Focal cortical dysplasia. A rare cause of epilepsy.
Explore the source record for details and available documents.
Cerebral Cortex↗
Biomedical subjects
O Robain
Publications and source records attributed to O Robain.
Inverted neurons in agyria. A Golgi study of a case with abnormal chromosome 17.
An anatomoclinical observation of agyria is reported. The karyotype revealed a partial deletion of the short arm of chromosome 17. The etiology of agyria is reviewed in the light of this chromosomal abnormality. In addition we describe the peculiar pattern of neurons in the cortex: Golgi stain demonstrated many inverted pyramidal cells in the superficial part of the cortical layer. The mechanism of this abnormality is discussed.
Abnormalities, Multiple↗
Role of VSV G antigen in the development of experimental spongiform encephalopathy in mice.
The ts G31 VSV mutant induced spongiform encephalopathy without any inflammatory response when injected i.c. into the mouse. In electron microscopy, no virions could be detected in spongiform lesions. In contrast, with the wild VSV strain inflammatory lesions were seen, which contained viral particles in great abundance. As previously shown in vitro, when using the ts G 31 mutant at the nonpermissive temperature, the G antigen can spread from membrane to membrane to distant sites, fusing a great number of cells even in the absence of virus multiplication. Therefore, we postulate that a comparable mechanism is responsible for extensive brain lesions originating probably from a relatively small number of G antigen-producing cells. Indeed, the spongious regions seen mainly in the grey matter contained vacuoles, whose walls were clearly stained by peroxidase-labelled immune serum to G antigen, without detectable virions or inflammatory lesions. The vacuoles probably represent altered and swollen dendritic cell membranes. The relationship between spongiosis development and antigen diffusion in the absence of significant virus replication is discussed.
Animals↗
Focal perivascular alterations of white matter in herpes simplex encephalitis--a histological and immunocytochemical study.
Several cases of human herpes simplex encephalitis treated with Vidarabin have been investigated with the histological and immunocytochemical techniques. Cases with a subacute evolution revealed areas of focal perivascular myelin destruction in the white matter. The distribution of herpes simplex antigen did not show any preferential localization of the virus in perivascular oligoglial cells. In contrast, a spatial and temporal relationship has been found between the appearance of immunoglobulin-bearing cells around the vessels and that of areas of focal perivascular myelin damage. Therefore, it is postulated that the areas of focal destruction of myelin are not related to the cytotoxic effect of the virus but are rather dependent on the immune response of the host.
Adult↗
Neonatal adrenoleukodystrophy.
Nine cases of neonatal adrenoleukodystrophy are described. All patients had abnormal facial features, moderate to severe hypotonia, hepatomegaly, and retinitis pigmentosa. The clinical course was rapidly progressive in six cases and more protracted in three others. Biological signs of adrenal insufficiency were present in five cases. CT scan showed a demyelinating process in four patients. Trilamellar inclusions were found in the liver of four cases and dark and complex lipidic inclusions in three other cases. In the three necropsied patients there was severe alteration of the white matter involving particularly the cerebellum in two cases. Gyral and cytoarchitectonic disturbances were absent in all three cases. Increased plasma levels of very long chain fatty acids (8/8), phytanic acid (7/8) and bile fluid trihydroxycoprostanic acid (2/4) confirmed the deficiency of multiple peroxisomal enzymes. Clinical, histopathological and biochemical findings of these nine cases are compared to those reported in other neonatal adrenoleukodystrophy cases and to those of other neonatal peroxisomal disorders, that is cerebro-hepato-renal syndrome of Zellweger and infantile Refsum's disease.
Adrenal Cortex↗
[Early diagnosis of cerebral malformations].
Explore the source record for details and available documents.
Abnormalities, Multiple↗
Detection of measles virus RNA in lymphocytes from peripheral-blood and brain perivascular infiltrates of patients with subacute sclerosing panencephalitis.
