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Biomedical subjects

O Dulac

Publications and source records attributed to O Dulac.

At least 163 records · Page 9Linked to original sources
PubMed1986

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↗
PubMed1986

Congenital muscular dystrophy and cerebral CT scan anomalies. Results of a collaborative study of the Société de Neurologie Infantile.

We present the results of a collaborative study on the association of congenital muscular dystrophy with central nervous system anomalies revealed by CT scan investigation of 10 patients. In seven children, an abnormal hypodensity of the cerebral white matter is found; in four of these patients, this radiological anomaly is either isolated, or associated with a moderate intellectual impairment; in one case, severe mental retardation and ocular changes had occurred; in the other two cases, the muscular disease was progressing slowly, in association with microcephaly, epilepsy, and moderate mental retardation. Three children were afflicted with a severe early encephalopathy and congenital muscular dystrophy, and presented signs of cortical and subcortical atrophy on CT scan. Two of these patients corresponded to different types of cerebro-ocular dysplasia-muscular dystrophy syndromes, and the third patient of Fukuyama's congenital muscular dystrophy. These observations are discussed and compared with those reported in the literature. The authors emphasize the need to investigate possible cerebral CT scan anomalies in congenital muscular dystrophies, and to look for muscular changes in some prenatal encephalopathies.

Atrophy↗
PubMed1986

[Electroclinical characteristics of seizures in the Aicardi syndrome].

Electro-clinical patterns of seizures were studied in 7 infants with Aicardi Syndrome. In all 7 cases, partial seizure preceded the appearance of asymetrical spasms. Partial seizures and spasms were recorded by polygraphing tracings of long duration. There was a constant correlation between the EEG localization of partial seizures and the side of asymetrical spasms. In 6 cases, a cluster of spasms followed the partial discharge whereas in the 7th case, the partial discharge appeared in the middle of the cluster. Therefore, both partial seizure and cluster of spasms seem to be part of the same critical manifestation. The authors have recorded the same king of seizures in other malformative syndromes (lissencephaly, Bourneville disease) and compare them to those recorded in Aicardi Syndrome. In Aicardi Syndrome, this type of seizures might be in relation with the association of a corpus callosum agenesy and paraventricular heterotopies.

Agenesis of Corpus Callosum↗
PubMed1986

[Benign epileptic infantile spasms].

Among 100 infants with infantile spasms studied before treatment, 14 had idiopathic spasms with a favourable outcome. They had a normal development, including reaching for objects before the age of 5 months and moderate regression without loss of eye following. Although it was hysarhythmic, the EEG tracing showed identifiable basic activity and sleep spindles; there was no slow waves focus even after diazepam administration and the spasms were "independent" even during a cluster. This type of infantile spasms seems to be a particular type of non-lesional epilepsy. It contrasts with idiopathic spasms of unfavourable outcome that apparently result from a preexisting and overlooked focal lesion responsible of later cognitive troubles.

Child, Preschool↗
PubMed1986

Sodium valproate monotherapy in childhood epilepsy.

154 patients with a mean age of 6 years 1 month were followed on valproate monotherapy for a period ranging from 5 to 27 months (mean 22 months). Absence epilepsies, benign myoclonic epilepsies and epilepsies with tonic-clonic seizures on awakening were the best controlled, followed by benign partial epilepsies and infantile spasms. Reduction to monotherapy resulted in improvement in 13 of 14 patients with primary generalized epilepsy. Sixteen per cent of the 154 patients suffered mild to moderate adverse effects. After cessation of treatment in 28 seizure-free patients, no recurrence was observed in absence epilepsy, benign myoclonic epilepsy, infantile spasms or benign partial epilepsy, whereas two thirds of the patients with generalized tonic-clonic seizures on awakening relapsed in the year following the cessation of the treatment.

Adolescent↗
PubMed1985

Congenital toxoplasmosis. Clinical and neuroradiological evaluation of the cerebral lesions.

A study of 31 observations of congenital toxoplasmosis shows that there is a clear relationship between the cerebral lesions as observed on CT scan, neurological symptoms, and the date of maternal infection. The appearance of the CT scan is characteristic of cases with early maternal seroconversion (before the 20th week of pregnancy), of cases with maternal infection between the 20 and 30th week of pregnancy, and of cases with late maternal seroconversion (after the 30th week of pregnancy). Preventive maternal treatment does not change the pattern of the cerebral lesions observed on CT scan and thus proves ineffective in conferring real protection.

