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Biomedical subjects

M Arthuis

Publications and source records attributed to M Arthuis.

At least 19 recordsLinked to original sources
PubMed1994

[Ethics and handicap].

The deprivation of numerous possibilities suffered by the severely-handicapped on account of their somato-psychic deficiency extends to many areas: mobility, relationships, autonomy in their everyday life, dependency, psychological or even mental consequences. The most elementary ethics would require to take all these deprivations into account, not just in the choice of accommodation structures but also in the behaviour of relatives and friends, or of qualified members of staff, in their approach to the handicapped. The range of application of these ethical rules must therefore extend from the quality of life and assistance, and the concern for efficiency of interventions, to the problems of security and securization, the assessment and the prevention of risks deriving from the handicap or its consequences, through the information of the patients regarding their elementary or specific rights, the introduction of the most favourable basis in order to make sure that they enjoy the dignity due to any human being, whatever his condition. Such a vast program requires a great many means of action, which all imply the recourse to the appropriate material and human assistance.

Persons with Disabilities↗
PubMed1991

Physical exercise and voluntary hyperventilation in childhood absence epilepsy.

The aim of this study was to compare the effects of a physical exercise test and of voluntary hyperventilation between controls and children with absence epilepsy. Eighteen children (6 controls and 12 epileptics) were studied during rest (R), a maximal physical exercise test (15 min; PE), recovery (REC) and voluntary hyperventilation (3 min; VHPV). EEG and ECG were recorded during the experiment; respiratory parameters were measured to quantify PE; plasma levels of pH, lactate, pyruvate, glucose and antiepileptic drugs were determined. A decrease in the number of absences was observed during PE whereas an increase was observed during VHPV. We found significant positive correlations between the number of children with absences, the total number of absences for each state, frequency of absences per minute and the corresponding mean plasma pH, which demonstrate that the lower the pH is, the fewer absences occur. On the other hand, there was no relationship between the number of absences and the values of other parameters. Relations between variations of the plasma value of the pH, and thus the probable cerebral value of pH, and neuronal excitability are discussed. Our results indicate that children who suffer absence epilepsy should not be discouraged from sport practice.

Adolescent↗
PubMed1989

[Giant axonal neuropathy: intermediate filament disease with involvement of the peripheral and central nervous system].

We report a case of giant axonal neuropathy in a 14 year-old turkish boy with progressive chronic neuropathy and central involvement with mental retardation. CT showed a low density and MRI imaging multiple cavities and hypersignals of the white matter. Nerve and skin biopsies revealed an accumulation of neurofilaments in axonal swellings and an accumulation of intermediate filaments in fibroblasts, Schwann cells, endothelial cells. These findings are in accordance with the reported cases. Giant axonal neuropathy results from a generalized disorder of the intermediate filaments, but the precise biochemical defect is unknown. We would agree with Maia (1988) to name this affection "Giant Axonal Disease".

Axons↗
PubMed1988

Interferon gamma in acute and subacute encephalitis.

Intrathecal synthesis of interferon gamma was shown in 14 out of 16 samples of cerebrospinal fluid collected in the first days of disease in adults, children, and newborn infants with herpes encephalitis. This synthesis was concomitant with that of interferon alpha and was switched off when the specific antibodies in the central nervous system increased. No endogenous interferon gamma was detected in 11 serum samples or 13 samples of cerebrospinal fluid collected early in the course of the disease from patients with measles encephalitis and rubella encephalitis, or in serum and cerebrospinal fluid samples from seven patients with subacute sclerosing panencephalitis. In serum collected after the 10th day after the onset of neurological symptoms interferon gamma was present at low concentrations in only three out of 11 serum specimens from patients with measles encephalitis or rubella encephalitis. Interferon gamma was present in patients with acute herpes encephalitis and there was active virus replication, but it was not present in postinfectious encephalitis. Possibly the local production of specific antibodies masks the viral antigens and switches off the induction of interferons.

Acute Disease↗
PubMed1988

Multiple sclerosis in children: report of clinical and paraclinical features of 19 cases.

