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Biomedical subjects

O Dulac

Publications and source records attributed to O Dulac.

At least 181 records · Page 10Linked to original sources
PubMed1983

[Electroencephalographic aspects of classic agyria-pachygyria].

Four infants had agyria confirmed by CT scan. All were mentally retarded, microcephalic, 3 of them having characteristic facial dysmorphy and 2 infantile spasms. EEG was characterized by very high amplitude rhythms in the alpha range, associated with delta waves and with infrequent spikes. The tracing was poorly modified by sleep. Drugs reduced its amplitude and rapid rhythms appeared with benzodiazepines. The authors point to the usefulness of the EEG in suggesting diagnosis before the CT scan, as was the case in 2 patients. They point to the urgent need of studying infantile epilepsies according to etiology, and not only to seizure types.

Cerebral Cortex↗
PubMed1983

[Hypothalamic hamartomas and laughing seizures].

The authors report 7 new cases of children with laughing seizures associated with hamartoma of the hypothalamus. Precocious puberty is present in 4 cases and seizures of another type (clonic or tonic) in 5 cases. The diagnosis is confirmed by histology (2 cases) or by CT scan (5 cases). The interictal EEG abnormalities are detailed as well as those found during the laughing seizures which were registered (3 cases). The literature was reviewed and 18 cases were found with posterior hypothalamic lesions and laughing seizures: they have been studied and compared with ours. The role of hamartoma is discussed in the production of these seizures, either isolated laughing seizures or else laughing seizures accompanied with other symptoms.

Adolescent↗
PubMed1983

[Unilateral status epilepticus during the neonatal period].

Twenty-one newborns displaying a strictly unilateral status epilepticus were studied. Three groups have been isolated according to the aetiology: neonatal anoxia (n = 5), postcardiac surgery (n = 8), cerebral hemispheric lesions confirmed by CT scan (n = 7). The authors relate in detail the clinical features and the EEG abnormalities, ictal and interictal in each group, and comment on their results. They show the importance of EEG in establishing or detecting a unilateral status epilepticus and in helping the aetiologic diagnosis.

Brain Diseases↗
PubMed1983

[Apparent deafness as a manifestation of an aphasia-epilepsy syndrome].

Seven children with clinically evident deafness not confirmed by objective audiometric tests, and an acquired language deficiency, presented marked paroxysmal anomalies on electroencephalogram tracings. The aphasia-epilepsy syndrome can only be recognized by routine electroencephalograms, conducted in such cases even when a history of epileptic attacks is not abtained.

Aphasia↗
PubMed1983

[Monotherapy with carbamazepine in childhood epilepsies].

68 epileptic children were treated with carbamazepine (CBZ) monotherapy for a mean period of 10 months. The seizures disappeared in 43% and decreased in another 26.5% of cases. The treatment was particularly efficient in partial benign epilepsies (disappearance: 64.7%, reduction: 29.4%) and in partial unclassifiable epilepsies (disappearance: 50%, reduction: 25%). It is sometimes useful to achieve blood levels over 4 micrograms/ml; but over 8 micrograms/ml the frequency of side-effects becomes greater than improvement of fit frequency. The dose-concentration relation is well correlated to age, so that blood level measurements do not need to be systematically performed. In order to obtain mean blood levels of 7 micrograms/ml 3 to 4 hours after morning intake, mean daily dose should range from: 20 mg/kg/24 h before 5 years to 10 mg/kg/24 h after 10 years.

Adolescent↗
PubMed1983

[Nosological aspects of epilepsia partialis continua in children].

Among 26 patients suffering from Epilepsia Partialis Continua, 2 major groups were observed. The first, resulting from a fixed lesion of the rolandic area, showed electro-clinical correlation of seizures; the latter disappeared during sleep; clinical and radiological follow-up failed to disclose any worsening of the cerebral lesion. The second group was characterized by progressive mental and motor deterioration, lack of electro-clinical correlation of fits, persistence of the latter during sleep and frank increase of cerebral atrophy observed on serial neuroradiological examinations. This easily recognized group seems to result from a progressive inflammatory disease of unknown cause.

Child↗
PubMed1983

[Hypothalamic hamartoma and gelastic crises. Apropos of 7 cases].

Seven cases of hypothalamic hamartomas with gelastic seizures are reported. A precocious puberty was found in 4 cases. The normal neurologic examination and lack of sign of intracranial hypertension were in contrast with the severity of the epileptic seizures, of the mental impairment and of the behavioral disorders. The fact that the presenting symptom may be gelastic seizures is stressed. CT scan is the best means to assess the diagnosis and to follow the evolution of these tumors. Except for the management of the precocious puberty, the treatment is disappointing and neurosurgical indications are quite exceptional.

Adolescent↗
PubMed1983

[Electroclinical and developmental aspects of epilepsy in the aphasia-epilepsy syndrome].

