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Biomedical subjects

O Dulac

Publications and source records attributed to O Dulac.

At least 217 records · Page 12Linked to original sources
PubMedDate not supplied

Twenty-four-hour ambulatory EEG monitoring in infantile spasms.

Twenty-four-hour ambulatory EEG (AEEG) recordings were performed in 74 infants with West Syndrome (WS) who had not received corticosteroids before the recording. EEG analysis was performed visually for interictal background activity as well as for ictal events: spasms (isolated or in clusters) and other seizures either generalized or partial. Six hundred fifty-four seizures were recorded in 67 patients. Partial seizures (PS) were noted in 31 infants (51% of symptomatic WS cases, 33% of cryptogenic WS cases). In 14 patients, PS were immediately followed by a cluster of spasms consisting of a single ictal event. Patients with PS had an asymmetrical interictal background activity in 85% of cases, with no return to hypsarrhythmia between spasms in a given cluster. AEEG is a reliable method to detect and analyze ictal events in infants with WS. In this population, patients with unfavorable outcome of both epilepsy and psychomotor development have PS. Therefore, the existence of PS may contribute to etiologic diagnosis and prognostic evaluation.

Child, Preschool↗
PubMedDate not supplied

Visual inattention in West syndrome: a neuropsychological and neurofunctional imaging study.

Visual behavior is frequently impaired at onset of West syndrome (WS). We studied the neuropsychological outcome of eight children who had cryptogenic WS and moderate to severe visual impairment at the onset of epilepsy. At the last examination, a regional cerebral blood flow study using SPECT (single photon emission computed tomography) was performed. The behavior abnormalities observed initially evolved to various defects of cognitive function. Three patients had severe mental retardation with autistic features. Two had marked speech disorders but one had global cognitive impairment. Three patients had specific visual-spatial deficits. SPECT showed perfusion defects involving the parieto-occipital areas in 6 of 8 patients. These abnormalities were restricted to parieto-occipital regions in the three patients with selective visual-spatial deficits, whereas they were associated with other perfusion defects in the three remaining patients. This study demonstrates that the visual inattention observed at the onset of WS is frequently associated with long-term cognitive and/or perfusion defects involving the parieto-occipital regions.

Attention↗
PubMedDate not supplied

Value of magnetic resonance imaging in West syndrome of unknown etiology.

Magnetic resonance imaging (MRI) studies of 46 patients with West syndrome (WS) of unknown etiology were reviewed retrospectively. The criteria for cryptogenic WS were met by 25 and 21 were considered symptomatic because other types of seizure or psychomotor retardation were apparent before spasm onset. Computed tomographic (CT) scans were normal in 38 patients and showed diffuse atrophy in eight symptomatic patients. In five patients, MRI was more informative than CT, demonstrating one case of delayed myelination and four cases of focal lesion. The focal lesion in 2 of these patients was similar on MRI consisting of poor gray-white matter demarcation in the parieto-occipitotemporal region. Surgical resection was performed in one because of intractable seizures, and neuropathological examination revealed cortical dysplasia. The remaining two cases with focal lesion had increased signal intensity on T2-weighted images in the posterior frontal cortex and in the temporal lobe, respectively. Our data indicate that MRI is useful in some cases of WS, especially in demonstrating focal corticosubcortical lesions not visible on CT scan.

Brain↗
PubMedDate not supplied

Study of regional cerebral blood flow in West syndrome.

Focal cortical disturbances are frequent sequelae in West syndrome (WS) even though it is a generalized epileptic syndrome. Functional neuroimaging was used to determine whether focal perfusion abnormalities exist at WS onset and change during evolution. We studied regional cerebral blood flow (rCBF) at different stages of WS. Mean CBF (mCBF) and rCBF were measured using SPECT (single photon emission computed tomography) and 133Xe in 13 WS patients: at onset (20 cases), just after steroids (17 cases), and after a mean follow-up of 2 years (26 cases). At WS onset, interictal mCBF was increased as the result of foci of hyper- and hypoperfusion, which were, respectively, mainly located in the frontal and posterior cortex. Just after steroid therapy, mCBF decreased without any focal predominance. During follow-up, hypoperfused foci remained unchanged whereas the frontal hyperperfused foci decreased after spasm control. Our results show that focal abnormalities are present at WS onset. Focal hypoactivity could reflect a cortical lesion responsible for WS and focal hyperactivity could play a role in the persistence of generalized epilepsy.

Cerebrovascular Circulation↗
PubMedDate not supplied

Neurofibromatosis type one and West syndrome: a relatively benign association.

