Search PubMed⌕ Search

Biomedical subjects

J Roger

Publications and source records attributed to J Roger.

At least 73 records · Page 4Linked to original sources
PubMed1985

[Episodes of apnea in an infant: unusual forms of epileptic seizures].

The authors report the observation of an infant who begins at the age of 7 months to present episodes of epileptic apnea with cyanosis, lost of consciousness, hypotony and sometimes ocular revulsion and distal myoclonia. From their onset there are several a day, more frequent during sleep than when awake. From the EEG point of view they are characterized by a 5-7 c/sec large and diffuse rhythm. The infant also presents epileptic myoclonias, tonic and partial seizures. She has a very slight evolutive encephalopathy, disclosed at the age of 4 months by a psychomotor retardation. No etiology has been proved. The CT scan shows during the evolution a cortico-subcortical atrophy. Every type of seizure was very resistant to different treatments. The apneas disappeared after ACTH therapy. The authors demonstrate the epileptic nature of these apneas which are very rarely observed after the neonatal period.

Adrenocorticotropic Hormone↗
PubMed1985

[Progressive degenerative myoclonic epilepsy. Systematized olivo-cerebellar lesions].

A 15 year-old North-African female showed typical symptoms and evolution of Progressive Myoclonus Epilepsy of the Unverricht type. Pathological examination failed to show either inclusion bodies or any other storage material. The only relevant findings included degenerative changes in the inferior olives and, to a lesser extent, in the cerebellar cortex. The site of lesions was remarkable: in the inferior olives, lesions were bilaterally and symmetrically restricted to the external angles (lateral lamellae); in the cerebellum, loss of Purkinje cells and ascending fibres of the molecular layer was prominent in the lateralmost part of the hemispheres (semilunar lobules). Such a topography implies a system disorder involving the olivo-cerebellar pathway, particularly in that part which projects to the neocerebellum. Twelve other clinico-pathological cases of progressive myoclonus epilepsy of the degenerative group are reviewed. It is suggested that, here again, lesions--although more diffuse--may be related to a primarily olivo-cerebellar involvement.

Adolescent↗
PubMed1985

[Familial thenar amyotrophy of carpal origin].

Thenar amyotrophy of carpal origin was found in two sisters aged respectively 49 and 59 years and in a 75 year-old woman and her 56 year-old daughter. The literature contains reports on about 15 cases of familial carpal tunnel syndrome. The clinical features were sensory symptoms in most patients but there was also cases with amyotrophy. The coexistence in a same family of several cases of carpal tunnel syndrome is not by itself, evidence of a genetic factor.

Aged↗
PubMed1983

[Monotherapy with sodium valproate in generalized primary epilepsy. 2d phase: Study of long-term efficacy and tolerance].

From the analysis of 115 cases of primary generalized epilepsies treated for a mean duration of 43 months with sodium valproate as sole therapy, it appears that: the mean effective daily dosage is 21 mg/kg; the efficacy of valproate proved excellent in 82.6% of cases (seizures fully controlled: 74%, seizures occurring exceptionally: 9%); a loss of activity was never observed; in these circumstances of prolonged administration of the drug, no signs of major intolerance were seen; side-effects occurred in 29% of cases, including 20% long-term effects (weight gain, essential tremor); 64 series of laboratory tests including 15 parameters made it possible to evaluate the hematological, hepatic and pancreatic tolerance of valproate: the majority of the tests were normal. The authors believe that during long-term therapy with valproate, monitoring does not need to include the routine performance of liver function tests, but that it would be more advisable, should a suggestive clinical sign be noted, to investigate the platelet count, coagulation (partial activated thromboplastin time) and protein synthesis (fibrinogen).

Adult↗
PubMed1983

[Early diagnosis of Lafora disease. Significance of paroxysmal visual manifestations and contribution of skin biopsy].

The early diagnosis of Lafora's disease is often difficult, from primary generalized epilepsy and other progressive myoclonic epilepsies. The authors emphasize the diagnostic interest of paroxysmal visual manifestations, reported in only 25 per cent of the cases in the literature, and present in 17 of their 31 personal cases. These visual manifestations can be elementary or complex. The elementary ones appear often early, before other symptoms of the disease. Two demonstrative cases are reported. In one of them an occipital seizure was recorded, demonstrating the epileptic critical nature of these elementary visual manifestations. They have a high diagnostic value since they do not occur neither in primary generalized epilepsy (Grand Mal type), nor in the other progressive myoclonic epilepsies. In the 2 reported cases, skin biopsy, as proposed by Carpenter and Karpati, demonstrated the specific storage in the sweat ducts and glands. The interest of this skin biopsy is underlined, its positivity seeming both constant and specific in Lafora's disease.

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

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↗
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

[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↗
PubMed1980

[Exclusive parenteral nutrition. Results in 45 acute exacerbations of Crohn's disease (author's transl)].

Exclusive parenteral nutrition (EPN) was used in severe acute exacerbations of Crohn's disease, on 36 occasions for an acute episode during the course on the disease, in 8 in preparation for surgery and twice for digestive fistulae. The mean duration of EPN was of 33 days. Calorie intake was 65 to 74 cal/kg/d, the nitrogen part varying from 9 to 17 g/d. Lipids accounted for 40 to 60% of the total carbohydrate-lipid calorie intake in order to avoid fatty infiltration of the liver. Complications which occur, above all at the beginning of treatment, and due in part to insertion of the catheter and secondly to metabolic problems, have since been corrected. There was a weight gain of 5.78 +/- 1.1 kg, an increase in plasma albumin of 6.1 +/- 1.5 g/l and a return to normal in the number and weight of stools. In terms of the course of the disease, EPN offered excellent preparation for surgery and remission or stabilisation of acute exacerbations in young forms of the disease, in particularly with colonic involvement.

Crohn Disease↗