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Biomedical subjects

J Roger

Publications and source records attributed to J Roger.

At least 91 records · Page 5Linked to original sources
PubMed1980

The retina in Lafora disease: light and electron microscopy.

Lafora bodies are described in the retina of a 16 year old female who died five years after the onset of a typical familial progressive myoclonus epilepsy which was diagnosed as Lafora disease by brain biopsy and by autopsy findings. The patient was the offspring of consanguinous parents who had three affected siblings out of nine. The fine structure and distribution of Lafora bodies, which represent a specific non-lysosomal cell storage disorder, is reported for the first time in the human retina. The nature of the abnormal material in the Lafora bodies, which are identical to those present in the brain, heart and liver tissues in the same patient and in her brother, is discussed according to their iodide spectrum. In this respect, Lafora disease might be related to the inborn errors of carbohydrate metabolism and its relationship with Type IV Glycogenosis (Anderson's disease) must be verified by further investigations.

Adolescent↗
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

[Status epilepticus and multiple sclerosis (author's transl)].

Status epilepticus was a complicating feature of the last bout before the fatal outcome in a patient with a 11 years history of multiple sclerosis. The fits were seen as clonic movements of the eyes in a lateral direction, lasting for 30 to 90 seconds and repeated every 5 minutes on average. Electroencephalographic recordings demonstrated periodic slow spikes on the right hemisphere, starting in the anterior region. Neuropathological data eliminated an associated pathological condition and suggested that the development of acute cortico-subcortical demyelinating lesions was responsible of the epilepsy. The type of electrical activity observed, localized to one hemisphere, is found with recent ischemic lesions, and less frequently in cases of cerebral tumor, necrotizing encephalitis, metabolic encephalopathies or post traumatic disorders. A case with similar clinical and electroencephalographic features in a patient with multiple sclerosis has been reported in the literature.

Adult↗
PubMed1980

[Colonic complications of pancreatitis. A report on 39 cases (author's transl)].

Colonic complications of pancreatitis are not infrequent occurrences, as illustrated by the fact that the authors have personally treated 39 such cases. Lesions are found in the colon at sites which correspond perfectly to diffusion of pancreatic necrotic lesions along mesenteric pathways as described by Meyers. They occur mainly in the left colic flexure (17 cases) and transverse colon (10 cases) but may affect the ascending (6 cases) and descending (4 cases) colon by diffusion of the pancreatitis into the anterior extrarenal space. These topographical characteristics, associated with the extrinsic and inflammatory nature of the lesions, produce a very specific and typical radiological syndrome which should assist diagnosis.

Adult↗
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↗
PubMed1979

[Intermittent postoperative gastric aspiration. New equipment (author's transl)].

Gastric suction is prescribed routinely by some, even though it is responsible for complications which are sometimes extremely serious, such as haemorrhagic gastritis or even perforation. It is indicated in all cases of postoperative gastric ileus or of stenosis causing marked dilatation of the stomach. However, experience would tend to show that in addition to problems immediately secondary to the presence of the tube in the gastric lumen, it sometimes leads to a false sense of security since the apertures of the tube tend to suck in the mucosa, giving rise to a suction-like effect whilst the stomach remains full of fluid. In order to overcome this disadvantage, we have designed an apparatus which varies the times of suction over a selected period. It fills the troublesome role previously filled by nursing staff in confirming the satisfactory function of gastric tubes. Thus not only does the tube not become blocked but gastric transit is restored more rapidly which not only leads to savings in the cost of intensive care but offers additional technical security.

Gastric Dilatation↗
PubMed1979

[Recent data on Lafora disease. Apropos of 17 cases].

This study reviews 99 anatomically verified case of Lafora body disease (82 from the literature and 17 personal cases). The clinical symptoms of the disease are characterised by the triad; epilepsy, myoclonus and dementia. An anatomical and histochemical study has been undertaken and as a result emphasis is given to recent hypotheses that suggest there are similarities with Type IV glycogen storage disease (Andersen's disease) which, although clinically distinct, has the same enzyme defect.

Adolescent↗
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↗