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Biomedical subjects

B Rosenblatt

Publications and source records attributed to B Rosenblatt.

At least 37 records · Page 2Linked to original sources
PubMed1998

Hypothalamic hamartomas and gelastic epilepsy: a spectroscopic study.

BACKGROUND: Patients with hypothalamic hamartomas present with epileptic attacks of laughter and later experience multiple seizure types and cognitive decline, suggestive of secondary generalized epilepsy. It has been suggested in the past that gelastic seizures originate in the temporal lobes rather than in the hamartoma, but temporal resections have been ineffective. Recent electrophysiologic evidence suggests that the epileptogenic discharges may originate in the hamartoma itself. METHODS: We used proton magnetic resonance spectroscopic imaging to quantify the amount of neuronal damage in the temporal lobes and hamartomas of patients with hypothalamic hamartomas and gelastic seizures. Five patients were studied and the relative intensity of N-acetylaspartate to creatine (NAA/Cr) was determined for both temporal lobes as well as for the hamartoma. These values were compared with signals from the temporal lobes and hypothalami of normal control subjects. RESULTS: NAA/Cr was not significantly different from normal control subjects for either temporal lobe, nor was there a significant asymmetry between the two temporal lobes for any of the patients. NAA resonance signals were present in the hamartomas, and the ratio of NAA to Cr was decreased in the hamartomas compared with the hypothalami of normal control subjects (t = 4.5, p = 0.005). CONCLUSIONS: We found no detectable neuronal damage in the temporal lobes of patients with hypothalamic hamartomas and gelastic epilepsy. This is further evidence that gelastic seizures do not originate in the temporal lobes of these patients.

Adolescent↗
PubMed1997

Automatic seizure detection in the newborn: methods and initial evaluation.

Seizures are most common in the newborn period, but at that age seizures can be very difficult to identify by clinical observation. Therefore the EEG plays an even greater role in newborns than in older children and adults. The electrographic features of seizures and EEG background in the newborn are, however, very different to those found in adults. We present a set of methods for the automatic detection of seizures in the newborn. The methods are aimed at detecting a wide range of patterns, including rhythmic paroxysmal discharges at a wide range of frequencies, as well as repetitive spike patterns, even when they are not very rhythmic. The methods were developed using EEGs obtained from 55 newborns, recorded at 3 hospitals that used differing monitoring protocols. A total of 281 h of recordings containing 679 seizures were analyzed. An initial evaluation indicated that 71% of the seizures and 78% of seizure clusters (group of seizures separated by less than 90 s) were detected, with a false detection rate of 1.7/h. The methods were developed so that they can be implemented to operate in real time.

Electroencephalography↗
PubMed1997

Evaluation of an automatic seizure detection method for the newborn EEG.

In another publication, we described a set of methods for automatic detection of EEG seizures in the newborn. We describe here the evaluation of these methods using a completely new set of data, which were not used in developing the method. This testing data set consisted of recording from 54 patients, lasting an average of 4.4 h. Recordings had 8-16 channels and were obtained, in approximately equal numbers, from 3 institutions in Canada, the USA and Australia. Recording conditions varied from short recordings fully attended by a technologist to overnight recordings largely unattended. The average seizure detection rate was 69% (77%, 53%, 84% in the 3 institutions). False detections occurred at the average rate of 2.3/h (4.1, 1.0, 2.7 in the 3 institutions), with fluctuations that reflected largely the technical quality and level of supervision of the recordings. The results are similar to those obtained in the commonly used method of epilepsy monitoring in adults and allow us to envisage clinical application.

Electroencephalography↗
PubMed1996

"Pseudo-BECRS": intracranial focal lesions suggestive of a primary partial epilepsy syndrome.

