Jerrold M. Milstein

Near-death experiences: A neurophysiologic explanatory model

Prior hypotheses as to the etiology of near-death experiences (NDEs) have been limited to psychiatric explanations or brief discussions of endorphins as causative agents. We present a neurophysiological explanation for NDEs based on their similarities with lysergic acid-, ketamine-, and hypercapnia-induced hallucinations. We believe the core NDE is genetically imprinted and triggered by serotonergic mechanisms.

Cervical spine MRI in abused infants.

OBJECTIVEnTo determine clinical utility of screening with cervical spine MRI to detect unsuspected cord injury in children with head injury from child abuse.nnnDESIGNnProspectively collected case series. Setting-Tertiary care childrens hospital and county medical examiners office.nnnPATIENTSnTwelve children with intracranial injury secondary to child abuse. None was clinically suspected to have cord injury. Includes all eligible children whose attending felt: (a) needed follow-up cranial imaging: (b) could be safely imaged; and (c) whose caretakers consented between November, 1991 and September, 1994.nnnINTERVENTIONSnMRI scans of the cervical spine were obtained either more than 3 days after clinical presentation or postmortem.nnnMAIN OUTCOME MEASURESnClinical observations by neurologist, child protection team pediatrician and medicinal examiners by prospective protocol. MRI scans evaluated by prospective radiology protocol with emphasis on cervical cord injury.nnnRESULTSnFour of the five autopsied children had small subdural or subarachnoid hemorrhages at the level of the cervical spine; MRI scan did not identify them. MRI did not identify cord injury in any child studied.nnnCONCLUSIONnRoutine cervical spine MRI scans are probably not warranted in children with head injury secondary to child abuse without clinical symptoms of cervical cord injury.

Fatal Infantile Muscle Phosphorylase Deficiency

A premature female infant born of a consanguineous union exhibited joint contractures and signs and symptoms of perinatal asphyxia. A muscle biopsy examined by light microscopic, histochemical, and electron microscopic techniques exhibited changes of muscle phosphorylase deficiency and glycogenosis, identical to those of McArdles disease. Postmortem ultrastructural examination of liver and heart did not reveal lysosomal storage. This case and one previously reported example of fatal infantile phosphorylase deficiency suggest that the clinical spectrum of McArdles disease may be broader than previously recognized. (J Child Neurol 1989;4:186-188).

The prognostic significance of postoperative residual contrast enhancement on CT scan in pediatric patients with medulloblastoma

The clinical and therapeutic features of 20 patients with medulloblastoma treated at Childrens Hospital and Medical Center, Seattle, from 1980 to 1987, were retrospectively analyzed with regard to prognosis. The overall actuarial 5-year survival rate was 63%, with 57% of patients free from recurrence at 5 years. Residual contrast enhancement on CT scans taken immediately postoperatively was associated with a significantly decreased 5-year recurrence-free survival rate; the 5-year recurrence-free survival rate was 100% for those patients without post-operative residual enhancement compared to 41% for those patients with residual enhancement. A high risk group of patients with residual contrast enhancement persisting one year following diagnosis was identified. No patient in this group survived without disease progression. Other factors, including sex, age at diagnosis, evidence of tumor dissemination, or extent of surgical resection as reported by the neurosurgeon, did not significantly influence prognosis.

Favorable prognosis for brainstem gliomas in neurofibromatosis

Over a two year interval, we have treated five children with neurofibromatosis who developed brainstem gliomas. They had an insidious onset of symptoms and other indicators of low grade tumor despite several features that have been ascribed to rapidly progressive brainstem gliomas. Following standard radiation therapy a more protracted improvement was observed with the persistence of preradiation findings for several months. This experience confirms the better prognosis in brainstem glioma associated with neurofibromatosis.

Adrenocorticotropic Hormone Controls Infantile Spasms Independently of Cortisol Stimulation

Summary: Infantile spasms constitute a severe seizure disorder unresponsive to standard anticonvulsants. Both prednisone and adrenocorticotropic hormone (ACTH) have produced remission of seizures in some patients. The mechanisms of action of these hormones are not known. Eight infants with infantile spasms were treated with prednisone for 2 weeks. This controlled the seizures in two patients. In the remaining six patients, prednisone was continued and ACTH was added. This treatment produced cessation of spasms in four patients. Serum prednisone and cortisol were measured at a number of points during treatment. In infants receiving prednisone and then prednisone plus ACTH, serum cortisol was suppressed to about one‐quarter of baseline levels with the initiation of prednisone, and remained suppressed during ACTH administration. We conclude that ACTH can exert its effect on infantile spasms in the setting of adrenal suppression, and can act without stimulating endogenous cortisol production. A CNS site of action is suggested and should be sought.

Stroke and Mixed Connective Tissue Disease

We describe the clinical presentation and course of two girls with cerebrovascular disease and mixed connective tissue disease. One developed rapid onset hemiparesis and aphasia secondary to left internal carotid artery occlusion. She experienced a complete recovery after treatment with prednisone and cyclophosphamide. The other patient was diagnosed as having mixed connective tissue disease but had acute neurologic deterioration. She died due to an intracerebral hemorrhage. Autopsy demonstrated small-vessel fibrinoid necrosis. Although cerebrovascular disease secondary to central nervous system vasculitis is a manifestation of systemic lupus erythematosus, this is the first description of cerebrovascular disease as a primary sign in mixed connective tissue disease. These cases demonstrate the range of cerebrovascular disease observed in children with mixed connective tissue disease. (J Child Neurol 1993;8:256-259).

Childhood polymyositis as a paraneoplastic phenomenon

Childhood polymyositis and dermatomyositis are rare conditions that are not generally associated with malignancy even though an association between adult-onset and malignancy has been reported. A child is presented with typical polymyositis in whom an immunoblastic sarcoma subsequently became manifest; the literature also is reviewed concerning the association between malignancy and these childhood conditions.

Hypertrophic cardiomyopathy is a component of subacute necrotizing encephalomyelopathy

Twelve patients, ranging from the neonatal period through adolescence, with subacute necrotizing encephalomyelopathy (Leigh disease) were studied. Autopsies of these patients demonstrated an associated hypertrophic cardiomyopathy in seven; of these, four had asymmetric septal hypertrophy. In two patients, the cardiac lesion was observed by premortem echocardiograms. The common occurrence of a cardiac lesion emphasizes the probable systemic nature of SNEM and may serve to segregate these patients into two groups. Because of the involvement of the two systems, we suggest that SNEM may have some relation to a variety of other cardioneurologic syndromes.

Differentiation of a primitive neuroectodermal tumor into a benign ganglioglioma

We describe a case of cerebellar neuroblastoma with histologic documentation of maturation into a ganglioglioma sixteen months later. Only chemotherapy was administered following the initial surgery and the child is well and disease-free three years following her final surgical procedure. The outcome of this patient supports previous hypotheses that the cerebellar neuroblastoma may be a less malignant tumor than its other primitive neuroectodermal posterior fossa counterparts. Furthermore, this case suggests a role for second-look surgery in the management of selected pediatric brain tumors.