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Biomedical subjects

M Arthuis

Publications and source records attributed to M Arthuis.

At least 37 records · Page 2Linked to original sources
PubMed1983

[Monotherapy with clobazam in epilepsies in children].

Twenty-five epileptic children were treated with clobazam alone for periods of 10 days to 36 months (mean = 9 months and 16 months for children who responded well). Satisfactory results were obtained in 11 patients and marked improvement with a 75% decrease in the initial frequency of seizures in 6 patients. The seizures recurred after a few weeks in 3 other patients. This preliminary study shows that clobazam is well tolerated and remarkably effective, especially in benign partial epilepsy, even in carbamazepine-resistant cases.

Adolescent↗
PubMed1983

[Electroencephalographic aspects of classic agyria-pachygyria].

Four infants had agyria confirmed by CT scan. All were mentally retarded, microcephalic, 3 of them having characteristic facial dysmorphy and 2 infantile spasms. EEG was characterized by very high amplitude rhythms in the alpha range, associated with delta waves and with infrequent spikes. The tracing was poorly modified by sleep. Drugs reduced its amplitude and rapid rhythms appeared with benzodiazepines. The authors point to the usefulness of the EEG in suggesting diagnosis before the CT scan, as was the case in 2 patients. They point to the urgent need of studying infantile epilepsies according to etiology, and not only to seizure types.

Cerebral Cortex↗
PubMed1983

[Hypothalamic hamartomas and laughing seizures].

The authors report 7 new cases of children with laughing seizures associated with hamartoma of the hypothalamus. Precocious puberty is present in 4 cases and seizures of another type (clonic or tonic) in 5 cases. The diagnosis is confirmed by histology (2 cases) or by CT scan (5 cases). The interictal EEG abnormalities are detailed as well as those found during the laughing seizures which were registered (3 cases). The literature was reviewed and 18 cases were found with posterior hypothalamic lesions and laughing seizures: they have been studied and compared with ours. The role of hamartoma is discussed in the production of these seizures, either isolated laughing seizures or else laughing seizures accompanied with other symptoms.

Adolescent↗
PubMed1983

[Apparent deafness as a manifestation of an aphasia-epilepsy syndrome].

Seven children with clinically evident deafness not confirmed by objective audiometric tests, and an acquired language deficiency, presented marked paroxysmal anomalies on electroencephalogram tracings. The aphasia-epilepsy syndrome can only be recognized by routine electroencephalograms, conducted in such cases even when a history of epileptic attacks is not abtained.

Aphasia↗
PubMed1983

[Monotherapy with carbamazepine in childhood epilepsies].

68 epileptic children were treated with carbamazepine (CBZ) monotherapy for a mean period of 10 months. The seizures disappeared in 43% and decreased in another 26.5% of cases. The treatment was particularly efficient in partial benign epilepsies (disappearance: 64.7%, reduction: 29.4%) and in partial unclassifiable epilepsies (disappearance: 50%, reduction: 25%). It is sometimes useful to achieve blood levels over 4 micrograms/ml; but over 8 micrograms/ml the frequency of side-effects becomes greater than improvement of fit frequency. The dose-concentration relation is well correlated to age, so that blood level measurements do not need to be systematically performed. In order to obtain mean blood levels of 7 micrograms/ml 3 to 4 hours after morning intake, mean daily dose should range from: 20 mg/kg/24 h before 5 years to 10 mg/kg/24 h after 10 years.

Adolescent↗
PubMed1983

[Nosological aspects of epilepsia partialis continua in children].

Among 26 patients suffering from Epilepsia Partialis Continua, 2 major groups were observed. The first, resulting from a fixed lesion of the rolandic area, showed electro-clinical correlation of seizures; the latter disappeared during sleep; clinical and radiological follow-up failed to disclose any worsening of the cerebral lesion. The second group was characterized by progressive mental and motor deterioration, lack of electro-clinical correlation of fits, persistence of the latter during sleep and frank increase of cerebral atrophy observed on serial neuroradiological examinations. This easily recognized group seems to result from a progressive inflammatory disease of unknown cause.

Child↗
PubMed1983

[Herpetic encephalitis in infants and children. Methods of diagnosis].

Thirteen infants and 2 children with Herpes simplex encephalitis are reported and the authors emphasize the diagnostic value of several investigations: the neurological examination (fits followed by early motor deficit on the same side and coma), the EEG (periodicity and asymmetry of the trace), the CT scan (hypodensity in the frontotemporal areas), the level of the Interferon alpha in blood and cerebrospinal fluid, the electrophoretic pattern of cerebrospinal fluid proteins and the comparative study of cerebrospinal fluid/serum antibodies towards several viral antigens.

Adolescent↗
PubMed1983

[Hypothalamic hamartoma and gelastic crises. Apropos of 7 cases].

Seven cases of hypothalamic hamartomas with gelastic seizures are reported. A precocious puberty was found in 4 cases. The normal neurologic examination and lack of sign of intracranial hypertension were in contrast with the severity of the epileptic seizures, of the mental impairment and of the behavioral disorders. The fact that the presenting symptom may be gelastic seizures is stressed. CT scan is the best means to assess the diagnosis and to follow the evolution of these tumors. Except for the management of the precocious puberty, the treatment is disappointing and neurosurgical indications are quite exceptional.

Adolescent↗
PubMed1983

[Electroclinical and developmental aspects of epilepsy in the aphasia-epilepsy syndrome].

