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Biomedical subjects

M S Scher

Publications and source records attributed to M S Scher.

70 records · Page 4Linked to original sources

Neurophysiological and anatomical correlations in neonatal nonketotic hyperglycinemia.

Electroencephalographic (EEG) and brainstem auditory evoked response (BAER) findings have not been previously described and correlated with the pathological findings in an autopsied case of neonatal nonketotic hyperglycinemia (NKH). A 38 week gestation male infant presented within two hours of age with stimulus-evoked myoclonus and seizures in the context of progressive coma. Electrographic studies demonstrated cortical myoclonus and electrical seizures exquisitely localized to the midline region as well as a suppression-burst background disturbance. These vertex spike discharges were elicited after tactile stimulation. Prolonged intra-axial latencies for waves III and V were recorded on the BAER on the second day of life. Spongy leukodystrophy was noted on gross and microscopic examination of the brain involving all myelinated tracts especially in the reticular activating system, cerebellar peduncles and optic tracts. Neuropathological confirmation of brainstem involvement emphasizes the role of the nonspecific diffuse somatosensory projection system in the generation of myoclonus and stimulus-evoked seizures in the comatose patient with NKH.

Brain Diseases↗

Effects of anticonvulsants on hyperthermia-induced seizures in the rat pup.

We studied the effects of phenobarbital (PB), valproic acid (VPA), and phenytoin (PHT) on the electroencephalograms and behaviorally defined seizure threshold temperatures in rat pups exposed to ambient hyperthermia on the fifth day of life. Animals injected with 10, 20, or 40 micrograms PB/g body weight (GBW) or 80, 150, or 300 micrograms VPA/GBW daily from the second day of life showed seizure temperature thresholds that were about 1.0 degree C higher than those of controls. Pups injected with PHT had thresholds that were equal to or lower than those in controls despite blood levels greater than 20 micrograms/ml. Preictal rhythmic sharp wave activity appeared in VPA-injected animals at temperatures below the threshold temperature. All experimental animals showed electrographic epileptiform activity at the threshold temperatures. We conclude that this animal model shows similar anticonvulsant efficacies to those found in human febrile seizures. This model may be useful in determining the efficacy of potentially useful anticonvulsant drugs and their mechanisms of action.

Animals↗

Intraventricular hemorrhage in the full-term neonate.

Intraventricular hemorrhage (IVH) can occur in full-term newborns with a variety of clinical pictures. We studied five full-term infants who suffered IVH in the neonatal period and survived. No apparent cause for hemorrhage could be found in four. All had normal results of neurologic examinations at birth, and four had no major antecedent perinatal or postnatal difficulties. In 19 previously described full-term infants with IVH, no perinatal problems were noted in 45% of those who survived. Although more common in premature infants, IVH can occur in full-term infants and should be suspected when there is a sudden change in the neurologic status. In subsequent examinations, three of our five infants had mild spasticity and two appeared to be normal. The grading system developed for IVH in premature infants, while indicating severity of the hemorrhage, does not predict clinical outcome in full-term infants. A more definitive statement of outcome will require a longer period of observation.

Cerebral Hemorrhage↗

Pathologic myoclonus of the newborn: electrographic and clinical correlations.

Previous reports have described neonatal myoclonus as a benign movement in healthy premature newborns or as a clinical seizure in neonates with severe encephalopathies. The present report describes electroencephalographic-myoclonic correlations which occur independently from sustained electrical discharges in ten neonates. Cortical, reticular, and segmental types of neonatal myoclonus, similar to adult forms, are described.

Brain↗

Seizures and infarction in neonates with persistent pulmonary hypertension.

Seizures and cerebral infarction were observed in 10 near-term (greater than 36 weeks gestation) and term infants who had experienced moderate to severe peripartum asphyxia which resulted in persistent pulmonary hypertension. No patient received extracorporeal membrane oxygenation. Eight were outborn. Five patients were diagnosed initially with seizures during electroencephalography; electrical status epilepticus was demonstrated in five patients. The location of electrical seizures corresponded to the area of infarction in seven patients; the two remaining patients had white matter infarction. Prompt recognition of seizures and infarction in patients with persistent pulmonary hypertension is essential because of the higher probability of later neurodevelopmental difficulties.