76 children are studied--66 from the literature and 10 from a personal series--who developed epileptic seizures, paroxysmal EEG abnormalities and language deterioration after a period of normal development of language. The clinical features of the fits, the age at which the seizures appear and disappear, the age of the EEG normalization and the normal clinical and radiological examinations were reminiscent of partial benign epilepsies of childhood. Shortly after the language deteriorates, the awake EEG exhibits slow spikes that are diffuse or predominate in the temporal areas, sometimes asymmetrically. During sleep, the paroxysmal abnormalities are clearly bilateral and continuous or subcontinuous. Spontaneously or under medications, the intensity of the paroxysmal abnormalities decreases before clinical improvement occurs. In comparison with the mutism resulting from acquired hearing loss, it is suggested that the language disturbances in these children result from a functional disorganization of the language centers due to the important intercritical EEG abnormalities. In some children, these functional disturbances could result from a benign epilepsy. This view leads to discuss the prognosis and the therapeutic possibilities.

Anticonvulsants↗
PubMed1982

Infantile phytanic acid storage disease, a possible variant of Refsum's disease: three cases, including ultrastructural studies of the liver.

Three cases of phytanic acid storage disease with symptoms during the first months of life are reported. Hepatomegaly, facial dysmorphia, growth and/or mental retardation and osteopenia were observed in addition to retinitis pigmentosa and neurosensory deafness. The presence of phytanic acid in serum (160-320 mumol/1 (50-100 micrograms/ml)) was accompanied by hypocholesterolaemia. Electron microscopy showed that a storage material had accumulated in mesenchymal and parenchymal liver cells. Lamellar structures were seen in hepatocytes and other storing cells. These inclusions resembled the structures found in plant chloroplasts containing phytol. Some of the clinical and biological data obtained were consistent with Refsum's disease. However, other characteristics such as mental retardation, hepatomegaly, osteopenia, hypocholesterolaemia and hypoalphalipoproteinaemia, as well as the ultrastructural findings in the liver, suggested that our patients' illness was either a phytanic acid storage disease different from the classical form of Refsum's disease, or a more severe early symptomatic form of Refsum's disease. Early diagnosis by phytanic acid assay and electron microscopic liver examination calls for prescription of a low phytanate diet in the hope of improving the child's condition.

Child↗
PubMed1982

[Early myoclonic epileptic encephalopathy (EMEE) (author's transl)].

The authors describe the electroclinical and evolutive aspects of 4 cases (including 2 brothers) of myoclonic epileptic encephalopathy beginning between 2 days and 10 weeks of life. From the onset of myoclonic jerks, polymorphous fits (partial seizures, tonic seizures) and multifocal electrical abnormalities are associated. Repeated spasms and 'suppression-burst' patterns appear later. The neurological status deteriorates progressively, leading within a few months to decerebration posture with opisthotonus. In spite of thorough neuroradiological, biochemical, cytological to metabolic investigations, etiology remains unknown. However, the electroclinical and evolutive patterns are similar to that of metabolic diseases, especially non-ketotic hyperglycemia. The authors discuss the relations between their observations and those in the literature and the nosological problems of this particular epileptic encephalopathy of infancy.

Brain Diseases↗
PubMed1982

[Adrenoleukodystrophy in children. Apropos of 20 cases].

Adrenoleukodystrophy (ALD) is a sex-linked recessive metabolic encephalopathy usually beginning in childhood. This series of 20 cases showed the following associated symptoms: mental deterioration (20 cases), behavior modification (19 cases), decreased visual acuity (17 cases), deafness (13 cases) and seizures (7 cases). Adrenal insufficiency often consisted only of melanoderma (13 cases). Diagnosis was assessed by characteristic CT-scan findings (8 of 13 cases), adrenocortical insufficiency partial glucocorticoid (19 of 19 investigated cases) or global, mineralo- and involving both aldosterone and glucocorticoid hormones (9 of 17 investigated cases) and by the presence of specific inclusions on the skin and/or conjunctival biopsy (5 of 7 cases).

Adolescent↗
PubMed1982

[Continuous partial epilepsy: syndrome and disease].