Fifteen patients with neurofibromatosis type one (NF1) and West syndrome (WS) were studied. The evolution of WS was clearly different from that seen in the other neurocutaneous syndromes, especially tuberous sclerosis. Steroids were efficacious and spasms disappeared. There was no relapse after discontinuation of steroids and antiepileptic drugs were successfully stopped several years later. Mental outcome was also very good. The association of both conditions does not seem to be a coincidence. WS with NF1 seems remarkably benign and resembles idiopathic WS more than symptomatic WS.

Adrenocorticotropic Hormone↗
PubMedDate not supplied

Genetic predisposition to West syndrome.

To determine the recurrence risk of West syndrome (WS), we studied the familial antecedents of consecutively referred patients. Among siblings, there was an increased incidence of WS but not of febrile convulsions. Familial incidence of epilepsy was intermediate between the epileptic and nonepileptic control groups. When cases resulting from a genetically determined disease were excluded, incidence of epilepsy among siblings was similar to that in normal controls. Five of the 11 familial cases of WS were due to an identifiable cause: twin pregnancy, tuberous sclerosis, and recurrent maternal toxemia. In 4 of the remaining families, the clinical picture included spasms, erratic myoclonus, and postnatal microcephaly, suggestive of a previously unidentifiable progressive encephalopathy. Therefore, when identifiable familial diseases were excluded, the recurrence risk was < 1%.

Child, Preschool↗
PubMedDate not supplied

West syndrome due to perinatal insults.

Clinical, neuroradiological data and outcome of epilepsy of 32 patients with symptomatic infantile spasms caused by perinatal insults are reported. Neuroradiological investigations showed focal as well as diffuse cerebral lesions. Fifteen children had a porencephalic lesion, 12 had periventricular leukomalacia and five had diffuse bilateral cerebral atrophy, associated with status marmoratus in one child and bilateral ulegyria in another. Eight patients (25%) had epilepsy following West syndrome. In the group of 15 children with porencephalic cysts, the lesion was always unilateral, but topography and extension varied. Eight children had circumscribed cysts involving the rolandic or parieto-occipital regions, and the outcome of epilepsy was favorable. Seven patients had large lesions involving the frontal lobe, associated with unilateral hemispheric atrophy in four cases. These four children had an unfavorable epilepsy outcome. In the 12 patients with periventricular leukomalacia, all premature, eleven had no seizure relapse after the spasms; one of these children had epilepsy. In the five full-term patients with bilateral and diffuse cerebral lesions, three children had epilepsy at the last observation. In children with leukomalacia and in patients with localized porencephalic lesions the outcome of epilepsy appears to be better than in patients with diffuse cerebral lesions or in children with extensive porencephalic cysts, particularly those involving the frontal lobe.

Atrophy↗
PubMedDate not supplied

Predicting favorable outcome in idiopathic West syndrome.

Among 45 patients with cryptogenic West Syndrome (WS) we report 30 with a favorable outcome consisting of normal psychomotor development and cessation of epilepsy with at least 2 years follow-up (mean 4 years 7 months). These favorable patients could be recognized from onset by (a) absence of significant mental regression with a preserved visual function; (b) absence of focal interictal EEG abnormalities after intravenous diazepam; and (c) reappearance of hypsarrhythmia between consecutive spasms of a cluster. The latter criterion requires EEG recording of a series of spasms. The favorable outcome in these patients suggests that they had no cortical brain lesions. This new type of idiopathic epilepsy referred to as idiopathic West syndrome has important prognostic, therapeutic, and etiologic implications.

Diazepam↗
PubMedDate not supplied

Callosotomy in West syndrome suggests a cortical origin of hypsarrhythmia.

Hypsarrhythmia and spasms of West syndrome have been postulated by some investigators to have a brainstem origin. Cortical involvement is also postulated because of the frequent association with cortical lesions. Cortical discharges have been proposed to stimulate subcortical structures (i.e., brainstem) and hypsarrhythmia is a secondarily generalized phenomenon produced by the brainstem. Two children with cryptogenic West syndrome refractory for several years to steroids and conventional antiepileptic drugs underwent total callosotomy. Hypsarrhythmia that was bilateral before operation became unilateral after the procedure and spasms became asymmetrical, suggesting that a corticocortical pathway through the corpus callosum is important in the generalization of hypsarrhythmia.

Brain Stem↗
PubMedDate not supplied

Phenytoin monitoring in status epilepticus in infants and children.

