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Biomedical subjects

O Dulac

Publications and source records attributed to O Dulac.

At least 145 records · Page 8Linked to original sources
PubMed1989

Regional cerebral blood flow by SPECT imaging in Sturge-Weber disease: an aid for diagnosis.

Regional cerebral blood flow (rCBF) was studied using SPECT (single photon emission computed tomography) with 133-Xenon in 13 patients with confirmed Sturge-Weber disease, aged 9 months to 18 years. CT scan, performed at the same time, showed evident cerebral angioma in 10 but not in three. A marked hypoperfused area was found in all patients, ranging from -32% to -72% and of the same location as the CT signs. The hypoperfusion seems to result from post ictal phenomenon as well as from chronic ischaemia. SPECT imaging is therefore a sensitive method for visualising intracranial angioma in Sturge-Weber disease and it provides an aid for diagnosis when a CT scan is not reliable.

Adolescent↗
PubMed1989

Study of the cerebral blood flow in partial epilepsy of childhood using the SPECT method.

Cerebral functional imaging methods provide information on the location of the epileptic focus in partial epilepsy of adults. We report our experience of one of these methods, single photon emission computed tomography (SPECT), in epilepsy of children. SPECT enables the regional cerebral blood flow (rCBF) to be measured, after inhalation or injection of 133-Xenon, on 5 contiguous, 20 mm thick axial sections, with a 14 mm resolution and negligible brain irradiation. In Sturge-Weber syndrome (13 patients aged 9 months to 18 years) the rCBF was reduced in the same territory as CT abnormalities suggesting ischaemia of the brain tissue lying below the pial angioma; the SPECT image facilitated the diagnosis in 3 patients with atypical CT. In hemimegalencephaly (6 patients aged 1 month to 10 years) the rCBF was extremely low in the hypertrophic hemisphere and in 1 case the SPECT image was determinant in the decision to perform hemispherectomy. In partial epilepsy with normal CT and/or MRI (42 children aged 1 to 15 years) the rCBF was abnormal in 83% of the patients, and its abnormality was located in the same area as the EEG focus in three quarters of the cases. Between seizures, the rCBF was low in 3 out of 4 cases and abnormality decreased after the seizures had ceased (6 patients explored twice); it was high in 1 out of 4 cases. Thus, in children as in adults, cerebral functional imaging provides new data which contribute to the localization and follow-up of epileptic foci.

Adolescent↗
PubMed1989

[Hydrocortisone therapy of secondary generalized epilepsy in children].

Indications for glucocorticoids in the treatment of secondary generalized epilepsy after age one year is discussed. The present retrospective study showed that half of the children exhibited significant reduction of seizure frequency and 2/3 of them improved their cognitive abilities. Results were better in tonic and tonic-clonic seizures than in absences. The mean delay before improvement was 1 month for seizures and 3 months for cognitive abilities. Adverse events were mainly excessive weight gain and arterial hypertension. Both appeared within 2 months of initiating the treatment and disappeared when treatment was discontinued. The following factors seemed to influence the effect of glucocorticoids: the type of seizure, the nature of the epileptic syndrome, the delay between the onset of epilepsy and of treatment, and the beginning of treatment before 4 years of age. Glucocorticoids may be useful when conventional antiepileptic drugs have failed. The initial dose should be 5 mg/kg/day, reduced after 1 month of treatment. Glucocorticoids should be maintained at least for 6 months.

Age Factors↗
PubMed1989

Electroencephalographic aspects of hemimegalencephaly.

The authors retrospectively studied the electroencephalograms (EEGs) of 12 patients with hemimegalencephaly-a unilateral brain malformation which is often overlooked, despite having typical CT-scan and MRI aspects. They were characterized by three types of abnormal tracing, which correlated with prognosis: triphasic complexes of large amplitude were observed in patients with the earliest onset of seizures, and were associated with the most severe prognosis; unilateral, rhythmic 'alpha-like' activity was recorded in patients with seizures occurring after three months of age, and was associated with a relatively favourable outcome; asymmetrical suppression-bursts characterised by 'alpha-like' activity on the abnormal side were seen in patients with infantile spasms. The EEG pattern seems to make an important contribution to both diagnosis and prognosis.

Brain↗
PubMed1989

[Myasthenia and pregnancy: a clinical and immunologic study of 42 cases (21 neonatal myasthenia cases)].

