Marvin A. Fishman

Neck-tongue syndrome

Two patients with neck-tongue syndrome, a rare disorder of the upper cervical nerves that results in paroxysmal neck pain and paresthesia of the tongue, are described. Signs are the result of compression of the C2 root by disorders affecting the first two cervical vertebrae. Conservative management is effective in most cases if no masses are in the involved area. The disorder has been observed rarely in children. Clinical onset occurred at 6 and 11 years of age, respectively, in the reported patients.

Acute infantile hemiplegia with cerebrospinal fluid eosinophilic pleocytosis: An unusual case of visceral larva migrans

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The effect of malnutrition on human central nervous system myelin

For many years it has been suspected that moderate to severe undernutrition beginning in the neonatal period of human beings results in a population whose intellectual functions are impaired compared with those of a well-fed population of comparable genetic background.’ -3 Although such studies are frequently criticized because of the difficulty in controlling other environmental variables, accumulating evidence supports the concept that undernutrition early in life can impair cognitive development. In recent years, confirmatory evidence of the deleterious effects of undernutrition on cerebral development has been obtained by studies indicating that the rate of brain growth of undernourished infants and children is less than that of controls and that the brain’s DNA content is r e d ~ c e d . ~ If animals are undernourished early in neonatal life, they too exhibit a reduction in brain growth and DNA content5 However, the most striking change is in the lipid content of these undernourished brains when compared with brains of age-matched controk6 Total lipid is considerably reduced, but the most pronounced reductions are in cerebrosides, plasmalogens, and proteolipid proteins, all of which are concentrated in brain white matter and rise rapidly with the formation of myelin.’,’ Recently we noted that severely undernourished human infants exhibited the same lipid changes in their white matter as did malnourished animalsg The most obvious interpretation of these data is that undernutrition severely restricts myelin formation. However, it has been known for

Cerebellar hemorrhage in the term neonate: Developmental and neurologic outcome

To elucidate the effects of cerebellar hemorrhage on the term neonate, neurodevelopmental assessments were conducted at a mean age of 32 months on six children. In addition to cerebellar hemorrhage, ventriculomegaly was present on each subjects initial computed tomographic scan. All were managed without surgical evacuation. Two patients required shunts for progressive ventriculomegaly. Five patients had follow-up computed tomography indicating mild atrophy of the superior anterior vermis of the cerebellum; however, none had abnormal ventricular size or abnormalities of the cerebrum. On detailed examination conducted between the ages of 18 and 48 months, five had hypotonia, truncal ataxia, and intention tremor; two had nystagmus. Only one patient walked independently. Intellectual performance of four patients was within the retarded range and two had mildly delayed development. Two patients had markedly disordered expressive language. These data suggest that term neonates surviving cerebellar hemorrhage have neurologic deficits related to the site of hemorrhage, and cognitive deficits related to more generalized cerebral insult.

Studies with antisera against peripheral nervous system myelin and myelin basic proteins. I. Effects of antiserum upon living cultures of nervous tissue.

We studied the effects of antiserum against rat peripheral nervous system (PNS) myelin, rat or chicken central nervous system myelin basic protein (BP), or rabbit P2 protein from PNS myelin on myelinated cultures containing only rat dorsal root ganglion neurons and Schwann cells. While anti-PNS myelin serum consistently produced segmental PNS demyelination, anti-BP serum and anti-P2 serum did not. The culture results suggest that the myelin PNS proteins P1 (identical to basic protein from central nervous system myelin) and P2 are not exposed on the extracellular surfaces of myelin-related Schwann cells in tissue culture.