We report our experience concerning clinical and paraclinical features of multiple sclerosis in 19 children. The disease was highly variable in its presentation but acute episodes of retrobulbar optic neuritis or transverse myelitis or cerebellitis were commonly observed at the onset. Diagnosis was very often suspected as soon as the first episode when there was clinical evidence of more than one lesion (43%) or study of the cerebrospinal fluid demonstrated a local secretion of immunoglobulins (60%). Evoked potential studies and nuclear magnetic resonance imaging were performed during the course of the disease and exhibited abnormalities of the kind observed in adult patients and with a similar frequency; this suggests that such studies can be very useful in the evaluation of children suspected of having multiple sclerosis. When the initial form of the disease was a chronic myelopathy, the course was progressive from the onset, leading rapidly to a marked invalidity (15%). Most often a succession of relapses and remissions occurred after the first attack and major sequelae appeared 5 to 10 years later. Such features are not very different from those observed in adult patients and suggest that these patients can benefit from the progress resulting from therapeutic trials in adult patients.

Adolescent↗
PubMed1988

Peripheral neuropathy associated with erythrophagocytic lymphohistiocytosis.

A 12 year old patient who developed clinical, biochemical and histological features of erythrophagocytic lymphohistiocytosis is described. In contrast to previously reported cases, the prominent neurological feature was a subacute sensorimotor polyneuropathy. Sural nerve biopsy showed a marked reduction of myelinated fibres and severe axonal lesions, absence of histiocyte infiltration and deposits of IgM along the epineurium. In addition to the hypertriglyceridaemia previously described in this condition, an elevation of plasma very long-chain fatty acids and phytanic acid was found which suggests a transient impairment of peroxisomal functions.

Biopsy↗
PubMed1987

Soluble IL-2 receptors in acute and subacute encephalitis.

Elevated levels of soluble interleukin-2 receptors were present in the serum from patients with acute primary and postinfectious encephalitis and subacute sclerosing panencephalitis. In addition, soluble interleukin-2 receptors were detected in the cerebrospinal fluid from patients with acute primary encephalitis. Their presence in the cerebrospinal fluid was not explained by damage to the blood-brain barrier and our data attest to their local origin. This suggests that it may be possible in certain neurological diseases to detect cerebral T-lymphocyte activation through a specific marker in the cerebrospinal fluid.

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↗
PubMed1986

Congenital muscular dystrophy and cerebral CT scan anomalies. Results of a collaborative study of the Société de Neurologie Infantile.

We present the results of a collaborative study on the association of congenital muscular dystrophy with central nervous system anomalies revealed by CT scan investigation of 10 patients. In seven children, an abnormal hypodensity of the cerebral white matter is found; in four of these patients, this radiological anomaly is either isolated, or associated with a moderate intellectual impairment; in one case, severe mental retardation and ocular changes had occurred; in the other two cases, the muscular disease was progressing slowly, in association with microcephaly, epilepsy, and moderate mental retardation. Three children were afflicted with a severe early encephalopathy and congenital muscular dystrophy, and presented signs of cortical and subcortical atrophy on CT scan. Two of these patients corresponded to different types of cerebro-ocular dysplasia-muscular dystrophy syndromes, and the third patient of Fukuyama's congenital muscular dystrophy. These observations are discussed and compared with those reported in the literature. The authors emphasize the need to investigate possible cerebral CT scan anomalies in congenital muscular dystrophies, and to look for muscular changes in some prenatal encephalopathies.

Atrophy↗
PubMed1986

Sodium valproate monotherapy in childhood epilepsy.

154 patients with a mean age of 6 years 1 month were followed on valproate monotherapy for a period ranging from 5 to 27 months (mean 22 months). Absence epilepsies, benign myoclonic epilepsies and epilepsies with tonic-clonic seizures on awakening were the best controlled, followed by benign partial epilepsies and infantile spasms. Reduction to monotherapy resulted in improvement in 13 of 14 patients with primary generalized epilepsy. Sixteen per cent of the 154 patients suffered mild to moderate adverse effects. After cessation of treatment in 28 seizure-free patients, no recurrence was observed in absence epilepsy, benign myoclonic epilepsy, infantile spasms or benign partial epilepsy, whereas two thirds of the patients with generalized tonic-clonic seizures on awakening relapsed in the year following the cessation of the treatment.

Adolescent↗
PubMed1986

[Child health in the school environment].

The authors studied the consequences of the present school system on children's health. They emphasize the causes of failures, mostly related to a misappreciation of children's individual differences and inequalities of the rate of physical and psychoaffective development. Overloaded school days, the lack of respect of sleep and extra-curricular needs, the overlooking of attention capacities were also studied.

