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Biomedical subjects

R D Sheth

Publications and source records attributed to R D Sheth.

At least 19 recordsLinked to original sources

Electroencephalogram confirmatory rate in neonatal seizures.

The electroencephalogram (EEG) is confirmatory in 70% of children and adults with seizures, although gestation- and etiology-specific EEG confirmatory rates in neonates have not been well defined. All neonates treated for seizures and who underwent EEG were identified from 4,575 neonates admitted between 1985 and 1996 to a neonatal intensive care unit. The relationship between EEG findings (epileptiform discharges and background abnormalities) and gestation, mortality rate, and seizure etiology was examined using the Student t test. One hundred eighty-three neonates treated for seizures underwent a total of 352 EEGs: 144 of these neonates (79%) had an abnormal EEG (epileptiform discharges in 113 (60%) and nonepileptiform background abnormalities in 31). The EEG confirmatory rate increased with gestation (63% at 28 weeks vs 77% at term, P < 0.02). Etiology for seizures also influenced the EEG confirmatory rate: central nervous system (CNS) infection 95% (P < 0.05), hypoxic-ischemic encephalopathy 80% (P < 0.05), germinal matrix-intraventricular hemorrhage 65%, and CNS malformations 65%. The EEG confirmatory rate was predictive of neonatal mortality (19% vs 6%, P < 0.03). The EEG was directly confirmatory (epileptiform discharges) in 60% and supportive (nonepileptiform background abnormalities) in a further 17% of neonates with seizures. Gestation and etiology influence the EEG confirmatory rate in neonatal seizures.

Brain Diseases

Infantile botulism: pitfalls in electrodiagnosis.

Botulism in infants, unless recognized early, is associated with high mortality and morbidity. The diagnosis is suspected when infants present with sudden onset of weakness, respiratory failure, and constipation and is confirmed by demonstration of botulinum toxin in stool several weeks later. Electrodiagnosis allows quick and reliable confirmation of botulism. Low-amplitude compound muscle action potentials, tetanic or post-tetanic facilitation, and the absence of post-tetanic exhaustion support the diagnosis. Two infants with confirmed botulism did not exhibit the characteristic electrodiagnostic features, demonstrating the pitfalls in electrodiagnosis of infantile botulism.

Biopsy

Persistent occipital electrographic status epilepticus.

A 13-year-old girl of normal intellect had clinically silent occipital electrographic status epilepticus that persisted for more than 3 years. Neurologic examination and cranial magnetic resonance imaging were entirely normal. [18F]Fluorodeoxyglucose positron emission tomography demonstrated a hypermetabolic focus in the right occipital lobe. Electrographic status lasting years can be seen in epilepsia partialis continua. However, the absence of focal clinical seizures, nonprogressive course, and normal magnetic resonance imaging study seen in this patient are not features characteristic of epilepsia partialis continua.

Adolescent

Neonatal suck reflex pattern does not predict apnea.

Respiration and suck are gestational age-dependent reflexes modulated in the brain stem. To determine if the suck reflex pattern could be used to predict apnea, the relationship between the two was examined in 28 neonates. The suck reflex was quantified with respect to burst-pause rhythm, amplitude of negative suck pressure, and synchrony of the negative-positive pressure. Apneas were counted 5 days prior to and following measurement of the suck reflex pattern. Increasing gestational age correlated with a lower frequency of apnea (P < .01) and higher suck scores (P < .01). A mature suck reflex pattern, however, failed to predict the occurrence of apnea.

Apnea

Acute spinal cord infarction: vascular steal in arteriovenous malformation.

Central nervous system arteriovenous malformations typically present with chronic neurologic impairment. An 8-year-old boy presented with acute spinal cord infarction associated with a spinal arteriovenous malformation. Vascular steal phenomenon suggested by spinal angiography happens to underly the pathogenic mechanism.

Acute Disease

Electroencephalogram in developmental delay: specific electroclinical syndromes.

The electroencephalogram (EEG) plays an important role in the evaluation of a child with developmental delay. An EEG is often required to classify seizures in children with developmental delay. Equally important is the role of the EEG in the identification of specific electroclinical syndromes in children who may or may not manifest seizures. Specific electroclinical syndromes include the acquired epileptiform aphasia syndrome, Landau-Kleffner syndrome, and electrical status epilepticus during slow wave sleep. Other clinical situations where the EEG offers diagnostic and prognostic information, such as subacute sclerosing encephalitis, progressive myoclonus epilepsies, Rett syndrome, and Lennox Gastaut syndrome are also discussed.

Aphasia

Basilar artery occlusion and the dense artery sign in the newborn.

A child with basilar artery occlusion in the neonatal period is reported. The occlusion was documented by unenhanced computed tomography performed in the neonatal period demonstrating a "dense" artery at the tip of the basilar artery. The pattern of cerebral damage on MRI scan at 10 years of age confirmed the site of the vascular occlusion. The evidence suggests that embolization was the operating pathogenic mechanism of cerebral vascular occlusion. Neonatal arterial thrombosis involving the carotid circulation has been well documented and may be due to many pathological factors including direct trauma to the carotid artery and embolization from remote sites. Thrombosis of the vertebral artery in the neonate is only rarely reported and only in association with significant cervical trauma. A second child with a similar pattern of cerebral injury demonstrated on neuroimaging is described suggesting that this event may be more common than recognized. The clinical features of basilar artery occlusion as seen in the adult are not apparent in the neonate. Recognition of the neuroimaging characteristics seen in this condition may help to provide the clinician with a reasonable pathogenetic explanation for unexplained cerebral injury.