To clarify the relation between lymphocytes and measles virus in subacute sclerosing panencephalitis, we used in situ hybridization and a cloned measles virus DNA probe, specific for nucleocapsid protein, to detect measles virus RNA sequences in circulating lymphocytes and brain perivascular cuffs of patients with subacute sclerosing panencephalitis. Seventy to 90 per cent of peripheral mononuclear cells from three such patients were found to contain measles virus RNA sequences. In contrast, only a few infected cells were observed in four seropositive adults (0.1 to 5 per cent) and three age-matched children (10 to 15 per cent) used as controls. In one sample of brain tissue from a patient with subacute sclerosing panencephalitis, viral RNA sequences were also detected in nerve cells and in numerous cells from the perivascular infiltrates. In contrast, no hybridization was observed in brain tissue from a patient with herpetic encephalitis and from a patient with postlymphoma encephalitis. We conclude that measles virus has a strong tropism for lymphocytes and nerve cells in subacute sclerosing panencephalitis and that lymphocytes may be involved in the pathogenesis of the disease.
Adult↗
Developmental changes in the cerebellar cortex after locus ceruleus lesion with 6-hydroxydopamine in the rat.
Three-day-old rats received an infusion of a small dose of 6-hydroxydopamine (6-OHDA) (12 micrograms) directly into the locus ceruleus nuclei, in order to induce a subtotal lesion of the noradrenergic system. Significant modifications in cerebellum maturation were observed, mainly a regression failure in the perisomatic Purkinje cell processes in the depth of the fissura prima of 10-day-old rats, a reduction in the whole vermis surface area and in the total number of internal granule cells at 15 days of age, the number of external granule cells was slightly enhanced. To discriminate between the effects of the noradrenergic deafferentation and the nonspecific toxic effects of 6-OHDA, these results were compared to those obtained after an infusion of 6-OHDA + bovine serum albumin (BSA) into the locus ceruleus (which produced a selective deafferentation) and after an injection of 6-OHDA directly into the IVth ventricle (which increased the nonspecific toxic effects). Whatever the procedure used, the same reduction in the size of the vermis was observed. However, the fissuration index was not modified when BSA was added to the 6-OHDA solution. We conclude that noradrenergic deafferentation by itself was sufficient to reduce the growth of the cerebellum at certain periods of postnatal life.
Animals↗
[Oculocerebral anomalies in Walker's lissencephaly].
Two new cases of Walker's lissencephaly are reported. In this disease first described by Walker in 1942 important cerebral malformations and various ocular anomalies are associated. The main cerebral malformations consist of hydrocephalus and agyria, and the ocular anomalies concern the anterior segment as well as the retina which is frequently dysplastic. The originality of our cases is due to the retina which was not dysplastic but showed particular modifications which are discussed. Moreover, in the second case the cerebral cortex was rather microgyric. The aspect of the ocular lesions and of some of the cerebro-meningeal findings bring the authors to discuss the autosomal recessive inheritance proposed by some neuropathologists; these aspects can suggest a possible foetopathy.
Abnormalities, Multiple↗
Necrotising leukoencephalopathy complicating treatment of childhood leukaemia.
Nine children treated for acute leukemia or lymphosarcoma developed subacute encephalopathy starting with listlessness, depression and impairment of speech. Walking difficulties, ataxia, spasticity and sphincter disorders developed later. Transient intracranial hypertension and abnormal movements respectively developed in two patients. EEG frontal slow waves, raised CSF protein, abnormal white matter radioisotope uptake and CT scan hypodensity with patchy contrast enhancement were evident at the onset. Later, dilated ventricles and calcification appeared in the younger patients. Post-mortem neuropathological studies of three patients disclosed predominantly perivascular myelin loss in areas of white matter necrosis, abnormalities of small vessels and numerous axonal swellings. The spinal cord showed secondary degeneration of the corticospinal tracts. Analysis of the aetiological factors in this series points to the prevailing danger of cranial radiotherapy, probably increased by the young age of patients and by associated drug administration.
Acute Disease↗
Pachygyria and congenital nephrosis disorder of migration and neuronal orientation.
A case of pachygyria with associated nephrosis has been studied. Several microscopic abnormalities have been identified: cytoarchitectonic disorders including neuronal ectopies in the molecular layer and in the meninges, improperly oriented neurons shown with Golgi stain, fetal aspect of inferior olives. The mechanism of the disorder of migration and neuronal and dendritic orientation are discussed. The significance of the association of microcephaly and nephrosis is also reviewed in light of recent literature.