Adolescent↗
PubMed1985

[Status epilepticus in the infant. Semeiologic, etiologic and prognostic aspects].

During a 4 year period, 79 infants aged from 1 month to 2 years suffered a status epilepticus (SE). The seizures were most often either generalized of tonic-clonic or clonic type, or unilateral clonic; they lasted or were repeated with persistent unconsciousness between the seizures for periods ranging from 30 min to several days. All the cases of SE lasting over 6 h resulted from a recognizable acute brain injury, whereas 11 of 14 cases lasting 1 h or less were cryptogenic. In 2 cases only, the neurological status was worse after the status, apparently as a result of the seizures themselves. The authors discuss the hypothesis of purely febrile HH syndrome and point out the importance of detailed clinical ictal and interictal characteristics for the etiological approach.

Child, Preschool↗
PubMed1984

[Scanography in purulent meningitis in newborn infants].

Brain CT scans of 40 neonates with bacterial meningitis showed that the main lesions consisted of obstacles to CSF flow resulting in hydrocephalus during the second month; single or multiple foci of ischaemia of very early onset appearing as hypodense areas sometimes contrast-enhanced, and abscesses consecutive to haematogenous septic necrosis. The organisms responsible for these abscesses usually were Proteus spp., more rarely other enterobacteria. Attention is drawn to the usefulness of CT in detecting the nature and size of lesions which are often clinically silent, except for convulsions.

Brain↗
PubMed1984

Two patients with interstitial del (14q), one with features of Holt-Oram syndrome. Exclusion mapping of PI (alpha-1-antitrypsin).

Patient no 1, a boy, was carrier of a de novo del (14) (pter- greater than q23::q32- greater than qter). Patient no 2, a boy, had a de novo del (14) (pter- greater than q23::q24.2- greater than qter). Common dysmorphisms included bushy eyebrows, frontal bossing, and micrognathia. Patient no 2 had features of Holt-Oram syndrome, i.e. congenital heart defect and severe ulnar defect. Patient no 1 had congenital heart defect but no typical osseous disorders. The association of Holt-Oram syndrome and del 14q24.1 is stressed. Patient no 1 was heterozygous for Pl (alpha-1-antitrypsin) phenotypes. The gene locus could thus be excluded from q24 and q31, and tentatively assigned to q32.1.

Abnormalities, Multiple↗
PubMed1984

[Non-progressive congenital cerebellar ataxia].

The authors studied the main clinical and genetic aspects of congenital cerebellar ataxia in children, from the data obtained in 121 cases. The case reports were divided into 2 groups, according to the severity of the static ataxia. In the absence of absolute criteria allowing the recognition of the various forms of congenital cerebellar ataxia, they give the following genetic counsel: in cases with congenital cerebellar ataxia of unknown origin, the risk or recurrence is higher in cases with severe static ataxia (near 25%) but not negligible in the other types of ataxia, especially in cases associated with severe mental retardation (risk between 5 and 10%).

Adolescent↗
PubMed1984

[Pediatric dermatomyositis. Apropos of 28 cases].

The authors report 28 cases of juvenile dermatomyositis. Bohan and Peter criteria were fulfilled in all cases. All children received a treatment with prednisone as soon as diagnosis was established. Five patients (18%) died during this treatment. The remaining children were followed for at least 2 years after prednisone treatment was discontinued. An acute onset was found in 4 of the 5 children who died and in 6 of 23 children still alive. Swallowing disorders were present in all children who died and in 11 of the children still alive. Ten children recovered fully and 13 presented with sequellae. The average interval between clinical onset and diagnosis was 2 months 19 days for the children who recovered and 5 months 21 days for those presenting with sequellae. The average duration of the period of activity of dermatomyositis was 6 months for the children who recovered and 3 years 4 months for those with sequellae.

Adolescent↗
PubMed1984

[Calcinosis of childhood dermatomyositis. Apropos of 10 cases].

Twenty-eight children were diagnosed as having dermatomyositis (DM) on the basis of the criteria of Bohan and Peter. Ten of the 28 children developed calcinosis. Calcium deposits mainly occurred in DM with gradual onset of symptoms and with a chronic course. Calcinosis always appeared insidiously and early during the active stage of the disease. It persisted for a long time beyond the remission of the active muscle symptoms without tendency to spontaneous resolution. Long-term follow-up data substantiate calcinosis as the most frequent and the most serious sequela in this series of childhood DM.

Calcinosis↗