Twenty three patients are described with a Kojewnikow syndrome in order to identify possible etiological factors. According to their history, clinical and E.E.G. features, patients could be classified into two groups; 1) Eleven patients corresponded to the classical description of the Kojewnikow syndrome: variable age at onset of the disease, rare somatomotor seizures, delay of onset of myoclonic jerks often of long duration, myoclonus limited to a small region, normal neurological examination, stable hemiplegia, normal results of psychometric tests, localised E.E.G. alterations. In most of these patients the etiology of the disorder was known, and the symptoms could be related to a localised lesion of the central cortex; 2) the second group (11 patients) presented with completely different characteristics: early onset of fits (age: 2 to 10 years), presence of other seizure types, short delay of onset of myoclonic jerks, high frequency of seizures, localization of myoclonus over large parts of the body, progressive evolution of a motor syndrome, associated neurological signs and disorders of sleep and behavior, progressive mental deterioration, characteristic E.E.G. pattern with long subclinical paroxysms of slow spikes (sharp waves) with variable localization, diffuse cerebral lesions of unknown etiology were found. Only one patient could not be allocated to either group. Several circumstantial data suggest that patients of the second group may suffer from a slow virus infection. Should this hypothesis be confirmed, a reconsideration of the Kojewnikow syndrome from the etiological standpoint would be of great theoretical and practical interest.

Adolescent↗
PubMed1982

[Sturge-Weber disease: value of the topographic analysis of the facial angioma for the diagnosis of associated pial angioma (author's transl)].

A retrospective study was undertaken in 47 children with congenital capillary naevus of the face in order to appreciate the predictive value of the topography of the facial angioma for the diagnosis of associated pial angioma. In patients with Sturge-Weber disease, facial angioma concerned the whole upper eyelid, extending to the forehead and/or cheek. Less often, it was localized to the root of the nose; in such cases it was a port-wine, homogeneous angioma, with clearcut outlines, quite different from the angiomatous dots frequently seen in this area in neonates. Except for these rare cases, angiomas concerned only half or less of the upper eyelid and no pial angioma was associated. Finally, congenital capillary naevi with this topography typical of Sturge-Weber disease were frequently associated with pial angioma.

Angiomatosis↗
PubMed1982

[Ischemic cerebral softening in newborn infants. Possible etiology of neonatal convulsive states].

The authors report on 8 cases of full term neonates presenting, between the 8th and 72nd hours of life, with several often subintrant partial seizures, always in the same area for a given child. Pregnancy, delivery and the first hours of life had been normal. The first CT scan (performed a few hours to 3 days after the first fits) showed a triangular hypodensity with internal vertex, still observed at the second examination, with reduced dimensions and fuzziness of the edges. The features and localization, always in an elective arterial area, suggested an arterial infarction. The date of occurrence, the probably embolic nature and the etiology of such an arterial ischemia are discussed. The clinical, electric and CT-scan homogeneity of the picture is emphasized, as it makes diagnosis easy. Prognosis is relatively favorable among neonatal seizures states, except for possible intellectual and epileptic sequellae.

Anticonvulsants↗
PubMed1981

Aneurysms of the vein of Galen in infants aged 2 to 15 months. Diagnosis and natural evolution.

This is a report of seven cases of aneurysm of the vein of Galen (AVG) with a review of the clinical and radiological aspects of 48 cases in the literature. The natural evolution and pathophysiology of this vascular malformation are discussed. The clinical signs of this condition are often misleading. The appearance of AVG on CT is pathognomic. Surveillance of the vascular malformation and associated cerebral lesions can be maintained with subsequent CT. Spontaneous thrombosis of the aneurysm was observed in three cases. The diagnosis of thrombosis of the AVG was always made at operation or by histological examination. The radiological appearance of a thrombosed aneurysm is typical.

Cerebral Angiography↗
PubMed1981

[Acquired aphasia in epileptic children--four cases with electrical infraclinic status epilepticus during sleep (author's transl)].

Four cases of acquired aphasia in epileptic children, associated with sub-continuous bitemporal paroxysmal activities (PA) during sleep, are described. Clinical improvement always followed the decrease or the unilateralization of PA. Persistence or aggravation of aphasia was observed when PA again became bitemporal and sub-continuous. Electroencephalographic characteristics of PA during sleep stages are described. Relationships of these cases with the 'sub-clinical electrical status epilepticus induced by sleep' and the physiopathology of aphasia are discussed.

Aphasia↗
PubMed1981

[The pattern of cerebrospinal fluid proteins in infants and children. Determination of normal values (author's transl)].

Cerebrospinal fluid (CSF) protein values were measured in 652 children between the ages of 1 day and 17 years, allowing the authors to define the dynamics of the blood-brain barrier under normal conditions and during inflammation of the nervous system. The ratio of CSF albumin/serum albumin (whose upper limit was 0.65 in the study) was the best sign of alteration of blood-brain barrier permeability. The CSF IgG level, whose upper limit was 0.85 (for serum IgG between 10 and 14 g/l) is the most useful criterion for detecting an intra-thecal synthesis of IgG. Six patterns of CSF proteins are defined on the basis of immunochemical and electrophoretic studies. The ratio of CSF albumin/serum albumin and the CSF IgG level must be compared to the electrophoretic pattern of CSF proteins in order to better characterize one aspect of the blood-brain barrier under normal and pathologic conditions of the central nervous system.

Adolescent↗