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Stephen Ashwal

Publications and source records attributed to Stephen Ashwal.

31 records · Page 2Linked to original sources

Evaluation of the child with cerebral palsy.

Cerebral palsy (CP) is a common problem, occurring in about 2 to 2.5 per 1000 live births. The diagnosis of CP is based upon a history of abnormal motor development that is not progressive coupled with an examination (e.g. hypertonicity, increased reflexes, clonus) "placing" the lesion in the brain. In order to establish that a brain abnormality exists in children with CP that may, in turn, suggest an etiology and prognosis, neuroimaging is recommended with magnetic resonance imaging preferred to computed tomography. Metabolic and genetic studies should be obtained if there are atypical features in the history or on the examination. Detection of a brain malformation in a child with CP might suggest an underlying genetic or metabolic etiology. As cerebral infarction is high in children with hemiplegic CP, diagnostic testing for coagulation disorders should be considered. However, there is insufficient evidence at present to be precise as to what studies should be ordered. An electroencephalogram is not recommended unless there are features suggestive of epilepsy or a specific epileptic syndrome. As children with CP may have associated deficits of mental retardation, ophthalmologic and hearing impairments, speech and language disorders and oral-motor dysfunction, screening for these conditions should be part of the initial assessment.

Adolescent↗

Evaluation of coma and brain death.

Coma is a nonspecific sign of widespread central nervous system impairment resulting from various metabolic and structural etiologies. The rapid recognition of this neurologic emergency and results from the history, physical examination, and early investigative studies are key to the identification and treatment of its underlying cause. The prognosis for recovery depends greatly on the underlying etiology as well as on its optimal treatment, which seeks to preserve neurologic function and maximize the potential for recovery by reversing the primary cause of brain injury, if known, and preventing secondary brain injury from anoxia, ischemia, hypoglycemia, cerebral edema, seizures, infections, and electrolyte and temperature disturbances. Brain death must be diagnosed with similar care and precision, and families approached compassionately about the diagnosis and their decisions regarding organ donation.

Brain Death↗

Tethered cord syndrome in childhood: diagnostic features and relationship to congenital anomalies.

Tethered Cord Syndrome (TCS) is a stretch-induced functional disorder of the spinal cord that often develops and presents in childhood in association with spinal dysraphism. While the subtlety with which TCS can present makes it challenging to diagnose, awareness of the common neurological, musculoskeletal and urologic symptoms are of great value to the clinician, and can aid timely referral for neurosurgical evaluation. This article reviews these symptoms, as well as the clinical and radiological findings of the most common dysraphic conditions associated with TCS.

Cauda Equina↗

Medical aspects of the minimally conscious state in children.

The minimally conscious state is a condition of severely altered consciousness in which minimal but definite behavioral evidence of self or environmental awareness is demonstrated. This must be established on a reproducible or sustained basis by one or more of four types of behaviors including simple command-following, gestural or verbal 'yes/no' responses, intelligible verbalizations, or purposeful behaviors. The minimally conscious state can occur in children and usually is due to acquired brain injuries (traumatic and non-traumatic), central nervous system degenerative and neurometabolic disorders or congenital or developmental disorders. It is assumed that the lower limit of the minimally conscious state occurs when patients emerge from a vegetative state. What remains uncertain is how we can assess the upper limits, that is the degree of improvement that indicates that an individual is no longer minimally conscious. It also is unknown if, when and to what extent children can emerge from a minimally conscious state and whether their prognosis is better than children who are vegetative. It is assumed that the minimally conscious state may become 'permanent' 12 months after traumatic brain injury and 3 months after non-traumatic injury although there have been no studies that have examined this issue. Medical and rehabilitative treatment of children in a minimally conscious state should be provided to maintain comfort, reduce complications, and optimize functional recovery.

Adolescent↗

Predicting neuropsychologic outcome after traumatic brain injury in children.

The ability to predict long-term neurologic and neuropsychologic outcomes in 22 children, ages 1 week to 14 years at the time of traumatic brain injury, was investigated using proton magnetic resonance spectroscopy acquired post injury and compared with standardized neurologic, intellectual, and neuropsychologic testing done 1-7 years later. Clinical indicators of acute injury severity including age at injury, electroencephalography, spectroscopy metabolite ratio variables (N-acetyl aspartate/choline, choline/creatine) and lactate presence accurately classified children as functioning above or below the average range for most intellectual and neuropsychologic outcome measures. Combined clinical and spectroscopy variables accounted for approximately 50% of the variance in cognitive and neuropsychologic outcome confirming the validity of their predictive use. Of the injury severity indictors, presence of lactate is a particularly important prognostic marker of poor long-term intellectual and neuropsychologic functioning. Our findings indicate the potential for providing accurate estimates of long-term intellectual and neuropsychologic function after traumatic brain injury in infants and children using proton magnetic resonance spectroscopy in combination with clinical variables.

