Peer review of expert medical-legal testimony: a proposal for child neurology.
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Brain Damage, Chronic↗
Biomedical subjects
W D Shields
Publications and source records attributed to W D Shields.
Early onset intractable seizures: nonverbal communication after hemispherectomy.
The nonverbal communication skills of 10 children (mean age = 44.2 months) who underwent hemispherectomy for early onset intractable seizures were tested before and after surgery. A within-group analysis suggests that the 10 seizure-free children used more nonverbal communication after a mean follow-up period of 11.2 months than before surgery. Young normal language age matches were available for the 4 older and higher functioning subjects in the sample. Before surgery, the surgical subjects used less requesting gestures than did the normal children. After surgery, these differences were no longer apparent. The patients also employed more gestures to focus an adult's attention on objects and events than language-age-matched normal children. The children who underwent left or right hemispherectomy used similar nonverbal communication behaviors. The study's findings suggest that children with early onset intractable seizures have impaired early social communication that improves to some extent after hemispherectomy.
Child, Preschool↗
Surgical treatment of the epilepsies: presurgical evaluation.
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Brain Diseases↗
Middle childhood onset of interictal psychosis.
The authors describe three children (mean age = 7.8 years) with complex partial epilepsy, left temporal lobe involvement, and interictal schizophrenia-like psychosis. As described in adults with complex partial epilepsy, these children met DSM-III criteria for schizophrenia, their affect was intact, and they demonstrated no negative signs of schizophrenia. Unlike adult epileptic patients, these children demonstrated psychotic symptomatology despite inadequate seizure control and after a short latency period. The possible role of early onset seizures, temporal lobe lesions, and kindling on the developing brain are discussed.
Child↗
Infantile spasms: I. PET identifies focal cortical dysgenesis in cryptogenic cases for surgical treatment.
Positron emission tomography (PET) of local cerebral glucose metabolism in 13 children with infantile spasms of undetermined cause (cryptogenic spasms) revealed unilateral hypometabolism involving the parieto-occipito-temporal region in 5 female infants. Cranial computed tomography showed normal findings in all infants. Magnetic resonance imaging (MRI) demonstrated a normal appearance in 4 of the 5 infants; in 1 infant, MRI revealed a subtle abnormality consisting of poor demarcation between occipital gray and white matter. Surface electroencephalography (EEG) in 4 showed hypsarrythmia at some time in the patients' courses, but at other times showed localized or lateralized abnormalities corresponding to areas of PET-detected hypometabolism. Because of poor seizure control, 4 infants underwent surgical removal of the cortical focus guided by intraoperative electrocorticography and were seizure free postoperatively. Neuropathological examination of resected tissue in each showed microscopic cortical dysplasia. Our findings indicate that in infants with cryptogenic spasms, PET can effectively identify those due to unsuspected focal cortical dysplasia, for which resective surgery offers improved prognosis.
Cerebral Cortex↗
Reversal of microcephaly and developmental delay after cure of hyperinsulinemic hypoglycemia.
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Blood Glucose↗
Central nervous system involvement in nephropathic cystinosis.
Nephropathic cystinosis, an autosomal recessive lysosomal storage disorder due to impaired cystine transport, causes damage to multiple organs that results in end-stage renal disease, hypothyroidism, and retinopathy, usually in childhood. Dialysis and renal transplantation now frequently enable patients with cystinosis to live into adulthood. Examinations at autopsy of a 28-year-old man who died of complications of this disease showed deposits of cystine crystals in multiple organs. There was severe cerebral involvement with multifocal cystic necrosis, dystrophic calcification, spongy change, and vacuolization that had produced profound neurologic deficits. Electron microscopy of the brain documented cytoplasmic deposition of cystine crystals in membrane bound vacuoles within the cytoplasm of pericytes and within parenchymal cells of the white matter. While affected patients who have received renal transplants may no longer die from renal failure, serious, potentially life-threatening, neurologic complications of this disorder may supervene.
Adult↗
Status epilepticus.