76 children are studied--66 from the literature and 10 from a personal series--who developed epileptic seizures, paroxysmal EEG abnormalities and language deterioration after a period of normal development of language. The clinical features of the fits, the age at which the seizures appear and disappear, the age of the EEG normalization and the normal clinical and radiological examinations were reminiscent of partial benign epilepsies of childhood. Shortly after the language deteriorates, the awake EEG exhibits slow spikes that are diffuse or predominate in the temporal areas, sometimes asymmetrically. During sleep, the paroxysmal abnormalities are clearly bilateral and continuous or subcontinuous. Spontaneously or under medications, the intensity of the paroxysmal abnormalities decreases before clinical improvement occurs. In comparison with the mutism resulting from acquired hearing loss, it is suggested that the language disturbances in these children result from a functional disorganization of the language centers due to the important intercritical EEG abnormalities. In some children, these functional disturbances could result from a benign epilepsy. This view leads to discuss the prognosis and the therapeutic possibilities.

Anticonvulsants↗
PubMed1982

[Adrenoleukodystrophy in children. Apropos of 20 cases].

Adrenoleukodystrophy (ALD) is a sex-linked recessive metabolic encephalopathy usually beginning in childhood. This series of 20 cases showed the following associated symptoms: mental deterioration (20 cases), behavior modification (19 cases), decreased visual acuity (17 cases), deafness (13 cases) and seizures (7 cases). Adrenal insufficiency often consisted only of melanoderma (13 cases). Diagnosis was assessed by characteristic CT-scan findings (8 of 13 cases), adrenocortical insufficiency partial glucocorticoid (19 of 19 investigated cases) or global, mineralo- and involving both aldosterone and glucocorticoid hormones (9 of 17 investigated cases) and by the presence of specific inclusions on the skin and/or conjunctival biopsy (5 of 7 cases).

Adolescent↗
PubMed1982

[Larsen's syndrome. Clinical findings and inheritance (author's transl)].

Three new case reports of Larsen's syndrome (multiple congenital dislocations of the joints, distinctive facies and skeletal abnormalities) are presented. For the first time this condition is described to be associated with deafness and retinal dysplasia. Data in the literature show this syndrome to be inherited as sporadic, autosomal recessive or dominant. The 3 reported cases seem to have and autosomal recessive inheritance.

Abnormalities, Multiple↗
PubMed1982

[Thalamic tumors in children. A study of 38 cases (author's transl)].

The main clinical, evolutive and therapeutic features of thalamic tumors are reviewed in 38 children. Signs of increased intracranial pressure (76% of the cases) and of controlateral hemiparesis of varying degree (76% of the cases) were the main clinical symptoms. CT-scan is the best means of investigation. Therapeutic protocols that seem to obtain the best results consist of radiotherapy (between 45 and 55 grays) associated with shunting, when intracranial hypertension results from obstruction of an interventricular foramen by the tumor. Surgery is contra-indicated in most tumors in this area.

Adolescent↗
PubMed1982

[Sturge-Weber disease: value of the topographic analysis of the facial angioma for the diagnosis of associated pial angioma (author's transl)].

A retrospective study was undertaken in 47 children with congenital capillary naevus of the face in order to appreciate the predictive value of the topography of the facial angioma for the diagnosis of associated pial angioma. In patients with Sturge-Weber disease, facial angioma concerned the whole upper eyelid, extending to the forehead and/or cheek. Less often, it was localized to the root of the nose; in such cases it was a port-wine, homogeneous angioma, with clearcut outlines, quite different from the angiomatous dots frequently seen in this area in neonates. Except for these rare cases, angiomas concerned only half or less of the upper eyelid and no pial angioma was associated. Finally, congenital capillary naevi with this topography typical of Sturge-Weber disease were frequently associated with pial angioma.

Angiomatosis↗
PubMed1981

[Progressive bulbar paralysis. Report of a juvenile case (author's transl)].

The authors report the case of a 16 year-old girl with the following features: clinically, a progressive bulbar paralysis, a weakness and wasting of muscles predominantly in the upper limbs; pathologically, a severe neuronal loss in the motor nuclei of the VIIth, IXth, XIIth cranial nerves and in the anterior horns of the cervical and thoracic spinal cord, a demyelinisation of the corticospinal tracts. A classification of progressive bulbar paralysies in infants and children is proposed. In the first group, the peripheral motoneuron is the only involved. Such cases are often called Fazio-Londe disease and can be related to those cases of infantile spinal amyotrophy either of the Werdnig-Hoffmann type or, most often, of the Wolfhardt-Kugelberg type. In the second group, the corticospinal tract is also involved. Some of these cases can be included in the spinocerebellar degenerations but others, such as the case reported here, are strongly reminiscent of the adult amyotrophic lateral sclerosis.

Adolescent↗
PubMed1981

[The pattern of cerebrospinal fluid proteins in infants and children. Determination of normal values (author's transl)].

Cerebrospinal fluid (CSF) protein values were measured in 652 children between the ages of 1 day and 17 years, allowing the authors to define the dynamics of the blood-brain barrier under normal conditions and during inflammation of the nervous system. The ratio of CSF albumin/serum albumin (whose upper limit was 0.65 in the study) was the best sign of alteration of blood-brain barrier permeability. The CSF IgG level, whose upper limit was 0.85 (for serum IgG between 10 and 14 g/l) is the most useful criterion for detecting an intra-thecal synthesis of IgG. Six patterns of CSF proteins are defined on the basis of immunochemical and electrophoretic studies. The ratio of CSF albumin/serum albumin and the CSF IgG level must be compared to the electrophoretic pattern of CSF proteins in order to better characterize one aspect of the blood-brain barrier under normal and pathologic conditions of the central nervous system.

Adolescent↗
PubMed1981

[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↗