Forty-two pregnancies in 39 myasthenic mothers were studied between 1978 and 1987. In 4 cases myasthenia gravis began during pregnancy and for 20 patients the clinical condition exacerbated in 15 cases, usually during the first 3 months, or during the postpartum. Except for 1 case, clinical exacerbation was controlled by anticholinesterase drug adjustment. Obstetrical problems were uncommon: abortion in 1 case and premature delivery in 4 cases. In 2 babies with severe fetal involvement polyhydramnios was present. Twenty-one babies had neonatal myasthenia gravis (NMG). In 17 cases, transient symptoms were present at delivery or shortly afterwards and full recovery occurred from a few days to 4 months. In 4 babies the clinical presentation was atypical: 1 showed a long evolution (15 months) and residual facial bilateral weakness, and three others presented fetal involvement (arthrogryposis). In these latter cases, presentation was severe with polyhydramnios, respiratory failure and long standing evolution in 2 cases (4 months and 1 year). Antiacetylcholine receptor antibodies (anti-AChR Ab) were found in all myasthenic babies and in 19 out of 20 asymptomatic babies. Maternal antibody titers were usually slightly higher than umbilical cord titers. There was a good correlation between maternal titer and onset or severity of disorder in baby. Among the 15 high titer mothers (greater than 60 nM), 13 had a myasthenic baby, 6 of them with serious disease. Conversely all low titer mothers (less than 10 nM) had a symptom-free baby. Therefore, anti-AChR Ab titration in the mother is predictive for NMG onset. Mother's myasthenia gravis severity and treatment were not correlated to the clinical condition of the newborns.

Acetylcholine↗
PubMed1988

Interferon gamma in acute and subacute encephalitis.

Intrathecal synthesis of interferon gamma was shown in 14 out of 16 samples of cerebrospinal fluid collected in the first days of disease in adults, children, and newborn infants with herpes encephalitis. This synthesis was concomitant with that of interferon alpha and was switched off when the specific antibodies in the central nervous system increased. No endogenous interferon gamma was detected in 11 serum samples or 13 samples of cerebrospinal fluid collected early in the course of the disease from patients with measles encephalitis and rubella encephalitis, or in serum and cerebrospinal fluid samples from seven patients with subacute sclerosing panencephalitis. In serum collected after the 10th day after the onset of neurological symptoms interferon gamma was present at low concentrations in only three out of 11 serum specimens from patients with measles encephalitis or rubella encephalitis. Interferon gamma was present in patients with acute herpes encephalitis and there was active virus replication, but it was not present in postinfectious encephalitis. Possibly the local production of specific antibodies masks the viral antigens and switches off the induction of interferons.

Acute Disease↗
PubMed1988

Agyria--pachygyria and Miller-Dieker syndrome: clinical, genetic and chromosome studies.

Twelve cases of lissencephaly are reported. A high resolution chromosome study was performed on each in order to detect small chromosomal anomalies, undetectable with routine techniques. Only one case was shown to have an unbalanced karyotype with a microdeletion of the short arm of chromosome 17 (del 17p). This child also had symptoms of the Miller-Dieker syndrome, consisting of lissencephaly, characteristic facies, pre- and post-natal growth retardation and other birth defects. As proposed by Dobyns, it seems justifiable to classify lissencephalies into four different groups, according to other clinical manifestations and results of chromosome studies.

Cerebral Cortex↗
PubMed1988

Immaturity of muscle fibers in the congenital form of myotonic dystrophy: its consequences and its origin.

Skeletal muscle maturation is impaired in children with congenital myotonic dystrophy. This immaturity is characterized at the light microscopy level by an abnormal presence of myotubes, small fascicles of muscle fibers, thin myofibers, and delayed muscle fiber type differentiation with a peripheral halo lacking mitochondrial oxidative enzyme activity. At an ultrastructural level, the characteristics are a paucity of myofibrils with a peripheral rim devoid of mitochondria and myofibrils in the fibers. In time the muscle is able to gain a certain degree of maturity as shown in one of our cases who had two successive muscle biopsies. The muscle, however, never becomes normal but retains discrepancies in fiber size and fiber type distribution and shows some fiber necrosis. Maturation of the motoneurons is normal, which may explain necrosis of immature muscle fibers. In an experimental study carried out to look for evidence of a circulatory factor in mothers of children with congenital myotonic dystrophy, it was found that sera from these mothers administered intra-peritoneally to newborn rats does in fact impair muscle maturation, whereas rats injected similarly with sera from control women showed normal muscle maturation.

Animals↗
PubMed1988

[Focal lesions in infantile spasms].

Among 174 cases of infantile spasms examined by CT-scan between January 80 and December 85, 17 (9.8%) had a focal lesion without microcephaly. It consisted of an angioma (1 case), a cortical dysplasia (1 case) and porencephalic lesions (15 cases). In 11 cases with porencephaly, an acute event had occurred between 28 weeks of gestation and 3 months of life. In 7 cases, the lesion extended to the major part of one hemisphere. In the 10 others, it was localized either to the rolandic or to the temporo-occipital regions. In all the cases, the lesion concerned at least one of these areas. It involved the cortex in 15 cases but in the 2 others it seemed to only concern the white matter. The lesion was on the left in 14 cases. After steroids, the epilepsy recurred in 6 cases of which 5 involved the frontal lobe (P less than 0.05). This study seems to show that it is mainly the rolandic and the temporo-occipital areas that favour the occurrence of infantile spasms, whereas extension to the frontal area is responsible of a later partial epilepsy.