Benign epilepsy of childhood with rolandic spikes (BECRS) is an electroclinical entity that is the most common primary partial epilepsy syndrome of childhood. Typically presenting between the ages of 3 and 13 years, it is characterized by a well-recognized seizure pattern arising in a normal child with EEG findings restricted to rolandic/centrotemporal regions. Seizure control is usually easily achieved and prognosis is believed to be uniformly good. Some authors have suggested that individuals fitting the electroclinical parameters of this entity need not undergo neuroimaging due to the benign evolution of this disorder. Five patients presenting over a 13-year period with peribuccal seizures, normal neurologic examinations, and EEG data initially suggestive of BECRS found to have focal lesions on neuroimaging are summarized. Independent bilateral centrotemporal epileptiform abnormalities were seen in 3 patients. Imaging studies (CT, MRI, or both) documented a mass lesion in all in variable locations. Histologic examination documented a low-grade astrocytoma in 3 patients and a cavernous angioma in another. The fifth patient refused treatment or biopsy. Careful retrospective review of the clinical features of these patients reveals variable atypical features in each. Therefore, despite an electroclinical phenotype initially suggestive of the BECRS presentation, the presence of atypical clinical features raises the possibility of an underlying structural lesion and thus a negative neuroimaging study may in some patients be essential to the definitive accurate diagnosis of BECRS.

Adolescent↗
PubMed1996

Evoked potentials as predictors of outcome in neonatal intensive care unit survivors: review of the literature.

Neonatal intensive care unit survivors are at substantial risk for a range of neurodevelopmental sequelae, and therefore a variety of clinical diagnostic techniques have been evaluated as predictors of outcome. We summarize the prognostic value of evoked potentials in newborns at risk. A review of the literature reveals that brainstem conduction abnormalities in auditory brainstem evoked potentials are associated with neuromotor impairment; however, there are many false negative studies. Visual evoked potentials are highly accurate in predicting neurologic deficits in early childhood in asphyxiated term neonates. Sensitivity and specificity are consistently high for somatosensory evoked potentials in term newborns; however, correlations with outcome in premature infants is controversial. Several studies have compared neonatal findings on neuroimaging studies and evoked potentials, and concordant results between these two tests are highly predictive. However, neurologic sequelae often can most accurately be predicted by visual or somatosensory evoked potentials. Evoked potentials may therefore be a useful adjunct to the clinical investigation and prognostication of outcome in the high risk newborn.

Evoked Potentials, Auditory, Brain Stem↗
PubMed1996

Cauda equina histiocytosis X.

A young child was admitted with a cauda equina syndrome believed to be progressive, but unrelated to a congenital anomaly. Magnetic resonance imaging studies revealed the presence of an intradural tumor in the cauda equina. A yellow pasty tumor with nerve root infiltration was identified at surgery. Histologic features were pathognomonic for histiocytosis X. The patient's management and review of the literature are discussed.

Cauda Equina↗
PubMed1995

Prediction of outcome at school entry in neonatal intensive care unit survivors, with use of clinical and electrophysiologic techniques.

OBJECTIVE: To determine the predictive value of multimodality evoked potentials as well as the neonatal neurobehavioral assessment in neonatal intensive care unit survivors at school entry. STUDY DESIGN: In this prospective study, healthy (n = 24) and high-risk newborn infants (n = 78) were assessed in the newborn period with auditory brain-stem responses (ABRs) and somatosensory evoked potentials (SEPs), as well as the Einstein Neonatal Neurobehavioral Assessment Scale (ENNAS). Healthy and high-risk newborn infants were assessed in a blind fashion at 1, 3, and 5 years of age by a psychologist and a pediatric neurologist. Of those healthy (all 24) and high-risk newborn infants (72/78) with a neonatal ABR, SEP, or both, 62.5% were assessed at 5 years of age with the Wechsler Preschool and Primary Scale of intelligence, the Beery-Buktenica Test of Visual-Motor Integration, the Griffiths Locomotor Subscale, and neurologic examination. Chi-square analyses were carried out on neonatal and outcome measures, and sensitivity, specificity, and predictive values for each of the neonatal tests were ascertained. RESULTS: The ENNAS and the ABR had good negative predictive value for cognitive (85.7% to 93.3%), locomotor (83.3%), and visual-motor (91.4% to 100%) performance. The ABR had good specificity, whereas the ENNAS was more sensitive. The SEP was an excellent prognostic tool, with high sensitivity (100%) and specificity (80% to 81.3%) for motor as well as cognitive domains. All infants with normal SEPs had favorable outcomes, whereas those with absent potentials did poorly. The SEP abnormalities were associated with findings on neurologic examination in all cases. CONCLUSIONS: Normal neonatal evoked potentials and ENNAS are associated with favorable outcomes. The SEP most accurately predicts neurodevelopmental status at school entry.