Cerebral Infarction↗

Estimation of gestational age by electrographic, clinical, and anatomic criteria.

Current methods for estimating gestational age utilizing clinical parameters can be inaccurate in the very premature neonate. Neuropathologic studies have been used to verify these clinical estimates of gestational age. Estimates of maturity using neonatal electroencephalography have documented the evolution of regional and hemispheric electrical patterns at different gestational ages; however, electroencephalographic studies have not been compared with both anatomic brain studies and clinical examination criteria. Therefore, we studied 25 neonates with at least one electroencephalographic study prior to death and subsequent neuropathologic examination. Twenty-three estimates (92%) of gestational age in weeks, which were based on specific electrical patterns, agreed with sulcal-gyral development (e.g., the delineation of inferior frontal, superior temporal, calcarine sulci, and cytoarchitecture in various brain regions). Clinical (Ballard score) criteria and anatomic estimations of gestational age agreed in 18 of 24 patients (74%). Electroencephalographic estimates were significantly better than clinical estimates (p less than .05) for sick neonates who were less than or equal to 30 weeks gestational age.

Birth Weight↗

Intraventricular nafcillin-induced seizures in a neonate.

Treatment of cerebrospinal fluid shunt infections commonly involves a combination of intravenous and intraventricular antibiotics. We report a premature infant who developed seizures associated with intraventricular administration of nafcillin and discuss the need for electroencephalographic monitoring of patients receiving intraventricular antibiotics.

Abscess↗

A developmental marker of central nervous system maturation: Part I.

Sleep is the predominant behavioral state of the neonate and the neurophysiologic expression of cerebral function during rapid brain growth and maturation that characterizes the neonatal period. The ontogeny of electroencephalographic (EEG) sleep in the neonate can be monitored by serial EEG/polygraphic recordings. A variety of physiologic markers accompany neonatal sleep, such as specific EEG patterns, rapid eye movements, arousals, autonomic signs, and body movements. These parameters vary in occurrence and abundance during different segments of the neonatal sleep cycle. Time-dependent interrelationships among these different physiologic components can be assessed by visual inspection of EEG recordings to monitor specific patterns that appear during one or more sleep cycles. Mathematical modeling and computer technologies permit the study of interactions among neurophysiologic signals which are not apparent by visual inspection. An understanding of biological rhythm relationships among different sleep events offers insights into the effects of extrauterine environment on the development of the central nervous system in the healthy preterm neonate and the disturbances in the sleep EEG rhythm in abnormal neonates. Sleep EEG analysis may be useful prognostically because "normalization" of abnormal EEG patterns often occurs following the initial acute illness. Suitable, normative data from very premature neonates are needed before pediatric neurologists can rely on neonatal EEG as an indicator of brain maturation in this low-birth weight group. An understanding of the biologic significance of the ontogeny of neonatal sleep, as well as the clinical significance of perturbations in this sleep cycle with disease, will lead to more reliable analyses of sleep physiology for research and clinical purposes.

Central Nervous System↗

A developmental marker of central nervous system maturation: Part II.

Computer technology facilitates simultaneous comparison of changes among various neurophysiologic measures. Evaluating relationships among various sleep events provides information concerning normal central nervous system development, as well as perturbations in the electroencephalographic rhythms that accompany pathologic conditions in the neonate. Prognostic information may be derived from the "recovery" pattern of the abnormal electroencephalogram after acute neonatal illness.

Central Nervous System↗

EEG diagnoses of neonatal seizures: clinical correlations and outcome.