Brain↗
Experimental phenylketonuria: effect of phenylacetate intoxication on number of synapses in the cerebellar cortex of the rat.
In experimental phenylketonuria, induced in the rat by exposure to phenylacetate during the first 21 days of life, there was a significant reduction of boutons, a decrease of an average of 25% in the whole cerebellar molecular layer. Both the density of synaptic profiles per square unit and the number of synapses per unit volume were decreased in the phenylacetate-treated rat as compared to the age-matched control. Neuronal density was unaffected. Results are interpreted to show a deficit of synapses per neuron, probably due to a decrease in synaptic formation in phenylacetate-induced phenylketonuria. Undernutrition was eliminated as a contributing factor.
Animals↗
Developmental changes of synapses in the cerebellar cortex of the rat. A quantitative analysis.
Synaptogenesis in the molecular layer of the vermis cortex in Wistar rats between 1 and 25 days after birth was investigated. After staining with OSO4, the following parameters were measured: the density of synaptic profiles; the percentage of the neuropil area occupied by synapses; the mean diameter of the boutons; and the numerical density of synapses in a defined volume. The detailed topographical analysis enabled us to show the following: the first synapses appear diffusely in the molecular layer; after the 10th day a synaptic gradient is present between the depth and the surface area (this gradient can be found by the count of the synapses and by studying the spatial distribution of the synaptic area); this gradient is no longer seen at the 25th day, when the density of synapses is relatively uniform throughout the whole molecular layer.
Aging↗
Purkinje cell dendritic development in experimental phenylketonuria. A quantitative analysis.
A comparison was made of cerebellar dendritic development in the normal rat and in a new model of phenylketonuria, the phenylacetate-treated suckling rat. Golgi stain analysis of the Purkinje cells shows striking regional variations in the dendritic growth. These variations were observed in both the control and phenylacetate-treated animals and were especially striking before 15 days of life. Quantitative analysis of the dendritic tree revealed, in the phenylacetate-treated rat, a significant reduction in the total number of dendritic branches. However, the individual terminal dendritic length was largely unaltered. These effects of phenylacetate differ from those of deafferentation, and starvation. Results of this investigation clearly define the harmful effects of phenylacetate on developing neurons and are compatible with the clinical observation that brain damage in phenylketonuria occurs mainly during the first few years of life, the critical period of neuronal development.
Animals↗
[Progressive bulbar paralysis. Report of a juvenile case (author's transl)].
The authors report the case of a 16 year-old girl with the following features: clinically, a progressive bulbar paralysis, a weakness and wasting of muscles predominantly in the upper limbs; pathologically, a severe neuronal loss in the motor nuclei of the VIIth, IXth, XIIth cranial nerves and in the anterior horns of the cervical and thoracic spinal cord, a demyelinisation of the corticospinal tracts. A classification of progressive bulbar paralysies in infants and children is proposed. In the first group, the peripheral motoneuron is the only involved. Such cases are often called Fazio-Londe disease and can be related to those cases of infantile spinal amyotrophy either of the Werdnig-Hoffmann type or, most often, of the Wolfhardt-Kugelberg type. In the second group, the corticospinal tract is also involved. Some of these cases can be included in the spinocerebellar degenerations but others, such as the case reported here, are strongly reminiscent of the adult amyotrophic lateral sclerosis.
Adolescent↗
[Kearns-Sayre syndrome with hypoparathyroidism and diabetes (author's transl)].
Explore the source record for details and available documents.
Arrhythmias, Cardiac↗
Effects of undernutrition on glial maturation.
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.
Animal Nutritional Physiological Phenomena↗
[The jimpy mouse].
Ultrastructural study of the Jimpy mice shows lack of myelin in the central nervous system: low density of glial cells, defect of maturation of oligodendrocytes and deficiency in axonal growth. The lack of myelin is more proeminent in late myelinating structures: corpus callosum and corticospinal fasciculus. Failure in myelin is rather the consequence of an early interruption of myelination as that of demyelination (a destruction of the sheats).
Aging↗