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Biomedical subjects

B Rosenblatt

Publications and source records attributed to B Rosenblatt.

54 records · Page 3Linked to original sources
PubMed1990

Congenital inflammatory myopathy.

We present 3 patients with congenital inflammatory myopathy and summarize the literature. CNS involvement (microcephaly/intellectual delay) may or may not be present. Serum creatine kinase activity is elevated, the EMG is myopathic, and the muscle biopsy reveals inflammatory infiltrates, muscle fiber damage, and class I major histocompatibility complex products in muscle sarcolemma. Possible etiologies include intrauterine viral infection or an autoimmune process. Treatment with steroids may result in some motor improvement but has no effect on the CNS involvement. Despite a common time of presentation, these patients have a heterogeneous clinical profile, often suggesting a congenital muscular dystrophy syndrome.

Female↗
PubMed1988

Prognostic significance of the auditory brainstem evoked response in high-risk neonates.

The prognostic significance of the auditory brainstem evoked response (ABR) was examined in this prospective study of neonates at risk for neurodevelopmental sequelae. ABR testing was performed in the neonatal period (37 to 45 weeks conceptional age) and at two and/or six months corrected age. 34 high-risk newborns and 14 controls were followed to one year of age, when they received neurological and developmental assessments. Increased I to III and I to V interwave latencies predicted gross motor delay at one year, with a positive predictive power of 83 per cent and a specificity of 94.7 per cent. Increased brainstem conduction, dispersal of waves III to V and V/I amplitude ratio abnormalities predicted abnormal neurological findings at one year, with positive predictive values of 100, 100 and 80 per cent, respectively. A standard clinical examination of the newborn, performed on the at-risk and control infants at 40 weeks conceptional age, was not found to be strongly predictive of neurodevelopmental deficits at one year.

Brain Stem↗
PubMed1987

Subacute necrotizing encephalomyelopathy (Leigh disease): CT study.

Leigh disease, or subacute necrotizing encephalomyelopathy (SNE), is a familial, degenerative disorder characterized by lesions of the gray and white matter in the brain and spinal cord. Low attenuation in the putamina on computed tomography (CT) scans is considered to be characteristic of the disease. The authors used CT to study five patients, in three of whom the disease was confirmed histologically. In one of the patients with documented SNE, there was extensive gray matter lucency with normal basal ganglia. CT scans obtained in a second patient showed diffuse, diseased white matter with focal cortical extension and bilateral caudate involvement. In the other three cases, CT scans revealed the usual changes that occur in the basal ganglia. The appearance of SNE on CT scans thus reflects the variable and widespread distribution of the disease. The absence of radiologically detectable abnormalities in the basal ganglia should not deter one from the diagnosis of Leigh disease given in the appropriate clinical context.

Basal Ganglia↗
PubMed1986

Giant axonal neuropathy: central abnormalities demonstrated by evoked potentials.

Previous studies of giant axonal neuropathy have reported clinical and pathological findings that indicate involvement of the central nervous system. We studied 3 boys with giant axonal neuropathy, who were 14 to 16 years of age, using auditory, visual, and somatosensory evoked potentials. Absence of waveforms and prolongation of peak and interwave latencies were found. Abnormalities were noted in all modalities. The auditory brainstem evoked response in particular indicated a significant increase in brainstem conduction time. These studies add clinical neurophysiological confirmation of the central nervous system involvement in this disorder and may also provide a means of quantitative evaluation of its progression.

Adolescent↗
PubMed1985

A distinctive facial appearance in neurofibromatosis von Recklinghausen.

Three children with neurofibromatosis from two unrelated families have unusual facial appearance due to telecanthus, antimongoloid slant of the palpebral fissures, broad nose, and tapering chin. The two boys have large hands and feet, and one is very tall. There were some facial and dermatoglyphic similarities to individuals with the Noonan syndrome, but the manifestations in the children reported here are sufficiently different to suggest the presence of a different type of neurocristopathy in these individuals.

Adolescent↗
PubMed1982

Auditory brainstem response abnormalities in autistic children.