Adolescent↗

Post-transplant seizures in infants with hypoplastic left heart syndrome.

Seizures are common in infants undergoing cardiac transplant and are usually attributed to a non-specific "post-pump" phenomenon. In this study, we determined which variables were associated with the occurrence of post-transplant seizures in infants with hypoplastic left heart syndrome and the need for continued treatment with antiepileptic medication. Of 127 infants studied over an 11-year period, 27 (21%), ages 9 to 90 days, had post-transplant seizures. These patients were compared to 27 age-matched transplanted infants without seizures. We compared multiple variables before, during, and after transplant including growth parameters, time of diagnosis, cyclosporine levels, maternal variables, circulatory and bypass parameters, laboratory data, neuroimaging and electroencephalographic studies, neurologic examination findings, and peri-operative complications. Post-transplant seizures were associated with total cardiopulmonary bypass time and the presence of post-transplant complications. Deep hypothermic circulatory arrest time was inversely correlated with seizure severity. Pre-transplant electroencephalographic abnormalities and total bypass time were associated with seizures requiring continued use of antiepileptic therapy. Post-transplant electroencephalograms were not associated with the need for continued treatment. Identification of variables associated with the development of post-transplant seizures is essential for early intervention to reduce long-term morbidity and mortality. Future studies to reduce risk of post-transplant seizures are warranted.

Chi-Square Distribution↗

Serial proton magnetic resonance spectroscopy of the brain in children undergoing cardiac surgery.

We used proton magnetic resonance spectroscopy to study 11 children (age < 8 years) with congenital heart disease undergoing cardiopulmonary bypass to determine whether low (10 +/- 4; n = 6) vs high (20 +/- 4; n = 5) perfusate hematocrits during bypass resulted in changes in brain metabolites which correlate with neurologic injury. Long and short echo time single voxel magnetic resonance spectroscopy in occipital gray matter and neurologic assessment were performed preoperatively and 2 and 5 days postoperatively. We also determined whether prolonged periods at low flow rates during bypass affected spectroscopy variables. We found no significant differences in metabolite ratios between the low vs high hematocrit groups or the lower vs higher flow rate groups (repeated measures analysis of variance of observation ranks converted to normal scores). However, our study was limited by statistical power due to the small sample size, therefore no conclusions could be made. Additional studies involving a greater number of patients are necessary. In all 11 children, magnetic resonance spectroscopy detected a significant decrease in brain N-acetyl-aspartate, and increases in myoinositol and glutamate/glutamine after surgery (Quade test) demonstrating that magnetic resonance spectroscopy is sensitive in detecting subtle postoperative changes in brain metabolites.

Aspartic Acid↗

Hemorrhagic shearing lesions in children and adolescents with posttraumatic diffuse axonal injury: improved detection and initial results.

PURPOSE: To compare the effectiveness of a high-spatial-resolution susceptibility-weighted (SW) magnetic resonance (MR) imaging technique with that of a conventional gradient-recalled-echo (GRE) MR imaging technique for detection of hemorrhage in children and adolescents with diffuse axonal injury (DAI). MATERIALS AND METHODS: Seven young patients with a mean Glasgow Coma Scale score of 7 +/- 4 (SD) at admission were imaged a mean of 5 days +/- 3 after injury. High-spatial-resolution three-dimensional GRE imaging performed with postprocessing by using a normalized phase mask was compared with conventional GRE MR imaging. The total and mean values of lesion number and apparent hemorrhage volume load determined with both examinations were compared. Mean values were compared by using paired t test analysis. Differences were considered to be significant at P < or =.05. RESULTS: Hemorrhagic lesions were much more visible on SW MR images than on conventional GRE MR images. SW MR imaging depicted 1,038 hemorrhagic DAI lesions with an apparent total hemorrhage volume of 57,946 mm3. GRE MR imaging depicted 162 lesions with an apparent total hemorrhage volume of 28,893 mm3. SW MR imaging depicted a significantly higher mean number of lesions in all patients than did GRE MR imaging, according to results of visual (P =.004) and computer (P =.004) counting analyses. The mean hemorrhage volume load for all patients also was significantly greater (P =.014) by using SW MR imaging according to computer analysis. SW MR imaging appeared to depict much smaller hemorrhagic lesions than GRE MR imaging. The majority (59%) of individual hemorrhagic DAI lesions seen on SW MR images were small in area (<10 mm(2)), whereas the majority (43%) of lesions seen on GRE images were larger in area (10-20 mm(2)). CONCLUSION: SW MR imaging depicts significantly more small hemorrhagic lesions than does conventional GRE MR imaging and therefore has the potential to improve diagnosis of DAI.