Electroencephalographic seizures were evaluated in 112 neonates. The first portion of the study involved 80 neonates with clinically identified abnormal movements, 8 of whom (10%) had electroencephalographic evidence of seizures coincident with this activity. Patients with abnormal movements (90%) had no concurrent electrical seizures. In the second part of the study, 40 infants who had electrical seizures were investigated. Eight of these infants had been identified during the first part of the study. Two-thirds of the patients (25) were premature. Sixteen patients (40%) died; 90% had brain lesions documented by computed tomography and/or postmortem study. Cerebral infarction and intraventricular hemorrhage were the most common lesions. One-third of the survivors (8 of 24 patients) were normal at a mean age of 3 years, while two-thirds had significant neurologic and developmental abnormalities. Neonatal seizures often are subtle, not associated with observable clinical expression, and associated with adverse development. Electroencephalographic confirmation is important in the evaluation of neonatal seizures.

Electroencephalography↗

Neurodevelopmental outcome of children with evidence of periventricular leukomalacia on late MRI.

Fifteen children, 8 months of age or older, from a neonatal follow-up program underwent magnetic resonance imaging and neurologic, cognitive, and language evaluations. Magnetic resonance imaging findings in all children included increased white matter signal on T2-weighted images and ventricular enlargement adjacent to regions of abnormal white matter. The extent of degree of abnormal white matter signal and the degree of sulcal prominence were variable. Twelve children had cerebral palsy; 5 children, 4 of whom had cerebral palsy, manifested significant sensory impairments. The median score on cognitive testing was 89; only 2 children exhibited severe-to-profound cognitive disability. Cognitive scores were stable on retesting. The degree of motor disability was correlated with the extent of white matter signal abnormality; however, cognitive outcome was not related to the extent and degree of white matter signal abnormality or to the degree of sulcal prominence. Despite the association of a major handicapping condition and periventricular leukomalacia, cognitive and language functioning may be relatively spared.

Child Development↗

Neonatal seizures: electroclinical dissociation.

Electroclinical dissociation is a phenomenon in which the clinical component of a seizure occurs at times with or without an electrical correlate. The epidemiology of this observation was studied in a neonatal intensive care unit from July, 1983 to December, 1988. Infants demonstrating electroclinical dissociation were compared to those having exclusively electroclinical seizures. Sixteen percent of infants with electrographically-confirmed seizures and 19% of 243 analyzed seizures demonstrated electroclinical dissociation. The two groups revealed very few differences with respect to perinatal factors, etiology, and outcome. The subsequent electroencephalographic background was more disturbed in the electroclinical dissociation group, but did not correlate with clinical outcome. Extremity movements occurred at a statistically significant higher rate during electroclinical seizures. Electroclinical dissociation seizures arise from foci not consistently reflected in surface electrodes.

Brain Diseases↗

Timing of brain insults in severe neonatal encephalopathies with isoelectric EEG.

In a neonatal intensive care unit of a large obstetric hospital, 20 neonates (7 preterm, 9 term, 4 postterm) with at least one isoelectric recording were treated over a 6-year period. Seventy-four EEGs were obtained in this cohort, including 36 isoelectric recordings. Seven infants in this group had evidence of a predominant antepartum component of a pathologic process based on placental, postmortem examination findings, or clinical history. Of the 16 placentas available for review, chronic lesions were observed in 13 of 16 specimens, including villitis, infarction, dysmaturity, and thrombosis. Seven of 9 patients with postmortem neuropathologic examinations had evidence of chronic lesions, principally neuronal necrosis, infarction, and microcalcifications. An additional 10 infants had evidence of an antepartum contribution to a pathologic process that continued into either the intrapartum or neonatal periods, based on maternal and/or neonatal medical factors. Clinical findings supportive of antepartum insults included intrauterine growth retardation, antepartum hemorrhage, abnormal antepartum fetal heart rate patterns, and maternal medical complications. Three patients had either intrapartum- or neonatal-onset of injury. Clinical signs of severe encephalopathy, however, were present in the immediate postnatal period in most patients (18 of 20; 90%). Assessment of clinical and pathologic information on neonates with isoelectric EEGs may estimate the timing of brain injury to the antepartum period, as opposed to, or in addition to, the labor and delivery periods. A neonate who suffered brain injury before parturition may be neurologically depressed after birth with absence of electrocerebral activity on EEG.

