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Biomedical subjects

O Dulac

Publications and source records attributed to O Dulac.

At least 199 records · Page 11Linked to original sources

[Optic neuritis in children. Clinical aspects and evolution in 14 patients (author's transl)].

Fourteen children presenting with optic neuritis are reported. Bilateral ocular lesions and papilledema were present in 80% of cases. Biological investigations did not show any local production of IgG or anti-measles antibodies, contrary to what is observed in adults. CAT scan most always rules out a tumoral compression that could be responsible for a sudden decrease in visual acuity. Prognostic factors helpful in predicting evolution towards multiple sclerosis are discussed.

Adrenal Cortex Hormones↗

Pharmacokinetics of carbamazepine in the neonate and in the child.

1. Pharmacokinetic of carbamazepine were made in 7 new-borns and in 5 children. They were hospitalized for epilepsy and were receiving drugs such as phenobarbital alone or in association with other antiepileptic drugs, but not with carbamazepine. 2. The drug was given by oral route with a mean dose of 17.2 mg.kg-1. 3. The determination of carbamazepine concentration in serum was made by gas liquid chromatography on a 50 microliter sample. 4. A one compartment body model was used to determine the pharmacokinetic constants with first order rate constants for absorption and elimination. 5. Absorption was generally delayed by about half an hour, the maximum concentrations ranging from 3.14 to 10 microgram.ml-1 at 2 and 9 hr after administration. The mean half-life for absorption was 1.42 +/- 0.34 hr. The mean half-life for elimination was 8.76 +/- 0.85 hr. The half-life for elimination was much shorter than those already described even in multiple dosing epileptic adult patients. The pharmacokinetic parameters were used to predict blood levels in chronic treatment in 3 children. The predicted steady state concentrations disagreed with the concentrations measured.

Adult↗

[Severe viral hepatitis in childhood: course and prognosis].

In 22 children with severe acute viral hepatitis, the course of the disease followed 3 patterns: 8 children completely yielded (regenerative hepatitis); 8 died during the first three weeks of evolution (aregenerative hepatitis); 8 had a prolonged evolution (hyporegenerative hepatitis). In the latter group, 6 patients died after an average survival time of 55 days and 2 patients rapidly developped a cirrhosis. This type of evolution was characterized by persistence of liver failure manifestations, in spite of liver regeneration, as indicated by increased levels of alpha-foetoprotein and presence of pseudo-acini, giantcells and nodules at histological examination. During the second week of evolution, the size of liver, levels of clotting factors VII +X and alpha-foetoprotein concentrations seem to constitute important prognostic factors.

Child↗

[Epidural spinal hematoma after lumbar puncture].

Three days after a lumbar puncture in a 7 month old child with pyogenic meningitis and without any blood clotting defect, appeared symptoms of spinal cord compression. Myelography led to surgery, 5 days after the puncture. At operation, an epidural hematoma, from L1 to L5 was removed. The exceptional character of this accident and its cause are discussed.

Hematoma, Epidural, Cranial↗

Early myoclonic epileptic encephalopathy (E.M.E.E.).

The authors describe the electroclinical aspects and evolution of nine cases of myoclonic epileptic encephalopathy which began between two days and ten weeks of life. At onset it is associated with: myoclonic jerks, partial fits and periodic paroxysmal EEG abnormalities. Repeated spasms coexisting with partial fits and 'suppression-bursts' (both appearing later) complete the electroclinical picture. The neurological status (initially normal) progressively deteriorates leading within a few months to a decerebrate posture with opisthotonos. In spite of thorough neuroradiological, biochemical, cytological, metabolic, and ultrastructural investigations, the etiology remained unknown. However, the electroclinical and evolutive patterns are similar to those of some metabolic diseases (Polyodystrophy, Non-Ketotic Hyperglycinemia, etc.). All these observations display a homogeneous electroclinical pattern for which the authors propose the name of Early Myoclonic Epileptic Encephalopathy. This type deserves to be classified as a particular electroclinical entity among the epileptic encephalopathies of the first year of life; since its course is regularly downhill in all cases there may be a familial recurrence due to the possibility of a metabolic etiology.

Age Factors↗

[Clinical and electroencephalographaic aspects of seizures due to electrolyte and water disturbances in infants (author's transl)].

Clinical seizures, or subclinical EEG discharges, were observed in sixteen new-born babies or infants under one year of age admitted to hospital. Clinical observation and initial biological tests showed that: --seizures occurring during rehydration of infants with salt retention are not associated with severe neurological lesions,--in dehydrated babies, a dissociation between electroencephalographic and clinical manifestations (electrical discharges without clinical signs or vice versa) and large abnormalities in the intercritical tracing are bad prognostic elements;--in new-born babies with severe dehydration and meningeal hemorrhage, the occurence of clinical and electroencephalographic seizures does not imply that there will be an unfavourable evolution. The authors discuss the physiopathological correlations arising from the study of this series.

Child, Preschool↗

[Affective symptoms during attacks of epilepsy in children (author's transl)].

The authors present the results of an electroclinical study in 20 children (16 girls and 4 boys) having epileptic attacks with affective symptoms of a terrifying type. These were often interpreted (6/20) initially as nocturnal terrors or acute anxiety attacks. In 8 cases (40%) there was a previous family history of epilepsy. The age of onset was between 2 and 10 years. The attacks occurred several times during the 24 hours, both day and night, but were controlled rapidly and permanently (4 children over 15 years of age have had no further attacks 4 years after discontinuing treatment), and neurological and intellectual development was normal. This benign progression is correlated with a homogeneous critical and intercritical electroclinical picture enabling an early favorable prognosis to be made. The classification of this type of epilepsy, and its analogy with epilepsy with rolandic spikes is discussed.