Aging↗
PubMed1995

Lesionectomy of MRI detected lesions in children with epilepsy.

The results of complete excision of cerebral lesions detected by MRI in 18 children presenting with epilepsy were analyzed. There were 14 boys and 4 girls with a mean age of 9.2 years. The average age of onset of seizures was 6.8 years. The mean time from onset of seizures to surgery was 2.3 years. Often, CT scans suggested that the lesions were indolent. MRI was better in differentiating neoplastic from developmental lesions. Angiography was non-contributory in this series. Interictal EEGs showed epileptiform activity correlating with imaging studies in 54% of children. The lesion was completely surgically excised in all patients. This was confirmed by intra-operative ultrasound and postoperative imaging. Electrocorticography was performed prior to and after the resection, but residual spiking did not lead to further resection. The average postoperative follow-up was 5.7 years. Five patients had low grade astrocytomas, 4 had gangliogliomas, 1 a mixed astrocytoma-oligodendroglioma, 3 had cortical dysplasia, 2 infantile desmoplastic gangliogliomas, 2 hamartomata, and 1 cavernous angioma. Sixteen patients have been seizure-free since surgery. Only 2 have partial seizures. Thus, all patients benefited from the resection, with respect to seizure control. In those with temporal lobe lesions, improvement in IQ was seen postoperatively. Early consideration of surgery in patients with epilepsy and lesions demonstrated by MRI is suggested.

Adolescent↗
PubMed1995

The determination of sensory deficits in children with hemiplegic cerebral palsy.

Therapeutic intervention for children with cerebral palsy begins with accurate appraisal of abilities and disabilities. Currently, treatment focuses on the identified motor deficits, with any underlying sensory deficits often being overlooked. Sensory input is an essential component of motor function and motor control. Therefore, the objective of this study was to determine the presence and extent of sensory deficits in school-aged hemiplegic children using a formal clinical sensory battery, as well as somatosensory evoked potentials. A cross-sectional comparative study was performed comparing sensory function in hemiplegic children and healthy controls. Nine hemiplegic children and 41 healthy controls between 4 and 19 years of age were assessed. Hemiplegic children were included if they had a minimum level of receptive language function of 30 to 33 months and expressive language ability of 24 to 27 months, and no severe limitation of joint range of the hand. Significant bilateral sensory deficits (88.8%) were ascertained in hemiplegic children (P < .05), when compared to the performance of the healthy controls. Stereognosis and proprioception were the chief modalities affected bilaterally. The extent of sensory loss did not mirror the severity of motor deficit. Conversely, findings on somatosensory evoked potentials were closely related to motor function. Thus, a clinical sensory evaluation should be an integral part of the assessment of children with cerebral palsy. The likelihood of sensory impairment in one or more modalities on the hemiplegic or nonhemiplegic side is underappreciated and needs to be identified by rehabilitation specialists to maximize the functional potential of these children.

Adolescent↗
PubMed1994

Encephaloduroarteriosynangiosis (EDAS) for the treatment of childhood moyamoya disease.

Moyamoya disease is defined by the angiographic demonstration of stenosis or occlusion of the vessels of the anterior circulation at the base of the brain and the concomitant development of collateral blood supply. Untreated, the disease is often clinically progressive, resulting in significant neurologic sequelae. Encephaloduroarteriosynangiosis (EDAS), which involves the transposition of a segment of a scalp artery onto the surface of the brain, is a surgical treatment aimed at improving collateral blood flow. Six children underwent 8 EDAS procedures and were followed from 6 months to 9 years after surgery. No patient experienced further deterioration in neurologic status. Postoperative angiography demonstrated cerebral revascularization from the donor scalp artery on 3 of the 6 EDASs that were studied. The 2 patients who did not revascularize after EDAS demonstrated angiographic regression of their disease. The data suggest that EDAS is a safe procedure for the treatment of childhood moyamoya disease. Given the potential severity of the sequelae, early operative intervention is recommended in all children with this disease.

Anastomosis, Surgical↗
PubMed1994

Reliability of parental recall of developmental milestones.