Brain Diseases↗

Treatment of functional impairment in severe somatoform pain disorder: a case example.

OBJECTIVE: To describe the assessment and treatment of severe functional impairment in a young female adolescent with somatoform pain disorder. METHODS: Treatment included an inpatient hospital admission using a rehabilitation approach and behavior modification program. Standardized assessment of functional impairment and health-related quality of life was performed at baseline and follow-up. Diagnostic evaluation and treatment costs were computed using insurance and hospital billing data. RESULTS: Pre-/postintervention measures of functional disability indicated significant improvement in physical and psychosocial functioning in everyday activities. Although costly, the inpatient admission decreased frequent health care use over the short term. CONCLUSIONS: Delay in diagnosis of somatoform disorders may seriously extend children's disability and require more intensive treatment. Functional disability is a critical measure of treatment outcome in children with severe somatoform disorder. Future research concerning interventions for children with a broad range of recurrent and chronic pain symptoms can be strengthened through a focus on reducing functional disability.

Adolescent↗

Ictal and interictal electrographic seizure durations in preterm and term neonates.

The effect of gestational age on neonatal ictal and interictal durations has not been investigated. Sixty-eight neonates with 644 electrographic seizures were identified retrospectively. Thirty-five full-term (FT) neonates were compared with 33 preterm (PT) neonates. Eighteen older preterm infants (OPT) [> 31 weeks estimated gestational age (EGA)] were also compared with 15 young preterm infants (YPT) of < or = 31 weeks EGA. Ictal/interictal durations were calculated for the total cohort with and without status epilepticus (SE). Statistical analyses were two-tailed t tests, chi-square calculations, and one-way analysis of variance (ANOVA) with Duncan's multiple-range test. Eleven of 35 (33%) FT had SE as compared with 3 of 33 (9%) PT (chi-square = 7.8, p < 0.05). The mean ictal duration was 14.2 min for FT infants as compared with 3.1 min for PT infants (p < 0.01); only borderline differences were noted after those with SE were excluded. Interictal durations were longer for OPT than YPT (p < 0.05). By ANOVA and Duncan's multiple-range tests, group differences included longer mean ictal durations for FT infants as compared with OPT infants (p = 0.06, ANOVA; p < 0.05, Duncan's), and longer mean interictal durations for FT infants versus OPT and OPT versus YPT (p = 0.02, ANOVA; p < 0.05, Duncan's). More developed neuronal networks result in longer ictal durations in FT than in PT neonates, including FT infants with SE.(ABSTRACT TRUNCATED AT 250 WORDS)

Analysis of Variance↗

Clinical examination findings in neonates with the absence of electrocerebral activity: an acute or chronic encephalopathic state?

Although the presence of an isoelectric electroencephalogram (EEG) in an older patient may reflect brain death caused by an acute brain injury, this electrographic abnormality may appear in more diverse clinical situations in the neonate with encephalopathy. During a 6-year period, 20 neonates were identified with a severe encephalopathy on neurologic examination who had at least one isoelectric EEG during their treatment in a neonatal intensive care unit. Seventy-four EEG recordings were obtained including 36 isoelectric EEG records. Partially preserved clinical brain function was present in 15 (75%) of 20 infants at the time an isoelectric EEG was obtained. The initial EEG was isoelectric in 16 of 20 infants. Although electrographic activity reemerged in nine of these infants, significant clinical improvement was seen in only two patients. Thirteen of 20 neonates also had electrographic or other evidence of clinical seizures. Of the five survivors (25%), three had severe neurologic sequelae. The remaining two had either transient or persistent neurologic deficits. An isoelectric EEG may be obtained in the neonate with partially preserved brain function and, therefore, may not be a reliable confirmatory test of neonatal brain death. In addition, serial EEGs not only can help assess the severity of a neonatal encephalopathy but also may correlate with chronic and acute neurologic insults.

Brain Death↗