Status epilepticus generally is considered to be a medical emergency requiring immediate intervention to prevent permanent injury to the brain. Tonic-clonic status epilepticus is a very frightening event; the inexperienced parents invariably believe that their child is dying. Emergent management is imperative for convulsive tonic-clonic (grand mal) status epilepticus, but there are nonconvulsive types of status epilepticus in which the problem is more one of correct diagnosis than emergent management. This article reviews aspects of status epilepticus, particularly the classification and treatment of the various types of status.
Anesthesia, General↗
Normal neurodevelopment in four young children treated with bone marrow transplantation for acute leukemia or aplastic anemia.
Longitudinal neurodevelopmental studies of four consecutive young children treated by bone marrow transplantation for acute leukemia or aplastic anemia are presented. The children, the only four children less than 2 years of age who have received bone marrow transplants for these diseases at UCLA Medical Center, ranged in age from 36 weeks to 24 months at the time of transplantation. Conditioning involved high-dose cyclophosphamide treatment; three also had total body irradiation prior to bone marrow transplantation. Their respective outcomes after follow-up times of 28 months to 71 months posttransplantation are remarkable for normal somatic growth and normal development of intelligence, language, perception, and motor coordination. These findings indicate that future therapeutic studies of infants and young children with acute leukemia or aplastic anemia using total body irradiation, cyclophosphamide, and bone marrow transplantation are not contraindicated by risks of debilitating neurodevelopmental sequelae.
Anemia, Aplastic↗
Relationship of pertussis immunization to the onset of neurologic disorders: a retrospective epidemiologic study.
A retrospective epidemiologic study examining the relationship of the time of onset of neurologic disorders with the time of pertussis immunization in two cohorts of children who received pertussis immunization at different ages is reported. Before April 1970, children in Denmark were vaccinated with diphtheria and tetanus toxoids with pertussis vaccine at 5, 6, 7, and 15 months of age. Since 1970, children were given monovalent pertussis vaccine at 5 and 9 weeks and at 10 months of age. A total of 554 cases of epilepsy with onset between 28 days and 24 months of age were reviewed, 286 from the 1967-1968 period and 268 from the 1972-1973 period. There was no relationship between the age of onset of epilepsy and the scheduled age of administration of pertussis vaccine. A total of 2199 children with febrile seizures were reviewed, 830 from the 1967-1968 period and 1369 from the 1972-1973 period. There was a statistical association between first febrile seizures and the scheduled age of administration of pertussis vaccine (p = 0.004). No relationship between pertussis immunization and the occurrence of central nervous system infections was noted.
Age Factors↗
Surgical treatment of intractable neonatal-onset seizures: the role of positron emission tomography.
We have performed positron emission tomography (PET) with 2-deoxy-2[18F]fluoro-D-glucose (FDG) in eight infants and children (aged 18 days to 5 years) with medically refractory epilepsy of neonatal onset. It was hypothesized that in at least some of these infants a surgical approach (focal resection, cerebral hemispherectomy) might be of benefit in achieving seizure control, and that PET might assist in surgical selection. In three of the eight subjects, interictal PET revealed unilateral diffuse hypometabolism; following cerebral hemispherectomy in these three patients, all seizures ceased and there were no adverse effects. In one child, ictal PET showed hypermetabolism in the left frontal cortex, left striatum, and right cerebellum; a partial left cerebral hemispherectomy guided by intraoperative electrocorticography was performed, following which all seizures ceased. One infant had relative hypermetabolism in the right temporal and occipital lobes, right thalamus, and left frontal lobe on ictal PET, and EEG telemetry revealed a right occipitotemporal epileptic focus; this infant died from anesthetic complications following right occipitotemporal cortical resection. Of the three unoperated patients, one is a potential candidate for right frontal lobectomy, but the other two were not considered to be surgical candidates due to bilateral epileptogenicity. Neuropathologic correlation in our series revealed that PET is a sensitive test capable of detecting cytoarchitectural disturbances whereas CT and MRI failed in this regard. In addition, PET provides a very unique and important assessment of the functional integrity of brain regions outside the area of potential resection.
Brain↗
Infants and children with convulsions and hypotonic-hyporesponsive episodes following diphtheria-tetanus-pertussis immunization: follow-up evaluation.
