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Auditory brain stem responses in neurological disease.

A sequence of seven low-amplitude (nanovolt) potentials that occur in the initial 10 msec following click signals can be recorded from scalp electrodes in human subjects using computer averaging techniques. The potentials, termed auditory brain stem responses, are thought to be the far-field reflection of electrical events originating in the auditory pathway during its course through the brain stem. We have studied auditory brain stem responses in a variety of neurological disorders and found them to be of assistance in evaluating the mechanisms of coma, the localization of midbrain and brain stem tumors, the localization of demyelination of the brain stem, and tumors, the localization of demyelination of the brain stem, and the presence of diminished brain stem circulation.

Acoustic Stimulation↗

Thiamine triphosphate levels and histopathology. Correlation in Leigh disease.

Thiamine and thiamine triphosphate (TTP) values were assayed in various brain regions in 11 controls and 13 patients with subacute necrotizing encephalomyelopathy (SNE, Leigh disease). The TTP values of normal brain were 5% of the total thiamine value. The relative TTP (or % TTP) level was consistently low in the pons, midbrain, and cerebellum of all the SNE brains. Twenty-five percent of the SNE brains had normal TTP levels in the frontal region. The TTP values correlated with the degrees of pathologic involvement in all sampled regions of the brain except the cerebellum. The concentration of thiamine in the mammillary bodies exceeded its concentration elsewhere in both control and SNE brains. The finding of low TTP levels in morphologically abnormal regions supports the hypothesis that TTP deficiency is etiologically related to SNE.

Adult↗

Development of cerebrovascular architecture and its relationship to periventricular leukomalacia.

Using microangiography combined with benzidine stains, we studied the development of vessels in the meninges, cortex, and white matter in 30 neonates, 16 infants and children, and eight neonates with periventricular leukomalacia. The vessels of the deep white matter, especially the ventriculofugal arteries, are useful as an index of cerebrovascular maturity. The lesions of primary leukomalacia utilizing the combined radiographic-histological techniques are localized to the ends of ventriculofugal arteries or between medullary arteries. The infants with leukomalacia have either poorly developed vessels or severe clinical complications producing ischemia or both.

Adolescent↗

Periventricular leukomalacia. A one-year autopsy study.

We review the frequency and distribution of periventricular leukomalacia (PVL) in consecutive neonatal autopsies in a complete 12-month period. Periventricular leukomalacia occurred in 88% of the high-risk infants. The demographic factors that define risk are a birth weight between 900 and 2,200 g and a survival of six days or more. Virtually all infants with PVL suffered severe clinical insults, and/or had additional necrotic lesions in their viscera. Preferentially, PVL occurs in the cerebral axons in the occipital and sensory-motor radiations. The frequency with which this lesion is found at autopsy may correspond to the frequency with which visual, auditory, and motor dysfunctions occur in surviving premature infants. Electrophysiologic investigations of the high-risk premature infant seem warranted.

Birth Injuries↗

Perinatal risk factors for the development of extensive cystic leukomalacia.

Seventeen infants with extensive cystic leukomalacia without associated intraventricular hemorrhages were compared with 34 infants with normal cranial ultrasound findings and 34 infants with large intraventricular hemorrhages to establish whether different risk factors could be elicited for these lesions. Adverse obstetric factors that were associated with extensive leukomalacia were the place of birth (outborn [ie, not born at our hospital but transferred here after delivery]) and the need for emergency cesarean section. Neonatal risk factors, with the exception of gestational age, were equally distributed between the infants with the two types of lesions. The results of this study suggest that extensive cystic leukomalacia and large hemorrhages have similar predisposing factors, but the type of lesion that develops depends on the maturity of the infant.

Birth Weight↗