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Biomedical subjects

C Dravet

Publications and source records attributed to C Dravet.

At least 73 records · Page 4Linked to original sources
PubMed1983

Benzodiazepines: efficacy in status epilepticus.

Both our personal experience and the findings of others indicate that the benzodiazepines (a) are the drugs of first choice for control of status epilepticus occurring in patients with primary generalized epilepsy (90%-100% effective) or control of hemiclonic convulsions in children without brain lesions, (b) are effective in approximately 60% of cases of status epilepticus occurring in partial epilepsy, (c) are effective in only 15% to 59% of cases of tonic status or various types of absence status occurring in secondary generalized epilepsy (but no other drug is more effective), (d) are helpful in status epilepticus occurring in nonepileptic patients if there is no overt brain lesion (but give only temporary relief when status is the result of a severe organic brain lesion), and (e) are generally safe drugs.

Adult↗
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

[Spinocerebellar degeneration, optic atrophy, epilepsy, myoclonus and mitochondrial myopathy: a case report (author's transl)].

A 23-year-old man presented with a history characterized by a myoclonic syndrome developing over a period of seven years. Predominant symptoms were intention and activity myoclonus, generalized epileptic seizures occurring infrequently from the age of 20, a slowly progressive cerebellar syndrome first apparent at 19 years, and the sudden onset of loss of visual acuity at 19, which then partially regressed; optic atrophy and clinical and campimetric signs were suggestive of Leber's disease. Intellectual ability was not affected. E.E.G. records showed generalized spike-waves with photosensitivity, progressive reduction in basal rhythm, and sleep organization disturbances with focal abnormalities. Obvious clinical signs of muscle disease were lacking but muscle biopsy confirmed the presence of a mitochondrial myopathy (ragged-red fibers). An indefinite history of familial neurological disease was obtained. Diagnosis was established as myoclonic cerebellar dyssynergy with spastic hereditary ataxia and Leber's disease. Their association with a mitochondrial myopathy has been previously reported by Tsairis et al, Fukuhara et al, Fitzimons et al (familial case), and Niedermeyer et al (sporadic case). In spite of the non-specific nature of associated mitochondrial abnormalities, all these cases would appear to correspond to a single nosological entity.

Adult↗
PubMed1981

[The partial epilepsies in childhood--evolution and prognosis factors (author's transl)].

The authors have studied 119 cases of partial epilepsies, selected from the patients treated in the Centre Saint-Paul between 1966 and 1970 according to the following criteria: seizures beginning before the age of 12; electro-clinical evolution known about for at least 5 years (mean duration: 13 years 3 months). The cases are separated in 4 groups according to the severity of epilepsy: (1) complete recovery and withdrawal of treatment; (2) no seizure with treatment; (3) persisting seizures with treatment; (4) frequent and severe seizures with treatment. The severe epilepsies are characterized by an early commencement of fits (before 3 years), associated symptoms of encephalopathy, lack of familial antecedents, association of several types of seizures, or the existence of a particular critical semiology (either complex partial motor seizures, or succession of simple motor and complex semiology, sudden fall, or reflex triggering). However, it is also necessary to consider the syndromic grouping of signs which is of greater value than a simple analysis of benignity or malignancy factors.

Age Factors↗
PubMed1981

[The benign myoclonic epilepsy of infancy (author's transl)].

The authors report observations on 7 children who have shown an early epilepsy characterized only by brief myoclonic fits, with generalized spikes and waves on the EEG. This type of epilepsy had a favorable evolution in all cases under treatment (usually VPA). In 2 cases, rare grand mal seizures appeared between 9 and 12 years. The authors discuss the situation of this epilepsy among all the myoclonic childhood epilepsies reported in the literature. They conclude that it is an early type of primary generalized epilepsy.

Age Factors↗
PubMed1980

Carbamazepine intoxication due to triacetyloleandomycin administration in epileptic patients.

In 17 epileptics receiving carbamazepine (CBZ) alone or in combination with other anticonvulsant drugs, administration of triacetyloleandomycin (Tri A) led to an acute and unexpected intoxication (drowsiness, nausea, vomiting, and dizziness). Similar symptoms occurred again in 3 patients after Tri A was administered a second time. The same toxic manifestations were observed in two patients receiving CBZ and erythromycin. A rapid increase in plasma levels of CBZ occurred after institution of Tri A therapy in 6 patients, the CBZ levels quickly returning to normal after withdrawal of Tri A. Thus, it is suggested that the observed intoxication is due to the simultaneous administration of CBZ and Tri A (or erythromycin). The possible role of hepatic dysfunction in this syndrome of intoxication is discussed. Furthermore, the intoxication may be at least partially related to serum electrolyte disturbances, as suggested by one case in which obvious signs of water intoxication were detected. The severity and frequency of intoxication should lead to proscribing Tri A or other macrolide antibiotics in patients receiving CBZ.

Adolescent↗
PubMed1980

[Paroxysmal dyskinesia during treatment with diphenylhydantoin].