In an attempt to clarify issues of brainstem dysfunction and hearing thresholds in autistic children, we studied the Auditory Brainstem Responses (ABRs) in 32 children who clearly fit within the criteria of autism established by the National Society for Autistic Children (1977). ABRs were recorded between Cz and ipsilateral ear in response to click stimuli. Interwave latencies and auditory threshold in each ear were determined. Of the 32 children, 11 had moderate hearing loss (8 bilaterally) and 3 had severe to profound hearing loss, all bilaterally. 8 of the 14 with hearing loss also had associated features (e.g., perinatal encephalopathy). The I-III and I-V interwave latencies were significantly longer in the autistic children compared to normal control children; the increased conduction times were found mainly in the early portion of the auditory brainstem pathway. These data confirm some earlier reports of ABR abnormalities in autistic children and are concordant with some theories of the etiological basis of autism. The high incidence of hearing loss in these children is significant and routine ABR testing is recommended.

Adolescent↗
PubMedDate not supplied

A longitudinal study of short latency somatosensory evoked responses in healthy newborns and infants.

Maturational changes in short latency somatosensory evoked responses (SERs) were studied in 18 healthy full-term newborns in the first week of life and consequently repeated at 2-3 and 6-7 months of age. Both median nerves were electrically stimulated individually and evoked responses were recorded at 3 levels: Erb's point (EP), second cervical vertebra (CII), and contralateral parietal scalp (C'c). In the neonatal period, results of 32 stimulated nerves were obtained in all cases at the EP and CII levels. At the parietal level, potentials were present in 85% of cases, absent in 9% and questionable in 6%. Parietal potentials were occasionally noted on one side only. Repeat examinations at 2-3 and 6-7 months of age demonstrated significant maturational changes in the SERs. These changes were most prominent in the neonatal period and 2 months of age. They included decreased interpeak latencies, increased amplitude and markedly diminished dispersion of parietal potentials. Minimal changes in wave form configuration and latency were noted at the EP and CII level. These findings most likely reflect myelination and increased synaptic efficiency predominantly in the central sensory pathway. The purpose of this investigation was to delineate a reliable technique for SERs in newborns and infants that could be applied both to research and clinical settings. Normative data were established in newborns and infants as this will help us in accurately differentiating a normal from an abnormal group of neonates and infants.

Electroencephalography↗
PubMedDate not supplied

Somatosensory evoked response abnormalities in high-risk newborns.

In a previous study from our laboratory, the prognostic significance of the auditory brainstem evoked response was assessed in high-risk neonates. An abnormal auditory brainstem evoked response predicted neurologic deficits at age 1 year; however, a normal result did not predict a normal outcome. In order to evaluate the prognostic utility of examining other sensory pathways, somatosensory evoked responses were elicited following median nerve stimulation. Testing was performed at 37-44 weeks conceptional age (defined as gestational age plus chronologic age) and at 2 and 6 months conceptional ages. Those patients studied included 34 high-risk neonates and 18 healthy, term infants as controls. Ten of the 34 patients had abnormal somatosensory evoked responses. Abnormalities included increased absolute (N19, P22) and interwave (N13-N19, N19-P22) latencies and flat potentials, alone or in combination. Three children with flat potentials demonstrated a persistence of this abnormality on subsequent examination and they later presented clinically with spastic quadriparesis. Four infants with increased latencies manifested normal responses on subsequent examination. Recently, these 4 patients exhibited tone abnormalities and mild developmental deficits; developmental outcome, however, will be assessed in a blind study at 1 year of age as part of this ongoing prospective study. Preliminary results suggest that somatosensory evoked responses may be valuable as an electrophysiologic predictor of outcome.

Brain Damage, Chronic↗
PubMedDate not supplied

Prognostic significance of multimodality evoked response testing in high-risk newborns.

Exposure to hypoxic-ischemic events in fetal or neonatal life may lead to permanent brain damage and subsequent neurodevelopmental deficits. Clinical and diagnostic tools have been somewhat helpful in identifying an at-risk group, particularly those patients sustaining significant neurologic sequelae. In this prospective study, the prognostic significance of multimodality evoked responses in high-risk newborns was examined. A group of 44 high-risk newborns, as well as 14 healthy newborns, were tested during the newborn period with auditory brainstem responses and somatosensory evoked responses; these tests were repeated at 2 and 6 months corrected age. A neonatal neurologic examination, the Einstein Neonatal Neurobehavioral Assessment Scale, was also conducted. At 1 year corrected age, both groups were assessed in a blind fashion by a pediatric neurologist and a psychologist to determine neurodevelopmental outcome. Results indicated that somatosensory evoked response abnormalities in particular predict an abnormal neurologic status at 1 year of age. Abnormalities that persisted or worsened correlated with severe neurologic impairment, whereas an abnormal somatosensory evoked response that improved or normalized in infancy was associated with mild to moderate neurologic sequelae. Increased brainstem conduction in the auditory brainstem responses was also associated with neurologic sequelae. Normal findings from auditory brainstem responses and somatosensory evoked responses predicted normal developmental scores in all areas, as well as a normal neurologic outcome at 1 year with negative predictive powers ranging from 85-100%. Evoked response testing appears to be an important adjunct to the neurologic investigation of high-risk newborns.