Brain↗
PubMed1985

[Status epilepticus in the infant. Semeiologic, etiologic and prognostic aspects].

During a 4 year period, 79 infants aged from 1 month to 2 years suffered a status epilepticus (SE). The seizures were most often either generalized of tonic-clonic or clonic type, or unilateral clonic; they lasted or were repeated with persistent unconsciousness between the seizures for periods ranging from 30 min to several days. All the cases of SE lasting over 6 h resulted from a recognizable acute brain injury, whereas 11 of 14 cases lasting 1 h or less were cryptogenic. In 2 cases only, the neurological status was worse after the status, apparently as a result of the seizures themselves. The authors discuss the hypothesis of purely febrile HH syndrome and point out the importance of detailed clinical ictal and interictal characteristics for the etiological approach.

Child, Preschool↗
PubMed1985

Infantile status epilepticus as a complication of 'near-miss' sudden infant death.

Among 13 infants with apparently cryptogenic status epilepticus occurring between two and four months of age, two shared several features of 'poliodystrophy' encephalopathy. For the other 11, an anoxic-ischaemic event was suggested by the history, the seizure and EEG characteristics, and clinical and CT-scan evolution. The possible relationship with 'near-miss' sudden infant death is discussed.

Brain↗
PubMed1985

[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↗
PubMed1985

[Extradural hematoma in children under 1 year of age].

The authors report 3 cases of epidural hematoma in infants under one year of age. The trauma had been minimal with a long symptom-free interval preceding the first clinical symptoms: anemia and its consequences, pallor, bradycardia, and finally shock. CT scan revealed the hematoma. In one case, it showed an asymptomatic hematoma which posed a therapeutic problem.

Craniocerebral Trauma↗
PubMed1984

Abnormal galactoside excretion in urine of a patient with early myoclonic epileptic encephalopathy.

An abnormal carbohydrate pattern was found in urine of a patient with early myoclonic epileptic encephalopathy. Three major oligosaccharides have been isolated from the urine; structural studies including sugar analyses, methylation procedure and enzymatic hydrolysis allow us to propose the following structures: beta-Gal-(1 leads to 3)-Gal beta-Gal-(1 leads to 3)-beta-Gal-(1 leads to 3)-Glc beta-Gal-(1 leads to 3)-beta-Gal-(1 leads to 3)-Gal Such oligosaccharide structures have not previously been described in any biological fluid. The origin of these compounds, and the possibility of a specific metabolic defect are discussed.

Chemical Phenomena↗
PubMed1984

[Scanography in purulent meningitis in newborn infants].

Brain CT scans of 40 neonates with bacterial meningitis showed that the main lesions consisted of obstacles to CSF flow resulting in hydrocephalus during the second month; single or multiple foci of ischaemia of very early onset appearing as hypodense areas sometimes contrast-enhanced, and abscesses consecutive to haematogenous septic necrosis. The organisms responsible for these abscesses usually were Proteus spp., more rarely other enterobacteria. Attention is drawn to the usefulness of CT in detecting the nature and size of lesions which are often clinically silent, except for convulsions.

Brain↗
PubMed1984

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↗
PubMed1984

[Pediatric dermatomyositis. Apropos of 28 cases].

The authors report 28 cases of juvenile dermatomyositis. Bohan and Peter criteria were fulfilled in all cases. All children received a treatment with prednisone as soon as diagnosis was established. Five patients (18%) died during this treatment. The remaining children were followed for at least 2 years after prednisone treatment was discontinued. An acute onset was found in 4 of the 5 children who died and in 6 of 23 children still alive. Swallowing disorders were present in all children who died and in 11 of the children still alive. Ten children recovered fully and 13 presented with sequellae. The average interval between clinical onset and diagnosis was 2 months 19 days for the children who recovered and 5 months 21 days for those presenting with sequellae. The average duration of the period of activity of dermatomyositis was 6 months for the children who recovered and 3 years 4 months for those with sequellae.

Adolescent↗
PubMed1983

[Monotherapy with clobazam in epilepsies in children].

Twenty-five epileptic children were treated with clobazam alone for periods of 10 days to 36 months (mean = 9 months and 16 months for children who responded well). Satisfactory results were obtained in 11 patients and marked improvement with a 75% decrease in the initial frequency of seizures in 6 patients. The seizures recurred after a few weeks in 3 other patients. This preliminary study shows that clobazam is well tolerated and remarkably effective, especially in benign partial epilepsy, even in carbamazepine-resistant cases.

Adolescent↗