Adult

Trends in incidence and severity of intraventricular hemorrhage.

The incidence of germinal matrix-intraventricular hemorrhages declined from 50% in 1977 to 24% in 1985. Over the last decade intraventricular hemorrhage rates ranging from 8% to 56% were reported, leaving uncertainty as to the direction of recent intraventricular hemorrhage trends. Records of all 1950 neonates weighing 2250 g or less at birth (867 weighing 1500 g or less and 1083 weighing 1501-2250 g) at a university neonatal intensive care unit between 1986 and 1995 were studied. Intraventricular hemorrhage rate declined by 53%, from 11.5% in 1986 to 5.5% in 1995 (P < .01), and was consistent across all birthweight groups: 750 g or less from 36% to 24%, 751-1000 g from 38% to 22%, 1001-1250 g from 19% to 13%, 1251-1500 g from 12% to 2% and 1551-2250 g from 3% to 0.2% (P < .05). Proportionately, severe intraventricular hemorrhage (grades 3 +/- intraparenchymal hemorrhage) declined from 70% of all intraventricular hemorrhages in 1986 to 23% in 1995 (P < .005). Overall mortality declined by 65% between 1986 and 1995 (P < .001), whereas mortality associated with intraventricular hemorrhage declined by 30% (P = .34). Despite dramatic declines in intraventricular hemorrhage rates, 21% of infants weighing less than 1000 g and 12% of those weighing less than 1500 g at birth were affected in 1995.

Birth Weight

Frequency of neurologic disorders in the neonatal intensive care unit.

Neonatal intensive care unit survival rates have improved significantly over the past decade. This improvement primarily reflects declining mortality rates among preterm infants. Neurologic morbidity increases with prematurity and is the major predictor of long-term disability. Accordingly, concern has been expressed that the burden of neurologic dysfunction among contemporary neonatal intensive care unit survivors may be increasing. To define the trends of neurologic disorders in the contemporary neonatal intensive care unit, all 4164 admissions between 1986 and 1995 to a tertiary neonatal intensive care unit were examined. Neonatal intensive care unit admissions (413 +/- 49 per year), proportion of births at less than 37 weeks (70 +/- 3% per year), and referral patterns were stable between 1986 and 1995. Over the study period, 773 (18%) of 4164 neonatal intensive care unit infants had a total of 1062 neurologic disorders. The neonatal intensive care unit mortality rate declined from 12% in 1986 to 4.2% in 1995 (P < .01). Neurologic disorders declined, from 27% of infants born in 1986 to 12% in 1995 (P < .001): 356 had seizures (14% in 1986 to 4% in 1995; P < .001), 235 had hypoxic-ischemic encephalopathy (8% in 1986 to 4% in 1995, P < .01), and 167 had intraventricular hemorrhage (7% in 1986 to 1.4% in 1995, P < .005). Frequency of congenital or chromosomal aberration affecting the nervous system was relatively constant (4.5% per year). Despite a three-fold improvement in neonatal intensive care unit survival between 1986 and 1995, the frequency of perinatally acquired neurologic disorders declined by more than 50%.

Female

Rolandic epilepsy and cortical dysplasia: MRI correlation of epileptiform discharges.

An 8 year-old girl presented with simple facial motor seizures. Although the electroencephalogram (EEG) demonstrated left hemisphere centrotemporal spikes with features consistent with benign rolandic epilepsy, magnetic resonance imaging (MRI) indicated a left hemisphere focal cortical dysplasia. MRI-assisted EEG dipole analysis of the spikes suggested that the rolandic fissure rather than the focal cortical dysplasia was the origin of the epileptic spike discharge. This noninvasive method may be a useful adjunct in evaluation of some patients with epilepsy and focal superficial cerebral lesions.

Brain Mapping

Mental retardation plus macrocephaly in a 16-year-old boy.

The evaluation of mental retardation is a frequent and challenging problem in pediatric neurology. Often clues obtained on history or by physical examination will help to direct the evaluation in the appropriate channel. This 16-year-old boy had clinical features which suggested the appropriate diagnostic studies.

Adolescent

Neck pain and headache in an afebrile 15-year-old.

Nuchal rigidity and headache are important signs of bacterial meningitis, although, in the absence of fever other etiologies would be considered. An evaluation of a 15-year-old boy with the above features, focal neurological deficits, and two cerebral contrast enhancing ring lesions is discussed.

Adolescent

Recurrent coma.

Recurrent episodes of coma are usually associated with a metabolic disorder. A healthy 9-year-old boy of normal intellect and intact corpus callosum on neuroimaging had recurrent episodes of coma associated with profound spontaneous hypothermia. An evaluation, differential diagnosis and insights into the pathogenesis of this disorder are discussed.

Body Temperature Regulation