Adolescent↗

Infantile spasms in Down syndrome--effects of delayed anticonvulsive treatment.

To investigate the impact of treatment lag in infantile spasms (IS) on treatment response, occurrence of later epilepsy, and long-term cognition and behavior in patients with one single etiological entity, we examined 18 patients with Down syndrome (DS) and earlier IS retrospectively (follow-up period of 32-180 months with a mean of 85.1 months), and determined their history and present condition, in terms of previously mentioned items. There was a statistically significant correlation between treatment lag and lag to cessation of spasms (R=0.55, P=0.02), developmental quotient (DQ) (R=-0.75, P=0.003), and score of autistic features (AF) (R=0.57, P=0.04). Moreover we found that the later the response to treatment of IS, the lower was the DQ (R=-0.86, P=0.001) and the higher was the score of autistic features (R=0.5, P=0.06). A long duration of spasms also determined a low DQ (R=-0.93, P<0.0001) and a high score of autistic features (R=0.66, P<0.01). All patients with persistent epilepsy (n=5) had had a treatment lag of over 2 months. Conversely, for all children treated within 2 months (n=8) spasms ceased within 3 months of treatment and none of them had later epilepsy. This group of patients with a treatment lag of less than 2 months had earlier treatment response (P=0.002), higher DQ (P=0.004) and lower score of autistic features (P=0.006). The data stress the importance of a short treatment lag in view of mental development and prevention of later epilepsy and autistic features, and raise the question of antiepileptogenic effect in this specific condition.

Adolescent↗

Vigabatrin in the treatment of childhood epilepsies: a single-blind placebo-controlled study.

Sixty-one pediatric patients (12-229 months of age) with refractory epilepsy were treated with vigabatrin [gamma-vinyl GABA (GVG)] in a 16-week, single-blind, add-on, placebo-controlled trial. Twenty-three patients (38%) showed a reduction of more than 50% in seizure frequency; 12 patients (20%) experienced a seizure increase; and the remaining 26 did not show significant differences between placebo and GVG treatment. Among the 216 patients who entered the long-term phase after having experienced more than 50% decrease in seizure frequency, 14 continued with the same degree of improvement after 2-11 months of follow-up (mean 7.7). GVG was particularly efficient in cryptogenic partial epilepsy. Conversely, nonprogressive myoclonic epilepsy tended to be aggravated. Agitation was the most commonly observed side effect, mainly at onset of therapy in mentally retarded patients, but was easily reversed by dose reduction. GVG is a promising drug in the treatment of refractory epilepsies of childhood.

4-Aminobutyrate Transaminase↗

Is there a partial benign epilepsy in infancy?

Benign idiopathic neonatal convulsions and partial benign childhood epilepsy, the most frequent types of childhood benign epilepsy, are both characterized by partial seizures. This study was performed to determine whether any kind of benign epilepsy with partial seizures occurs in the age range between these two syndromes. We identified 17 cases of benign epilepsy with partial seizures from a review of 442 records collected in a 5-year period in which seizures occurred between 8 days and 3 years of age. Six patients with onset before 6 weeks of life had clinical features characteristic of benign idiopathic neonatal convulsions, and appeared to be a delayed type of this syndrome. Eleven patients had clinical features characteristic of partial benign childhood epilepsy. In the age range between occurrence of these two recognized epileptic syndromes, we identified no case of partial epilepsy with favorable outcome.

Age Factors↗

Ictal characteristics of cryptogenic partial epilepsies in infancy.

Twenty-three patients with onset of partial seizures (PS) during the first 2 years of life were collected in a single neuropediatric center and ictal events were analyzed. All patients initially had normal developmental and neurologic examinations, negative etiologic investigations, and at least two nonfebrile PS of a single type. Mean follow-up from first seizure to the last contact with the patient was 51 months (SEM 8.17 months). Ictal semiology was characterized by three types of seizures: simple PS with motor signs, complex partial seizures (CPS) with impairment of consciousness at onset followed by motor manifestations, and CPS with motor activity at onset. Versive phenomena, oroalimentary automatisms, and vegetative manifestations were frequently associated. The topography of the ictal discharges and the elementary ictal events, suggested involvement of the temporal lobe and somatomotor area. The diagnosis of PS can be difficult during the first year of life since some patients beginning with generalized seizures may evolve into partial seizures. The prognosis is guarded with regard to seizure persistence, but the neurologic outcome is better as a whole than is reported for infancy-onset epilepsies.

Aging↗

Topographic comparative study of magnetic resonance imaging and electroencephalography in 34 children with tuberous sclerosis.

A series of 34 children with confirmed tuberous sclerosis (TS) were studied prospectively by both EEG and magnetic resonance imaging (MRI) at ages ranging from 5 months to 18 years. Size and topography of the cortical tubers were analyzed on axial and coronal views, in T2 sequences, and the large tubers greater than 10 mm were studied. In addition, EEG follow-up data were reviewed retrospectively. Twenty-six patients (76%) had both MRI large cortical tubers and EEG foci, 3 had normal EEG, 2 had normal MRI, and the remaining 3 had only small tubers. The number of large tubers was significantly related to EEG foci. A topographic MRI/EEG correspondence was observed for at least one tuber in 25 of the 26 patients, and correspondence was complete in 10 patients. Large cortical tubers without corresponding EEG foci were observed in 11 patients; these tubers mainly involved the frontal regions and were found before the patients were 2 years old. EEG foci without corresponding tubers were observed in 4 patients. In addition, secondary bilateral synchrony was preferentially observed in patients with frontal lesions and after the age of 2 years. These data confirm that the cortical tubers are epileptogenic and that their expression may be influenced by regional cortical maturation.

Adolescent↗