Brain↗
PubMed1988

[Myoclonic epilepsy with non-progressive encephalopathy].

We report 6 cases of particular type of myoclonic epilepsy with non-progressive encephalopathy. It consists of a syndrome characterized by an onset of seizures in the first year of life, frequent myoclonic status, generalized spikes and waves on EEG and an unfavourable outcome with encephalopathy. At the beginning, the diagnosis is difficult, the symptomatology later suggests a progressive encephalopathy. In the present study, a detailed analysis of the early electroencephalographic aspects and of the arguments in favour of a non-progressive encephalopathy is proposed. Hypothesis of perinatal vascular lesions mainly involving the central areas is forwarded.

Adult↗
PubMed1988

Kojewnikow's Epilepsia Partialis Continua: two cases associated with striatal necrosis.

The authors report two cases of children suffering from Epilepsia Partialis Continua (EPC). The first case concerned a boy primarily affected by abdominal neuroblastoma and secondarily by bilateral EPC; "pallidal posture" was the prominent clinical feature. An acute measles encephalitis was diagnosed and the CT scan showed necrosis of the putamina. The second case concerned a girl suffering from increased intracranial pressure due to suprasellar craniopharyngioma. Seven days after intervention, Diabetes Insipidus and EPC appeared. Enlargement of rolandic and sylvian spaces and lacunar necrosis of the putamen on the left side were also evident on the CT scan. The authors emphasize the significance of occasional metabolic disturbances, especially natremia, in the development of EPC.

Child↗
PubMed1988

Neonatal myasthenia gravis: a new clinical and immunologic appraisal on 30 cases.

Anti-acetylcholine receptor (AChR) antibody titers, toxin binding blocking antibody, functional activity of serum on rat myotube cultures, IgG subclasses, and clinical data were studied in relation to the onset of neonatal myasthenia gravis (NMG) in 30 children of myasthenic mothers. Fourteen had NMG, including 4 atypical cases. Anti-AChR antibody titer was the best indication of NMG onset. NMG in a previous baby was also predictive. Pattern of IgG subclasses, presence of toxin-binding blocking antibodies, and serum functional activity were less predictive, but cast light on the mechanism of anti-AChR antibody pathogenicity.

Animals↗
PubMed1987

[Severe neonatal myasthenia with arthrogryposis].

Two children born to myasthenic mothers presented at birth with severe arthrogryposis, hypotonia and respiratory distress which followed a favourable course. A family history in one of these two cases and in another case reported in the literature raises the problem of a possible recurrence.

Adult↗
PubMed1987

Cerebellar hemispheric agenesis.

A case report is reported of bilateral cerebellar hemispheric agenesis which was associated with secondary degeneration of cerebellofugal and cerebellopetal tracts. Somatotopic correlations between the cerebellar and the medullary olive lesions were obvious: preserved dorsal accessory olives pattern correlated with the spared vermis and normal medial accessory olives with the spared flocculi. Cerebellopetal degeneration was more difficult to analyse. The relation of cerebellar agenesis with basal ganglia abnormalities and microcephaly is discussed.

Brain↗
PubMed1987

[Cerebral blood flow in idiopathic West syndrome: correlation with electroencephalographic findings].

Seventeen patients with idiopathic West syndrome and normal CTscan underwent a SPECT investigation. It is possible to perform this investigation in infants and children; the technical and methodological aspects are analysed. In benign infantile epileptic spasms, it showed no significant abnormality. In all the other cases, areas of hypovascularisation were observed. They predominated in the parieto-temporo-occipital areas both in the acute phase and several months or years later, and they correlated with the topography of predominating EEG abnormalities.

Amphetamines↗
PubMed1987

[Ambulatory 24-hour EEG recording in epileptic infantile spasms].

Twenty-four h cassette recording (MEDILOG 9000) where obtained in 22 untreated infants (23 records) with a West syndrome (idiopathic: n = 12, symptomatic: n = 10). All these infants had had a 3 h polygraphic recording. Tracings were visually analyzed for sleep stages. Electro-clinical seizures and infraclinical discharges were listed according to their type and the stage of vigilance. Two hundred twenty-eight critical events were recorded in 22 cassette recordings: isolated spasms, clusters of spasms, partial or generalized discharges followed or not by clusters of spasms. Sixty per cent of these fits occurred during wakefulness, 27% during non REM sleep, 13% during awakening and none during REM sleep. Partial discharges, followed or not by clusters of spasms, were only present in cases with unfavourable outcome. In benign spasms the hypsarrhythmic pattern was present between spasms in each cluster, but not in cases with unfavourable outcome. Compared with 3 h polygraphic recordings, 24 h cassette recordings gave supplementary data on the presence, type and timing of seizures in 16 of out 22 cases.

Ambulatory Care↗