Barbiturates and hyperventilation during intracranial hypertension

OBJECTIVE The purpose of this study was to determine the effect of hyperventilation alone and hyperventilation plus barbiturate therapy on intracranial pressure, global and regional cerebral blood flow rates, cerebrovascular resistance, and cerebral perfusion pressure in adult dogs with and without intracranial hypertension induced by epidural balloon. DESIGN Prospective, randomized, controlled study. SETTING An animal laboratory of a university hospital. Four sequential global and regional cerebral blood flow determinations were made in each animal during monitoring of heart rate and systemic arterial pressure, during respiratory control and arterial blood gas monitoring, intracranial pressure monitoring, and with or without inflation of an epidural balloon catheter. SUBJECTS Acute mongrel dogs obtained from the Baylor Center for Comparative Medicine. Five groups of animals were studied. In group 1, the response to hyperventilation was assessed in dogs without increased intracranial pressure. In group 2, the response to hyperventilation was assessed in animals with acute intracranial hypertension. In group 3, the response to hyperventilation plus barbiturate therapy was assessed in dogs without increased intracranial pressure. In group 4, the response to hyperventilation plus barbiturate therapy was assessed in dogs with acute increased intracranial pressure. In group 5, a group of dogs with increased intracranial pressure was treated with neither hyperventilation nor barbiturates. INTERVENTIONS Hyperventilation, hyperventilation plus barbiturate therapy, or no interventions were studied in these experimental paradigms. MEASUREMENTS AND MAIN RESULTS The main outcome measures were changes in intracranial pressure and/or changes in regional or total cerebral blood flow. A significant decrease in intracranial pressure and cerebral blood flow rate was produced by hyperventilation alone in groups with intracranial hypertension. Combined hyperventilation and barbiturate therapy resulted in a significant further decrease in cerebral blood flow rate in animals with normal and increased intracranial pressure, but no greater decrease in intracranial pressure was seen compared with treatment with hyperventilation alone. Cerebral perfusion pressures remained normal despite significant decreases in cerebral blood flow rates. CONCLUSIONS These studies suggest that barbiturate administration in this model of intracranial hypertension was no more effective in reducing increased intracranial pressure than hyperventilation alone.

Basal ganglia calcifications in a case of biotinidase deficiency.

Biotinidase deficiency leads to a biotin-deficient state, with cardinal symptoms of ataxia, alopecia, and skin rash presenting in infancy. Previous reports of head CTs in patients with biotinidase deficiency did not note basal ganglia calcifications. We report the first case of biotinidase deficiency with basal ganglia calcifications. There were no symptoms referable to basal ganglia dysfunction.

The Validity of Psychometric Testing in Children with Congenital Malformations of the Central Nervous System

Serial psychometric testing from infancy to five years or older was performed on 31 patients with congenital malformations of the central nervous system. The developmental quotient obtained on the Cattell Intelligence Scale administered at 18 months was found to have a high correlation coefficient with later intellectual achievement—as measured by Stanford‐Binet Intelligence Scale at five years of age. Psychometric testing performed in infancy in this group of patients appears to have predictive value for later intelligence and may supply useful information for counseling parents and in planning educational needs.

Multicore Myopathy, Microcephaly, Aganglionosis, and Short Stature:

Multicore myopathy, classified with the benign congenital myopathies, is manifest clinically as proximal muscle weakness, hypotonia, and delayed motor development. We report an unusual case of multicore myopathy with an expanded clinical syndrome involving the central nervous system, as well as additional congenital malformations. Clinical manifestations included microcephaly, mental retardation, spasticity with hyperreflexia, cerebellar dysfunction, short stature, Hirschsprungs disease, pharyngeal web, and facial dysmorphism. (J Child Neurol 1994;9:275-277).

Studies with antisera against peripheral nervous system myelin and myelin basic proteins. II. Immunohistochemical studies in cultures of rat dorsal root ganglion neurons and Schwann cells

Antiserum against rat peripheral nervous system (PNS) myelin contained immunoglobulins which bound preferentially to the extracellular surfaces of myelin-related Schwann cells in intact cultures of dorsal root ganglion (DRG) neurons and Schwann cells, while antiserum against basic protein (BP) from central nervous system myelin or the PNS basic protein P2 did not. We demonstrate the presence of PNS myelin proteins P1 (identical to BP) and P2 by immunoperoxidase techniques in DRG cultures that had been treated to disrupt cellular membranes. These observations suggest that P1 and P2 are not exposed on the extracellular surfaces of myelin-related Schwann cells in culture. The results also support the hypothesis concerning the possible mechanisms by which anti-PNS myelin serum demyelinates DRG cultures, while anti-BP serum and anti-P2 serum do not.