Asphyxia Neonatorum↗
PubMedDate not supplied

Transient oculosympathetic paresis (group II Raeder paratrigeminal neuralgia) of childhood: migraine variant.

Although a recognized migrainous phenomenon in adults, transient oculosympathetic paresis in childhood has been rarely observed. Six pediatric patients are reported with transient oculosympathetic paresis occurring within the context of characteristic vascular headaches. The clinical profiles of the patients suggest transient dysfunction of third-order ocular sympathetic pathways and represent most likely a benign, self-limited variant of pediatric migrainous neuralgia.

Adolescent↗
PubMedDate not supplied

Influence of gestational age, birth weight, and asphyxia on neonatal neurobehavioral performance.

The objectives of this study were to determine the influence of birth weight, gestational age, and asphyxia on neurobehavioral performance at 40 weeks conceptional age, and to demonstrate that this performance may differ in preterm high-risk infants when using low-risk preterm rather than healthy term infant norms. A cross-sectional comparative design was used to compare neurologic behavior in healthy and high-risk newborns at 40 weeks conceptional age using the Einstein Neonatal Neurobehavioral Assessment Scale. Seventy-four high-risk newborns were selected from a consecutive sample of asphyxiated, very-low-birth-weight, and small-for-gestational-age neonates. Controls did not fulfill any of the high-risk criteria and had no pre- or perinatal complications. There were 37 healthy term and 17 low-risk preterm controls. Statistically significant differences occurred between high-risk newborns and term controls for the total score (P < .001) as well as for most individual items (P < .05). Analysis of high-risk subgroups revealed that small-for-gestational-age and term asphyxiated newborns had the most abnormal responses. When comparing test performance between preterm high-risk and term controls, the majority of test items achieved significance (P < .05); however, when compared to low-risk preterm controls, fewer items were abnormal, and the probability level on abnormal items was less significant than expected. This disparity in interpretation of neurobehavioral status in preterm high-risk neonates using a preterm versus term reference sample warrants further investigation. Appropriate normative standards for preterm infants require definition because it may increase the utility of the neonatal neurologic examination and its prognostic value.

Asphyxia Neonatorum↗
PubMedDate not supplied

Benign occipital epilepsy: a family study.

Benign occipital epilepsy is a newly recognized form of partial epilepsy in children. It is characterized by motor seizures preceded in some cases by visual symptoms and a relatively benign course. We present three siblings with this condition and a fourth with the EEG abnormalities. A family study including 25 relatives demonstrated a typical EEG abnormality in 26% of relatives. These EEG changes were more evident in younger members. These findings suggest an autosomal dominant pattern for the EEG abnormalities with age-dependent expression and variable penetrance of the seizure disorder.

Adult↗
PubMedDate not supplied

Agreement between the neonatal neurological examination and a standardized assessment of neurobehavioural performance in a group of high-risk newborns.

The degree of agreement between the neonatal neurological examination and a standardized neurobehavioural assessment was investigated in a group of 32 newborns with congenital heart defects. A paediatric neurologist performed a neurological examination, and an occupational therapist administered the Einstein Neonatal Neurobehavioural Assessment Scale on all subjects. Both examiners independently evaluated each subject, and were blinded to the diagnosis, to perinatal status and to each other's clinical findings. Statistical analysis demonstrated a significant association between the overall impression between both examiners (p < .0001), with a crude agreement of 96.9%. Sixteen neonates were determined to be normal and 15 abnormal by both examiners, with disagreement in only one subject. Although two distinct approaches were employed in the neurological assessment of high-risk newborns, both assessments evaluate the maturity and integrity of the immature central nervous system. The results demonstrate a strong agreement between these two approaches, suggesting that the neonatal neurological examination is consistent and valid.

Acoustic Stimulation↗