis in infancy. The patient, who had received treatment with intramuscular corticotropin for hypsarrhythmia, suffered disseminated intravascular coagulation and died on the 66th day of life. Neuropathological examination disclosed the presence of two fresh hemorrhages of moderate size within the cerebral hemispheres, one of which seemed to have originated from a capillary telangiectasis within the left frontoorbital white matter.

Coincidence of neurofibromatosis and myotonic dystrophy in a kindred.

Neurofibromatosis and myotonic dystrophy have occurred in ten members of a nonconsanguineous family with a high degree of concordance. The expression of neurofibromatosis is peripheral, and the expression of myotonic dystrophy has produced at least moderately severe disability. Neither disease has appeared to alter the phenotypic expression of the other when both have occurred simultaneously. Secretor typing supports the assumption that the myotonic dystrophy in this family is the commonly recognised secretor-linked entity. The segregation pattern of the two disorders in this family suggest the possibility of close linkage between the loci for neurofibromatosis and myotonic dystrophy.

Dystonia associated with carbamazepine administration: experience in brain-damaged children.

Carbamazepine is an anticonvulsant most effective in treating complex partial and generalized tonic-clonic seizures. We have cared for three children in whom four episodes of dystonia proceeding to opisthotonus occurred in association with carbamazepine use. The patients, a 4-year-old with microcephaly and severe retardation, a 1-year-old with cerebral dysgenesis, and a 5-year-old with spastic quadriplegia and mild retardation, all had seizures unresponsive to multiple anticonvulsant combinations. In all three patients carbamazepine was introduced and gradually increased to a maximum dosage of 25 mg/kg of body weight per day. Dystonic symptoms began two to three weeks after introduction of therapy and subsided within three weeks after discontinuation. In one child, a second course of carbamazepine resulted in a return of the dystonia. The currently available clinical and neuropharmacologic data suggest that carbamazepine may be an antagonist of dopamine and that this property is responsible for the production of dystonia.

Central nervous system lesions in childhood leukemia.

Central nervous system (CNS) lesions were found in 85 of 91 children with acute leukemia. Cerebral atrophy was the most common lesion, occurring in 65 percent. Atrophy was most severe in children treated with intrathecal methotrexate alone or in combination with radiation therapy, in children youngest at time of onset of leukemia, and in children in whom duration of leukemia was shortest. Longer duration was the determining factor among children who were older at onset. Lesions previously reported by other investigators, such as leptomeningeal infiltration of leukemic cells, infection, hemorrhage, degenerative grey and white matter disease and leukoencephalopathy were also seen. Central pontine myelinolysis occurred in four children.

Rickets associated with long-term anticonvulsant therapy in a pediatric outpatient population.

Over a 12-month period, an ambulatory pediatric population receiving long-term anticonvulsants was surveyed for the presence of biochemical and radiologic rickets. There were 74 treated children and 95 matched controls. Elevations of serum alkaline phosphatase activity occurred in 31 of the 74 (42%) treated children (23 of 47 children between 2 and 10 years and 8 of 21 children between 10 and 16 years). This frequency of abnormal values was significantly greater than that which occurred in our control population. Calcium and phosphorus abnormalities were minimal in both treated and control populations. Radiologic rickets occurred in 6 of the 74 (8%) of the treated children and in none of the control population. Neither the severity of the rickets nor the degree of hyperalkaline phosphatasemia were correlated with age of the patient, duration, and/or dose of anticonvulsant therapy.