Two successive protocols of phenytoin (PHT) plasma concentration monitoring were tested in 60 children with status epilepticus (SE). In each protocol, a loading dose of 15 mg/kg was injected and followed by three injections during the first 24 h. Clinical evaluation was performed at the end of the study by grouping patients into three classes according to seizure frequency during treatment: complete effect, partial effect, and no effect. In protocol 1, a complementary dose at the fourth hour was adjusted from individual plasma concentrations. Plasma concentrations at the 40th hour were within the therapeutic range in the 19 patients with complete effect (CE, mean 19 mg/L) and in the 5 patients with no effect (NE, mean 23 mg/L) whereas in the 11 patients with partial effect (PE), plasma concentrations were higher (mean 31 mg/L). In protocol 2, we added monitoring of the doses injected at the 16th and 24th hours to prevent the increase in PHT concentration noted in protocol 1. All patients but 1 were classified as either CE (13 patients) or NE (4 patients). In NE patients, average plasma concentration at the 40th hour (mean 10.5 mg/L) was lower than in CE patients (mean 15.7 mg/L). In both protocols, the NE patients were the youngest. In SE, PHT doses should be adjusted according to plasma concentrations to avoid overdosage and paradoxical inefficacy. Younger children had lower concentrations and appeared to respond less well than older children, but the etiology of SE may also play an important role.

Adolescent↗
PubMedDate not supplied

Lamotrigine in treatment of 120 children with epilepsy.

One hundred twenty children aged 10 months to 16 years 9 months were included in three studies with lamotrigine (LTG): a single-blind study (n = 60), a pharmacokinetic study (n = 23), and a compassionate group (n = 37). At 3 months, 11 patients had become seizure-free and 34 had > 50% decrease in seizure frequency. The best results involved absence epilepsy, Lennox-Gastaut syndrome (LGS), and other symptomatic generalized epilepsy. Forty-two patients were followed > 1 year, 22 for a mean of 2.2 years, and there was no significant increase in seizure frequency as compared with 3-month follow-up. Fourteen patients became seizure-free for > 6 months; all except 1 had generalized epilepsy. For 12 patients, treatment could be reduced to monotherapy, but for those with valproate (VPA) comedication LTG dosage had to be increased; 25% of patients with VPA monotherapy exhibited skin rash, appearing 3-18 days after starting LTG. For 4 patients, LTG could be reintroduced after VPA was withdrawn. Ten patients had ataxia and/or drowsiness and 2 had vomiting. For all other patients, tolerance was excellent.

Adolescent↗
PubMedDate not supplied

[Paraplegia complicating umbilical artery catheterization].

Two cases of definitive paraplegia following umbilical artery catheterization are reported. In both patients, the paraplegia can be attributed to a myelomalacia secondary to the thrombosis or embolization of the feeding arteries of the cord. The treatment by fibrinolytic agents was unsuccessful and the only therapeutic approach appears to be a preventive one. This prophylaxis would best be accomplished by limiting the indications and duration of umbilical artery catheterization.

Catheterization↗
PubMedDate not supplied

[Reversible frontal syndrome and epilepsy in an 8-year-old boy].

An 8 year-old patient with a frontal lobe syndrome associated with epilepsy is reported. The neuropsychological evaluation showed that the behavioural problems resulted from moderate but specific disorders of the executive system. Evolution was favorable with complete disappearance of the neuropsychological difficulties when the seizures were controlled. This dysfunction, similar to that observed in adults, may occur in the first decade and be functional in origin.

Brain Diseases↗
PubMedDate not supplied

[Radiculomyeloencephalitis caused by Angiostrongylus cantonensis in children].

A case of radiculoencephalomyelitis caused by central nervous system involvement by Angiostrongylus cantonensis is reported in a 14 month-old girl. Evolution was spontaneously favourable, which is not usual in severe types. Lesions concerned the nevraxe and especially the spinal cord and are due to the migration of the larvae, creating eosinophilic inflammatory reactions.

Angiostrongylus↗
PubMedDate not supplied

Menkes disease: a Golgi and electron microscopic study of the cerebellar cortex.

A neuropathological study of a case of Menkes disease is reported, illustrating the involvement of different types of neuronal cells. The cerebellum showed the most striking abnormalities: severe lack of internal granule cells. Purkinje cells with weeping willow pattern, numerous segmental enlargements of dendritic trunks and secondary branches, and presence of numerous eosinphilic spherical bodies in the molecular layer were the most conspicuous features. Using electron microscopy, the dendritic enlargements were observed to be made of both proliferated and enlarged mitochondria, and of saccules of smooth endoplasmic reticulum. The spheroid bodies in the molecular layer were mainly made of concentric lamellar structures which seemed to be proliferated smooth endoplasmic reticulum. The relationship between these morphological abnormalities and the metabolic disorder of Menkes disease is discussed.

Brain Diseases, Metabolic↗
PubMedDate not supplied

[Thrombosis of the superior longitudinal sinus in the treatment of lymphoma in children. 4 case reports].

The authors report four cases of children, beginning a treatment for a lymphoma; these children present neurological symptoms, which CT scan and digitalized intravenous angiography have shown to be connected to superior longitudinal sinus thrombosis. They are reexamining similar cases described in the literature, related to leukemias, lymphomas and other cancers, and describe their angiographic and CT scan appearance. The pathogeny of these complications is considered but is improven.

Adolescent↗