[Benign intracranial hypertension in infants and children. Comments on 33 cases (author's transl)].
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Biomedical subjects
Publications and source records attributed to G Ponsot.
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Four cases of acute bacterial meningitis complicated by medullary involvement are described. The complication presenting as a transverse myelitis occurred early and suddenly in the course of severe bacterial meningitis caused by different bacteria. Only two other comparable cases have been reported.
An 11-year-old girl with 10q26qter deletion is described and compared with another patient reported in the literature. The most characteristic features of monosomy 10qter seem to be: severe mental retardation; growth retardation; microcephaly; and facial dysmorphism with a long and triangular facies, a broad and prominent nasal bridge, a poorly developed tip of the nose, a short philtrum, and flattened angles of the mandible. Several of these features are opposed in type and countertype to features of trisomy 10qter.
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The main clinical features of acute infectious polyneuritis are described from a study of 100 cases. The distinction between Guillain-Barré syndrome and the atypical forms of the condition is arbitrary. In particular, the atypical features (pleocytosis, normal C.S.F. protein, relapses) had no influence on the final prognosis. In contrast, the severity of the distal neurological deficit, the duration of the acute illness, a late dissociation between the cells and proteins C.S.F., the age and sex were all statistically significant prognostic factors.
Thirty cases of primary intramedullary tumours have been analysed (17 astrocytomas, 6 ependymomas, 6 non-glial tumours) and 10 have died. The long delay in diagnosis (more than 3 years) must be emphasised. The value of myelography, the natural history and treatment are discussed.
Large quantities of type I interferon were detected in the cerebrospinal fluid (CSF) collected at the onset of herpes encephalitis. This interferon was synthesized intrathecally and disappeared about 10 days after the beginning of neurological signs. In 12 cases of post-eruptive measles encephalitis and in four post-rubella encephalitis, type I interferon was present only in low amounts, or not found at all, even in the CSF collected early. The existence of an intrathecal synthesis of interferon during encephalitis provides a valuable contribution to both the early diagnosis of herpes encephalitis and the study of the pathogenesis of virus infection of the central nervous system (CNS).
Rats of Wistar strain were conceived and breast-fed until the 25th day by mothers maintained on a low protein (5%) and low caloric (21 calories/day) diet, producing a severe deficiency in weight body growth (more than 50% at the 10th day) and of the weight of the central nervous system (40% on the 15th day) both in the cerebral hemispheres and the spinal cord. Histological and biochemical analysis of the central nervous system shows: (1) Glial proliferation is insufficient and delayed, the number of glial cells is reduced by 50% on the 10th day in the cuneatus and gracilis tracts and the density of the glial cells is reduced by 50% in the corpus callosum at the 19th day. (2) Maturation of the glial cells is greatly retarded, especially in the corpus callosum a structure which matures late. On the 19th day, the majority of the cells in this structure still have a glioblastic appearence, whereas in the normal rat, at this age the majority of the glial cells are oligodendrocytes. (3) These abnormalities of glial maturation agree well with the delay of the increase of DNA, RNA and protein measured in the spinal cord and cerebral hemispheres. (4) There is a defect in myelination assessed by estimation of the density of the myelin fibres, and a definitive reduction in the caliber of the spinal tracts.
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Twenty-two cases of sub-tentorium cerebral abscesses were observed in children. The first clinical symptoms were those of intracranial hypertension, disorders of consciousness, specially somnolence, fever and low general condition. Focal neurological signs were frequent but appeared later. A cerebral abscess should be suspected when such symptoms occur in children with heart disease or with respiratory infection. Simple investigations like examination of the fundi, X-ray of the skull and EEG give valuable clues. In case of such a clinical picture, lumbar puncture is useless and often dangerous. The best diagnostic test is a cerebral scintigram.
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An acute measles encephalitis with epilepsia partialis continua occurred three months after measles in a previously healthy child with no detectable immunological defect. Levels of measles antibodies in serum and cerebrospinal fluid were high, and tubular inclusions of the type seen in subacute sclerosing panencephalitis were found in nerve cell nuclei. A communicating high-pressure hydrocephalus developed in the later stage of the disease. This case demonstrates that measles encephalitis of the delayed type should systematically be considered in children with acute encephalitis.
The authors have studied 59 cases of Bourneville's tuberous sclerosis, including 44 sporadic cases from healthy parents and 9 familial patients. They stress the very high frequency of spasms in the infants of less than one year (80 %), the frequency and the early appearance of retinal phakomata and areas of depigmentation and the interest in the early detection of intracranial calcification. They discuss the specificity of the individual manifestations: cutaneous, retinal and systemic and they consider the problem of genetic counselling.
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Female rats were submitted to caloric and protein restriction 8 days before fecondation and during pregnancy. A reduction of the number of brain cells in the fetuses was evident only after the 20th day of pregnancy. This reduction appeared to be limited to glial cells, and not to affect neurons contrarily to what had previously been stated by some authors.
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