Adolescent↗

Child neurology in the 20th century.

Although considered a relatively new subspecialty, child neurology traces its origins to the Hippocratic descriptions of seizures and other neurologic conditions in children. Its true beginnings can be traced to the 1600s and 1700s with classical descriptions of chorea, hydrocephalus, spina bifida, and polio. It was, however, the remarkable clinical and scientific advances in neurology and pediatrics at the end of the 19th century that helped create its scientific foundation. Like other pediatric disciplines, child neurology evolved into a distinct clinical and scientific specialty early in the 20th century. Remarkable advances in the neurosciences, particularly in the fields of genetics, molecular biology, metabolism, immunology and nutrition, have greatly advanced our understanding of how the brain develops and responds to environmental influences. Advances in neuroimaging, electroencephalography, electromyography, muscle histology, biochemistry, and neuropharmacology have considerably improved our ability to evaluate and treat children with neurological disorders. These advances have allowed new and expanding approaches, unique to children, in the fields of epilepsy, neurodegenerative and neurometabolic disorders, nervous system infections, demyelinating diseases and tumors, neonatal neurological conditions, and neuromuscular diseases. They have also led to a better understanding of the neurobiologic basis of common problems such as global developmental delay, cerebral palsy, and autism. As remarkable as the advances have been in the past century, the accelerating pace of our understanding of the fundamental mechanisms responsible for brain development will lead to even greater achievements in the clinical care of children with neurological disorders in the 21st century

Child↗

The minimally conscious state in children.

The minimally conscious state (MCS) is a condition of severely altered consciousness in which minimal but definite behavioral evidence of self- or environmental awareness is shown. Diagnostic criteria recently have been proposed for entry into and emergence from the MCS. We present clinical and neuroimaging data on 5 children diagnosed with MCS and discuss the limited information available concerning its epidemiology, etiology, pathology, and prognosis. Issues related to the evaluation and care of children suspected of having MCS are also reviewed as well as current ethical and legal controversies.

Adolescent↗

Proton magnetic resonance spectroscopy improves outcome prediction in perinatal CNS insults.

OBJECTIVE: Prediction of neurologic outcome is difficult in neonates with acute nervous system injury. Previous studies using proton magnetic resonance spectroscopy ((1)H-MRS) have been used to predict short-term neurologic outcome in neonates with a variety of neurologic insults. We were interested in determining the effectiveness of combining clinical evaluation and spectroscopy obtained at the time of injury in predicting neurologic outcome at 24 months. STUDY DESIGN: We studied 33 neonates with acute central nervous system injury, 5.8+/-3.7 days of injury, owing to hypoxic-ischemic encephalopathy. Neonates were assessed using clinical variables (initial arterial pH, initial blood glucose, Sarnat score, electroencephalography) and spectroscopy (NAA/Cho, NAA/Cre, Cho/Cre, and lactate). Neonates were divided into two outcome groups: good/moderate and poor. Differences between the groups were assessed using chi(2) and t-test analyses. We analyzed the best predictors of outcome using discriminant analysis and calculated sensitivity, specificity, positive, and negative predictive values for each variable independently and in combination. RESULTS: There were significant differences between the good/moderate and poor outcome for the Sarnat score, EEG, lactate, and NAA/Cho. Spectroscopy combined with clinical variables improved sensitivity, but not specificity for predicting outcome. The presence of lactate had the best individual predictive value. Combination of the clinical with the MRS variables had the highest predictive value. CONCLUSION: Proton magnetic resonance spectroscopy done early after injury improves the ability to predict neurologic outcome at 24 months of age.

Aspartic Acid↗

Recovery of consciousness and life expectancy of children in a vegetative state.

The vegetative state does occur in children and is most commonly due to acquired traumatic and non-traumatic injuries. However, neurometabolic and degenerative diseases, as well as certain developmental brain malformations such as anencephaly, can also cause this condition. There are limited data available in children concerning recovery of consciousness and function from the vegetative state as well as life expectancy. This review concentrates on these issues and is based primarily on the data published in the Multi-Society Task Force Report on PVS which was published in 1994 as well as other epidemiological studies. Children in a vegetative state do have a poor prognosis for recovery of consciousness and function and do have a shortened life expectancy. Further research is needed to better understand what variables might contribute to recovery and what therapies might be of benefit.

Adolescent↗