Paroxystic-dyskinesias occurred in ten patients with encephalopathy and severe epilepsy associated with intellectual deficiences, during treatment of the epilepsy with diphenylhydantoin (DPH). The abnormal movements were usually paroxystic in nature and were mainly athetosic or choreic in nature affecting more particularly the facial region. None of the patients showed neurological signs during the attacks, which could have suggested an overdose of DPH, and the EEG showed no alterations. Blood levels of DPH were measured during the attacks in 9 of the patients and were within therapeutic limits in 6 cases. They were at toxic levels in the other 3 cases. All patients were receiving multiple therapy. The authors compare their findings with those in the published literature describing 46 such cases. Dyskinesias were usually observed in patients with encephalopaty. Of the 40 observations reported, blood levels of DPH were measured in 37 patients, and were found to be within normal therapeutic levels in 13 cases and at toxic levels in the other 24 patients. Clinical signs of an overdose were present in 26 cases. In all the cases reported by the authors, and in 28 cases described in published reports, the dyskinesias appeared when therapy was changed. Abnormal movements disappeared in 3 patients after an intravenous injection of diazepam. Experimental and biological arguments exist in favour of an action of DPH on the metabolism of cerebral mediators. The authors emphasize the need to investigate these dyskinetic manifestations which can be thought to be epileptic manifestations or signs of progressive encephalopathy.

Adult↗
PubMed1978

[Benign focal epilepsy and "electrical status epilepticus" during sleep (author's transl)].

The authors describe the electro-clinical state of four children having a type of epilepsy clinically characterized by rare partial motor seizures and frequent absences. From E.E.G. point of view they had focal (mainly frontal) and diffuse abnormalities. Such diffuse abnormalities became continuous during slow sleep, thus realizing an electrical status epilepticus. During such status partial subclinical seizures were recorded. At the onset such cases have electroclinical features resembling those observed in the form of epilepsy so-called "benign partial epilepsy of children with rolandic or mid-temporal foci". All cases, however, have behavioural problems (instability, desorientation) and decreased school performances. The epilepsy evolution, however, is favourable and such form should consequently be distinguished from the Lennox-Gastaut Syndrome.

Child↗
PubMed1978

Computerized tomography in the study of West's syndrome.

37 children presenting with West's syndrome were studied by computerized tomography scanning. An abnormality consisting of diffuse cerebral atrophy, predominating in the fronto-temporal region, was demonstrated in 30 cases. The abnormality was sometimes associated with calcification (tuberous sclerosis) or malformation (agenesis of the corpus callosum). The aetiology of the atrophy and its relationship to clinical and electroencephalographic signs is discussed. Computerized tomography scanning is recommended for children with West's syndrome in order to distinguish between primary and secondary cases, and for greater prognostic accuracy.

Adolescent↗
PubMed1977

[Partial epilepsy with complex symptoms and atrophic occipito-parietal lesions. Value of tachoencephalographic examination].

Eleven of the first 500 epileptics examined by axial tomography presented pseudo-porencephalic dilation (cavitary atrophy) of an occipital horn, associated or not with more extensive ventricular dilation. This lesion was found in 2.2% of all the epileptics and in 5% of the epileptics under the age of 20 in this group, a high incidence when compared with that of lesions of the same type located elsewhere. Clinical investigation and E.E.G. carried out on these 11 children showed that they were suffering from either hemiphegia or hemiparesis (8 out of 11 cases) and hemianopsia (7 out of 11 cases), and had serious epilepsy with partial fits (6 cases), usual temporal lobe epilepsy (5 cases), generalized secondary epilepsy (3 cases) or epilepsy defying classification (2 cases). These cases are compared with those recently published by Remillard, Ethier and Anderman. These authors consider that these cerebral lesions are the result of peri-natal thrombosis of the posterior cerebral artery. But in our group of cases, angiographic results do not appear to justify this claim.

Adolescent↗
PubMed1977

[Tomodensitometric study of cerebral accidents causing acute hemiplegia in children].

Computerized Axial Tomography (C.A.T.) easily distinguishes between the two types of cerebral accident responsible for the two major categories of acute hemiplegia in childhood. 1) In hemiplegias which develop in association with hemiclonic status epilepticus (H. H. and H. H. E. syndromes), in the majority of cases there is an appearance of cortico-sub-cortical atrophy involving the whole hemisphere contralateral to the hemiplegia. This atrophy develops following oedema of the hemisphere which accompanies the initial status epilepticus and which is clearly shown by a very early T.A.C. 2) In congenital or acquired hemiplegias not associated with status epilepticus there is, in most cases, an appearance of cerebral infarction or, very rarely, haemorrhage. It is thus possible, from a physiopathogenic standpoint, to draw a clear distinction between these two major forms of acute infantile hemiplegia. The first (H. H. and H. H. E. syndromes) usually result from hemispheric atrophy which develops in association with a unilateral or predominantly lateral episode of status epilepticus, whilst the second group are usually the result of cerebral infarction.

Adolescent↗