The developmental history is a key element of the pediatric evaluation. Precise determination of a child's milestones depends on the parent's recollection of developmental events. As part of a prospective study examining neonatal predictors of outcome, healthy and high-risk newborns were assessed by a neurologist at 1, 3, and 5 years of age. The consistency of a parent's recall of two milestones (i.e., first steps and first meaningful word) was examined at 3 and 5 years. The answers provided by the parent(s) at the first visit (mean/S.D.: 12.67/1.19 months) provided the comparison standard. Twenty-five healthy and 50 high-risk infants had acquired 1 or both developmental skills by the first visit. Of 75, 63 were reexamined at 3 and/or 5 years by the same neurologist. For first steps, excellent correlations were obtained at 3 years (r = 0.74) and modest correlations were determined at 5 years (r = 0.41). The mean difference in age reported between 1 and 3 years was 0.36 +/- 1.81 months, and 0.33 +/- 2.46 months between 1 and 5 years. For first word, correlations were poor at 3 and 5 years (r = 0.27 and r = -0.11, respectively), with mean discrepancies in recall of 2.43 +/- 4.37 months (3-year visit) and 2.74 +/- 5.56 months (5-year visit). Twenty percent (n = 13) of parents reported discrepancies of 6 or more months (mean: 9.4; range: 6-25 months). Furthermore, this error in reporting of first word was later than the correct age in all patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗
PubMed1994

Predicting outcome in high-risk newborns with a neonatal neurobehavioral assessment.

OBJECTIVES: Effective medical management and rehabilitation efforts in neonates at risk depend on early identification of underlying brain injury. The aim of this study was to determine the prognostic value of the Einstein Neonatal Neurobehavioral Assessment Scale (ENNAS) in high-risk neonates, and to compare its predictive validity at two stages in development (i.e., 1 and 3 years of age). METHOD: Twenty-three healthy neonates (control group) and 51 high-risk neonates (high-risk group) were assessed at term and were followed longitudinally. At 1 and 3 years, subjects in both groups were evaluated in a blind fashion by a psychologist and a pediatric neurologist. RESULTS: Developmental delays became more apparent as high-risk newborns matured; the percentage of subjects with an abnormal Griffiths general quotient increased from 1 year (13.7%) to 3 years (39%). Analysis revealed that a normal neonatal performance on the ENNAS in high-risk subjects accurately predicted a favorable outcome at 1 year and 3 years of age. Although an abnormal ENNAS was not consistently associated with a poor outcome, the positive predictive value improved markedly from 1 year to 3 years of age. CONCLUSION: The findings indicate that a normal neonatal neurobehavioral assessment is reassuring, as most of these children are free of neurodevelopmental sequelae at 3 years of age. For many persons, the ENNAS may provide early evidence of a pattern of brain injury that is manifested only as the child is challenged by more complex skill acquisition such as language, memory, and perceptual-motor tasks. The ability of this assessment to predict behavioral and academic skills at school age remains to be determined. Early identification of developmental deficits enables occupational therapists to direct infants to appropriate early intervention programs, thus optimizing their functional potential.

Brain Damage, Chronic↗
PubMed1993

Inflammatory myopathy and Walker-Warburg syndrome: etiologic implications.

Walker-Warburg syndrome is a well delineated clinical entity with characteristic brain and eye anomalies. Recent diagnostic surveys have revealed that muscular dystrophy is an obligatory feature of this syndrome. We report a patient with an inflammatory myopathy that preceded dystrophic changes. While reports of parental consanguinity and multiple affected sibships strongly suggest an autosomal recessive genetic basis for this syndrome, previous pathological analyses of the CNS have suggested an inflammatory process. Our case supports both the notion of an aberrant inflammatory process that is likely under genetic control or etiologic heterogeneity (phenocopies) underlying this syndrome.

Brain↗
PubMed1992

Ring chromosome 20 and possible assignment of the structural gene encoding human carboxypeptidase-L to the distal segment of the long arm of chromosome 20.

We report on a 14-year-old boy with ring chromosome 20. Clinical manifestations included postnatal growth retardation, epilepsy, microcephaly, behaviour disorder, minor facial anomalies, small sella turcica, possible partial growth hormone deficiency, and mental retardation. A decreased activity of enzyme carboxypeptidase-L/protective protein (CP/PP) in cultured fibroblasts was demonstrated in our patient and a patient with a karyotype 46,XY,-14, + der(14)t(14;20)(14pter----14q32.3::20q13.1----20qter)m at. This suggests possible assignment of the CP/PP gene to the distal segment of 20q.

