Search PubMed⌕ Search

Biomedical subjects

C Chiron

Publications and source records attributed to C Chiron.

90 records · Page 5Linked to original sources
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↗
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↗
PubMed1987

Hemimegalencephaly: MR imaging in five children.

Hemimegalencephaly is a rare brain malformation characterized by cerebral asymmetry and cortical dysplasia. Infants with the condition present with early seizures and severe encephalopathy. Five patients were studied with computed tomography and magnetic resonance (MR) imaging. MR imaging was the most efficient diagnostic method for this rare entity. It demonstrated brain hemispheric hypertrophy with lateral ventricle dilatation, abnormal gyral pattern, and a thick cortex on the enlarged side. The images correlate well with the known pathologic data.

Brain↗
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↗
PubMed1984

Lung function in children with hypersensitivity pneumonitis.

Pulmonary function tests were performed on 12 children with hypersensitivity pneumonitis (HP) aged from 4-15 years (10 with bird breeder's lung and 2 with farmer's lung). Lung volumes, lung mechanics (lung resistance, dynamic lung compliance (CLdyn], lung transfer factor for CO (TLCO), and blood gases were measured. Eleven children ceased to be exposed to the antigen, and the functional course was studied as a function of time after the cessation of exposure (CE). During the short term (less than 2 months after CE) initial hypoxemia was observed and CLdyn and TLCO were below normal. Two months after CE, blood gases were normal in most of the cases. A persistent hypoxemia appeared to be an unfavorable prognostic factor. CLdyn was normal by the eighth month after CE, while TLCO improved more slowly and remained significantly decreased in one case. In our series the children under 10 years had less functional abnormalities and normalized more rapidly than the older children. The one child without CE, had major functional abnormalities.

Adolescent↗
PubMedDate not supplied

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↗
PubMedDate not supplied

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↗
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

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

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

[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↗