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Biomedical subjects

N Brand

Publications and source records attributed to N Brand.

70 records · Page 4Linked to original sources

Kinetics of CSF phenytoin in children.

The efficacy of intravenous phenytoin for the treatment of status epilepticus is related to the rapid entry of phenytoin into brain parenchyma. There is no information concerning the correlation between phenytoin serum and CSF concentrations in children, and the application of CSF data to clinical use. We report 7 children (2-11 yrs) who were treated or exposed to phenytoin in doses between 10.5-230 mg/kg. Lumbar puncture was performed 9 times in 6 of the patients. In one patient, an intraventricular catheter permitted successive assessment of CSF phenytoin concentrations. The ratio of CSF/serum phenytoin concentrations was 0.16 +/- 0.08, with gradual increase over the first 8 hours as the serum phenytoin concentration decreased. There was good correlation between therapeutic outcome and CSF phenytoin levels higher than 2 mcg/ml. In one patient the coma state secondary to phenytoin intoxication was associated with high CSF concentration (6 mcg/ml).

Age Factors↗

Congenital rubella in Israel following the 1978-79 rubella epidemic.

We conducted a retrospective survey of children who were born with congenital rubella syndrome (CR) resulting from a recent rubella epidemic. Sources of information were hospital and laboratory records and data collected in an active search for deaf children born following the epidemic and attending rehabilitation centers for the deaf (Micha). Criteria for inclusion in the survey were: 1) major clinical defects, and 2) one or more of the following positive laboratory findings--virus isolation, presence of rubella-specific IgM antibodies, or the presence of hemagglutination inhibition (HI) antibodies in children beyond the age of 1 year. Excluded from the study were 28 children with persistent HI antibodies, but without clinically detected defects. CR was identified in 45, among them 43 with deafness. Other major defects were psychomotor retardation, microcephaly, cataracts and heart defects. Transient abnormalities included encephalitis, hepatosplenomegaly, jaundice, thrombocytopenia, intrauterine grown retardation and failure to thrive. Thirty-one mothers (70%) reported a history of clinical rubella in pregnancy, the others having experienced subclinical infection. Multiple defects were found in children born following early gestational rubella (less than 2 months); abnormalities also occurred as a consequence of rubella as late as the fifth month of gestation.

Antibodies, Viral↗

Spinal intradural lipoma with intracranial extension.

2 cases of intradural spinal lipomas with extension into the posterior fossa in infancy are presented. Clinical features included associated subcutaneous posterior spinal lipomas and hypotonia. The cases were diagnosed by metrizamide myelography and spinocranial computerized tomography scanning. Operative therapy consisted of combined suboccipital craniectomy, spinal laminotomy, flap elevation and subtotal tumor decompression. The entity is reviewed.

Brain Neoplasms↗

Fatal neonatal central nervous system infection caused by Citrobacter diversus.

Citrobacter diversus is a serious, albeit rare, offender of the central nervous system (CNS) in the neonatal period and in early infancy. We report here a case of neonatal CNS infection caused by C. diversus. The course of the illness was one of rapid deterioration, leading to recurrent seizures, coma and death. Since neonatal C. diversus CNS infection carries a grave prognosis, intraventricular administration of gentamicin should be considered.

Citrobacter↗

Metachromatic leukodystrophy without arylsulfatase A deficiency.

Two siblings of consanguinous parents were noted to have a neurologic syndrome marked by developmental delay, regression of psychomotor performance, marked spasticity and progressive central nervous system degeneration. Markedly delayed nerve conduction times and a sural nerve biopsy which demonstrated changes typical of metachromatic leukodystrophy (MLD) were evident. Impairment of sulfated glycolipid metabolism was documented by analysis of glycospingolipid in urinary sediment. In spite of these findings, activities of arylsulfatase A and cerebroside sulfatidase in white blood cells and cultured skin fibroblasts were near normal. However, when intact growing fibroblasts were loaded with 35SO4-sulfatide a clear defect in sulfatide cleavage, comparable to that seen in MLD patients, was observed. Thus, these patients represent a new form of sulfatide storage disease -- MLD characterized by intact enzyme activity in cell homogenates but defective sulfolipid metabolism in vivo and in intact fibroblasts.

Adult↗

Uncommon neurologic complications of burns in infants: a parkinsonian extrapyramidal disorder and massive cerebral infarction.

We report uncommon neurologic complications of moderate to severe burns in two infants aged 13 and 19 months, respectively. The first patient suffered a 25% total body surface area burn to her lower limbs; 3 days later she became mute and irritable, with increasing rigidity of limbs and trunk. Her face was expressionless and she also had mild dysphagea. Muscle-stretch reflexes were brisk and accompanied by episodes of coarse jitteriness. A diagnosis of an extrapyramidal parkinsonian disorder was made. The infant was treated with amantadine hydrochloride, and she recovered completely over a period of 2 months. The second infant was admitted with an extensive burn to his trunk and limbs; 3 days later he became comatose and had intractable seizures, which were more prominent over his right side. Dysphasia and right-sided hemiplegia became apparent; these conditions were and remained permanent, accompanied by focal seizures and a marked cognitive delay. Although relatively uncommon, central nervous system involvement may be a hazardous complication of burns in infants, who thus represent a high-risk group.

Amantadine↗

Familial dysautonomia with Riga-Fede's disease: report of case.

Due to insensitivity to pain, various complications in familial dysautonomia were reported. To the best of our knowledge, occurrence of Riga-Fede disease was not described. A simple restorative procedure helped to bring about the rapid healing of the ulcerative lesions of Riga-Fede disease.

Dysautonomia, Familial↗