Adolescent↗
PubMed1992

3-Methylglutaconic aciduria: a marker for as yet unspecified disorders and the relevance of prenatal diagnosis in a 'new' type ('type 4').

The Mendelian disorder known as 3-methylgutaconic aciduria (McKusick 250950) gives evidence of allelic and locus heterogeneity. Type 1 has a mild clinical phenotype and confirmed 3-methylgutaconyl-CoA hydratase deficiency; inheritance is autosomal recessive. Other forms have major clinical manifestations and subdivide into X-linked (type 2), a form in Iraqi Jews with optic atrophy (so-called type 3); and untyped (putative autosomal recessive) forms without identified enzyme defects. In the latter, 3-methylglutaconic aciduria may simply be a marker for another metabolic disorder. We describe a male proband with 3-methylglutaconic aciduria designated here as 'type 4' (autosomal recessive, with severe psychomotor phenotype and cerebellar dysgenesis). He is the offspring of Italian consanguineous parents. Born with congenital malformations, he has been followed for 18 years, showing profound developmental delay and cerebellar dysgenesis. Measures of hydratase activity in cultured fibroblasts from the proband and 11 additional patients (two with type 1 disease, 9 with either type 2 or an unspecified form) revealed deficient enzyme activity in type 1 cases and normal activity in the proband and the other 11 cases. Two of the untyped cases probably have 3-methylglutaconic aciduria of the type described here. Prenatal diagnosis in the form described here may be feasible by analysis of amniotic fluid metabolites in pregnancies at risk if the mother does not entirely remove elevated concentrations. A female sibling of the proband had normal metabolite values in amniotic fluid. Postnatal follow-up confirmed absence of the disease. We give the normal values for amniotic fluid and results on these additional fetuses at risk (none affected).

Amniotic Fluid↗
PubMed1992

Functional interhemispheric asymmetries at birth as demonstrated by somatosensory evoked potentials.

Functional cerebral hemispheric asymmetries detectable at birth have been suggested by a number of neuroanatomic, neuroradiologic, and clinical neurophysiologic modalities. The aim of this study was to determine whether functional interhemispheric asymmetries can be identified using electrophysiologic measures. As part of a prospective study, somatosensory evoked potentials following median nerve stimulation were recorded in nine healthy full-term newborns on day 2 or 3 of life, and somatosensory evoked potentials were repeated at 2 and 6 months of age. These children were subsequently examined at 1 and 3 years of age by a pediatric neurologist and all had normal examinations. Handedness was determined at 3 years by questioning the parent and by clinical observation. Three of nine were left-handers. All three left-handers had clearly identifiable neonatal N19 parietal potentials over the right hemisphere. Following right median nerve stimulation, contralateral parietal potentials were absent on two of the three and questionable in the third. Asymmetries were not clearly present in right-handers although only one showed an increased maturation of the right hemisphere relative to the left. At 2 months of age, interhemispheric differences were no longer clearly evident. This data suggests that preferential hemispheric asymmetries are masked by 2 months of age. This differential rate of development demonstrated by neonatal somatosensory evoked potentials may be an early indicator of ultimate handedness.

Cerebral Cortex↗
PubMed1990

The effect of gestational age at birth on somatosensory-evoked potentials performed at term.

Multimodality-evoked potentials are widely used in newborns to assess the maturation and integrity of the sensory pathways. Reliable normative data are needed to maximize the utility of this technique as a diagnostic and research tool. Several electrophysiologic studies on the maturational changes of the auditory brain-stem response have demonstrated that latency measurements decrease as a function of increasing conceptional age. However, maturational studies of the somatosensory-evoked potential, particularly in low-risk premature infants, are limited. The existing evoked potential literature in healthy newborns proposes that maturation of the central nervous system occurs at a predictable rate, irrespective of a given gestational age at birth. Behavioral studies of premature infants suggest that neurologic development may be altered by early extrauterine exposure. The purpose of this study was to determine whether brain-stem auditory- or somatosensory-evoked potential conduction times were comparable in premature and full-term infants matched for conceptional age. The results of this study suggest that myelination is determined by conceptional age, independent of premature birth.

Brain Stem↗