In a prior prospective study, we evaluated the nature and rates of adverse reactions occurring within 48 hours following 15,752 diphtheria-tetanus-pertussis (DTP) immunizations. Nine children had convulsions, and nine had hypotonic-hyporesponsive episodes. After an interval of 6 to 7 years, we were successful in contacting the families of 16 of these children to determine whether any had evidence of neurologic impairment too subtle to have been detected at the time of initial evaluation. All 16 were considered normal by their parents and were doing well in school. A complete neurologic and psychometric evaluation was performed on 13 of these children. No child had significant neurologic deficit, although four had minor neurologic abnormalities. Psychometric testing revealed normal performance IQ scores (104.3 +/- 15.8) but low verbal IQ scores (91.8 +/- 18.4); however, these lower verbal IQ scores can be explained by the proportion of Hispanic and bilingual children in this sample. Therefore, there is no evidence that any of these 16 children have any serious neurologic damage as a result of a convulsion or a hypotonic-hyporesponsive episode temporally associated with a prior diphtheria-tetanus-pertussis immunization.
Child, Preschool↗
Relationship of pertussis immunization to the onset of epilepsy, febrile convulsions and central nervous system infections: a retrospective epidemiologic study.
A change in the pertussis immunization schedule in Denmark allowed a retrospective study examining the relationship of the time of onset of selected neurologic disorders with the time of pertussis immunization in two core cohorts of children. Records of 2,199 children with febrile seizures were reviewed and a significant association between first febrile seizures and the scheduled age of pertussis immunization was noted (p = 0.004). The occurrence of epilepsy and CNS infections did not appear to be related to the time of pertussis immunization.
Central Nervous System Diseases↗
Rett syndrome. A commonly overlooked progressive encephalopathy in girls.
We report 15 cases of Rett syndrome, a slowly progressive disorder that occurs only in girls and is characterized by early deterioration of higher brain function with dementia and autistic behavior, loss of purposeful use of the hands, and deceleration of head growth. Epilepsy, with minor motor seizures being the predominant type, has its onset between 2 and 4 years of age in the majority of cases. Additional features include an extrapyramidal disorder with dystonia and choreoathetosis, and lactic acidemia. A precise biochemical marker of this disorder has not been identified.
Adolescent↗
Amantadine in the treatment of refractory epilepsy in childhood: an open trial in 10 patients.
Amantadine HCl was given to 10 children with medically refractory seizures; other anticonvulsant medications were continued unchanged through the 12- to 16-week trial. Several patients noted improvement in control of myoclonic or atypical absence seizures. Tonic seizures were controlled in one patient, but worsened in another. Tonic-clonic and atonic seizures remained unchanged or worsened. Amantadine may be useful as an adjunctive anticonvulsant in some children with refractory atypical absence or myoclonic seizures.
Adolescent↗
Correlation of ominous fetal heart rate patterns and brain-specific creatine kinase.
Creatine kinase isoenzyme analysis was performed on the cord blood of 125 infants who had undergone at least 30 minutes of intrapartum electronic fetal monitoring. The tracings were scored blindly according to severity of abnormal patterns, and the infants were grouped into ominous, intermediate, and normal scores. No differences were seen in creatine kinase MM or creatine kinase MB levels among the three groups. However, infants with ominous fetal heart rate patterns had higher creatine kinase BB levels and poorer outcome than infants with normal patterns.
Birth Weight↗
Recurrent seizures after diphtheria, tetanus, and pertussis immunization. Cause and effect v temporal association.
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Age Factors↗
Intracranial ultrasound diagnosis of neonatal periventricular leukomalacia.
Periventricular leukomalacia (PVL) is characterized by focal areas of white matter necrosis adjacent to the external angles of the lateral ventricles. The diagnosis of PVL has hitherto been limited to autopsy findings. The authors discuss pertinent literature and report in vivo diagnosis of nonhemorrhagic PVL by real-time intracranial ultrasonography. They recommend further study of ultrasound as a diagnostic method for evaluating infants at risk for PVL.
Brain↗