Patrick F. Bray

Epstein‐Barr nuclear antigen and viral capsid antigen antibody titers in multiple sclerosis

To characterize the antibody response to the Epstein-Barr virus (EBV) in MS, we studied serum anti-EBV nuclear antigen (anti-EBNA) and anti-EBV capsid antigen (anti-EBVC A) titers. Both titers were assayed in 93 age- and sex-matched pairs of MS patients and controls. Anti-EBVCA titers were measured by indirect immunofluorescence and anti-EBNA titers by anticomplement immunofluorescence. The seropositivity rate of both anti-EBVCA and anti-EBNA in MS patients was l00%, compared with 84% in controls (p < 0.0001). Both anti-EBVCA and anti-EBNA titers were significantly higher in MS patients than in controls (p < 0.0001). The data suggest that EBV has a significant seroepidemiologic association with MS, but they do not define what role EBV antibodies play in the pathogenesis of the disease.

Clinical and EEG manifestations of an unusual aphasic syndrome in children.

Three cases of acquired aphasia in childhood associated with marked spike-wave discharges with temporal lobe predominance are described. The degree of aphasia paralleled the EEG abnormalities; spike-wave discharges were almost continuous when the language disorder was at its peak. Improvement in the language disorder coincided with reduction in the number of paroxysmal discharges or with a change to focal origin. The aphasia appears to be the result of an underlying pathophysiology causing the abnormal EEG activity, and not the result of a gross structural lesion.

The coincidence of neuroblastoma and acute cerebellar encephalopathy

Three patients with neuroblastoma and acute cerebellar encephalopathy are reported. This coincidence of disease occurs more commonly than one would expect on the basis of chance. This observation is supported by the finding of six other cases cited in the literature. The pathogenesis which underlies the relationship is obscure. The authors suggest that all patients with acute cerebellar encephalopathy be surveyed for an inapparent neuroblastoma. The search should include chest and skeletal roentgenograms, intravenous urogram, bone marrow examination for malignant cells, and determination of the urinary excretion of catecholamines and possibly cystathionine.

Neuroradiographic abnormalities in congenital cytomegalovirus infection.

To determine the spectrum of neuroradiographic abnormalities associated with congenital cytomegalovirus (CMV) infection, CT brain scans of 15 infants with symptomatic infection were reviewed. The initial CT scans were abnormal in 13 patients. Abnormalities included intracranial calcifications, cortical atrophy, ventricular enlargement, subdural effusions, porencephaly and polycystic encephalomalacia. Intracranial calcifications were present in 33% of the infants. In addition, three of the 15 infants developed progressive hydrocephalus which required ventriculoperitoneal shunt placement. These cases illustrate that congenital CMV infection causes a variety of structural CNS lesions and suggest that progressive hydrocephalus may be a relatively common consequence of symptomatic congenital CMV infection.

Brainstem tumors in children.

tral nervous system, postinfectious encephalopathies, demyelinating processes, cerebrovascular accidents, and congenital brain defects, including the all-inclusive concept of cerebral palsy. There were 12 brainstem gliomas in the 100 unselected autopsy-verified cases of intracranial tumor reported by Bailey, Buchanan, and Bucy.1 Walker and Hopple,2 in reviewing the same number of cases (loo), also reported 12 instances of pontine tumor verified by tissue examination or operative visualization. Again in 100 cases, Smith and Fincher3 reported an incidence of five pontine neoplasms. Ingraham and Matson4 reported 30 cases in their series of 313 brain tumors in children, 23 of which had the diagnosis confirmed at operation or autopsy, Out of 85 verified cases collected by Cuneo and Rand,5 there were two pontine tumors. In reporting 427 cases of brain tumor in children, Keith, Craig, and Kernohan6 described them in terms of pathologic type and position in relation to the tentorium, but did not break down the group further as to tumor location. It is the purpose of this paper to document the pattern of neurologic symptoms and signs in this disease and to re-emphasize the important diagnostic role of pneumoencephalography. More important probably than either of of neurologic signs P requently make early diag-

The clinical findings in a patient with nonketotic hyperglycinemia.

Extract: A male infant with hyperglycinemia presented with neonatal seizures and lethargy, but had neither ketosis nor hematologic abnormalities. Treatment with a low protein diet and glycine-binding agents was instituted at the age of 2 ½ months. Although greater alertness and reduced irritability were noted immediately, the treatment failed to prevent severe developmental retardation and persistent seizures.Speculation: The structural and metabolic basis for the severe neurological damage seen in this rare hereditary metabolic defect is unclear. It seems imperative, however, that prompt diagnosis and therapy be instituted if one expects to offer a better prognosis. Attempts to maintain normoglycmemia by using a low-protein diet and glycine-binding agents may be useful if serum glycine levels are monitored carefully.

Brain Changes in Newborns from an Intensive Care Unit

A study of the brains of 105 newborns (82 pre‐term and 23 term infants), who failed to survive after treatment for severe respiratory distress in an intensive care unit, revealed that 63 of the pre‐term infants died as a result of subependymal germinal plate hemorrhage. Additional lesions were found in the cerebral cortex, hippocampus, cerebral white matter and cranial nerve nuclei. The clinical course of 25 of the 105 infants was studied intensively. In these infants the severity of the lesions appeared to correlate roughly with the length of survival under anoxic and acidotic conditions.

A metabolic myopathy associated with chronic lactic acidemia, growth failure, and nerve deafness†

Two sisters with signs of growth failure, severe muscle weakness, and moderate neural deafness had identical abnormalities on muscle biopsy. Light microscopy revealed areas of “granular necrosis” which proved to be large and numerous mitochondria. Biochemical assessment has revealed large amounts of alanine in the urine, as well as hyperalaninemia and reduced clearance from the blood of an oral alanine load. Pyruvate concentration in the blood was found to be elevated, and each girl had marked lactic acidemia. This led to a fatal episode of lactic acidosis in the younger girl.

Carotid thrombosis following neck irradiation

Therapeutic irradiation may accelerate atherosclerosis, increasing the risk of vascular stenosis or occlusion several to many years following radiation. However, intimal damage following irradiation may result earlier in thrombosis without stenosis. This report discusses three cases of carotid occlusion that occurred within 3 years of moderate dose irradiation. Angiographic studies showed that occlusion occurred in the absence of atherosclerotic stenosis. A review of the literature supports the conclusion that people who receive neck irradiation are at risk not only for the delayed development of diffuse atherosclerosis but also for thrombotic occlusion within months to several years. We suggest that patients who develop neurological symptoms or signs following neck irradiation, regardless of age, dose of radiation, or interval since radiation, should be evaluated for carotid or vertebral artery disease.

A danger of haloperidol therapy in children

Attention is directed to the potential danger of therapy with haloperidol in children. Its value in reducing the excessive movements of Sydenham chorea must be weighed against the usual tendency of this disorder to remit spontaneously and the occasional tendency of the drug to cause castastropic adverse reaction