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Biomedical subjects

J D Degos

Publications and source records attributed to J D Degos.

At least 73 records · Page 4Linked to original sources
PubMed1993

Pseudotumoral neuro-Behçet's disease.

Pseudotumoral presentation of Neuro-Behçet's disease has been described before. The diagnosis may be difficult obtain in patients without mucosal, cutaneous, and ocular signs. We report the observation of a young patient suffering from a right hemiparesis with computed-tomographic (CT) features suggestive of a thalamocapsular expanding lesion. Histologic study of brain biopsy tissue ruled out a tumor but did not show any specific diagnosis. The patient improved with steroid therapy.

Adult↗
PubMed1993

Severe frontal syndrome associated with infarcts of the left anterior cingulate gyrus and the head of the right caudate nucleus. A clinico-pathological case.

The clinicopathological findings in a patient who presented a severe frontal syndrome and who had ischaemic lesions in the left anterior cingulate gyrus and in the head of the right caudate nucleus are reported. The clinical features included complex disorders such as distractibility, docility, emotional unconcern, manual grasping, prehension and utilization behaviour, perseverations and anterograde amnesia. They are closely similar to those previously described in a patient with bilateral lesions involving the anterior cingulate gyri. These findings suggest that (i) in the presence of a lesion of the anterior cingulate gyrus on one side, a lesion of the head of the contralateral caudate nucleus may be equivalent to a lesion of the anterior cingulate gyrus, the anterior cingulate gyrus--caudate complex working as a single functional unit; (ii) the anterior cingulate gyri (and anterior cingulate gyrus--caudate complexes) play a crucial role in the expression of frontal lobe functions; (iii) they are necessary for sustained goal-oriented responses to (emotionally or not) discriminated stimuli.

Aged↗
PubMed1993

Peripheral neuropathies after arthropod stings not due to Lyme disease: a report of five cases and review of the literature.

Five patients developed neurologic symptoms a few hours to 2 months after being stung by a non-hooking arthropod with immediate cutaneous reaction. The patients had no clinical or serologic evidence for Lyme borreliosis and rickettsial disease. Clinical and electrophysiologic findings were consistent with a mixed axonal and demyelinating mononeuropathy, a monomelic multiple mononeuropathy, a mononeuropathy multiplex, a radiculoneuritis, and a distal symmetric polyneuropathy. Muscle and nerve biopsies showed lymphoplasmacytic small-vessel vasculitis in all patients, and wallerian degeneration in three. These patients, and 17 others from the literature, indicate a spectrum of peripheral neuropathies occurring after insect and spider stings.

Adolescent↗
PubMed1993

[Treatment of tremor by stereotactic surgery].

Thalamotomy in the ventrolateral thalamic nucleus is a common treatment of severe parkinsonian or essential tremor. Although results are often satisfactory, complications may occur and alter the success of the operation. High frequency stimulation of the target before thalamotomy leads to transitory abolition of the tremor. Therefore, a chronic stimulation device was developed and its use produced results similar to those of thalamotomy for parkinsonian tremor. Unwanted side-effect can be reduced by changing the parameters of the stimulation. Improvements in multiple sclerosis and post-traumatic tremors may be expected. We present a review of the indications and results of both technics.

Electric Stimulation Therapy↗
PubMed1993

Concentric MR patterns in multiple sclerosis. Report of two cases.

We report two cases of multiple sclerosis with concentric patterns of demyelinated foci on MR images. These were compared with the pathological data of Balò's disease. The T2-weighted MR images displayed alternating high and low signal areas which probably corresponded to the concentric type of demyelination. Our two cases were characterized by their acute onset and their regression following corticosteroid therapy. This suggests that Balò's disease may be a transient disease and not always associated with a fatal course.

Adult↗
PubMed1992

Brain energy metabolism in bilateral paramedian thalamic infarcts. A positron emission tomography study.

Positron emission tomography (PET) studies of the cerebral metabolic rate of oxygen (CMRO2) were performed in seven consecutive patients with bilateral paramedian thalamic infarcts (BPTI), selected on neuroradiological and clinical criteria. The latter consisted of sudden onset of coma or confusion followed by a persistent amnesia of varying severity, with or without language impairment and frontal lobe signs. There was a highly significant decrease of CMRO2 for the whole cortex as well as for all the regions analysed: medial-frontal, latero-frontal, temporal, sensorimotor and posterior associative cortex. The mean regional metabolic ratios (region/whole cortex CMRO2) were not significantly different from controls, indicating an essentially uniform effect in the cortex, except the sensorimotor ratio which was significantly increased. Diffuse cortical hypometabolism most likely reflects thalamo-cortical deafferentation secondary to damage to the 'non-specific' thalamic nuclei, while sparing of the latero-ventral thalamus presumably explains the relative preservation of the sensorimotor cortex metabolism. Although no clear-cut individual relationship was found between magnitude of cortical hypometabolism and the severity and pattern of neuropsychological impairment, the data suggest that the former underlies and/or reflects the latter. Further studies with higher resolution PET devices might shed more light on the relationships between distinct cognitive patterns and specific topography of cortical hypometabolism in BPTI patients.

Adult↗
PubMed1992

Hypothermia, Wernicke encephalopathy and multiple sclerosis.

Neurological exacerbation observed in MS patients is usually related to a demyelinating process. We report two patients where hypothermia (32.4 degrees C and 32.5 degrees C) and neurological exacerbation were probably due to a Wernicke encephalopathy (WE). The clinical features and the rapid efficiency of parenteral thiamine were suggestive of WE. Hypothermia is an exceptional symptom observed in MS and has been considered as resulting from hypothalamic demyelination; these two cases showed that WE which is another cause of reversible hypothermia, can be associated with MS.

Body Temperature Regulation↗
PubMed1991

[Multiple sclerosis. From lesion to symptoms].

Clinical manifestations of multiple sclerosis cannot be exactly correlated with lesions identified by brain imaging, electrophysiology or even anatomy. It is generally accepted that a continuous conduction is possible within some demyelinated fibers, and a conduction block may occur in the CNS white matter without demyelinization. These data explain the possible occurrence of brief, repetitive deficits besides the classical relapses. Among the latter, at least some are not correlated with demyelinization. The knowledge of these mechanisms suggest the possibility of therapy to restore the conduction without a direct action on the demyelinating process.

Animals↗
PubMed1991

Muscle mitochondrial DNA in encephalomyopathy and ragged red fibres: a Southern blot analysis and literature review.

Various mitochondrial DNA abnormalities have been described in patients with encephalomyopathies. We performed Southern blot analysis of skeletal muscle mitochondrial DNA in nine adult patients with clinical features and ragged red fibres suggesting mitochondrial dysfunction. Two patients with encephalomyopathy and two with the MERRF syndrome (myoclonus epilepsy with ragged red fibres) had the normal PvuII restriction pattern of muscle mitochondrial DNA. In contrast, mitochondrial DNA deletion was observed in two of six patients with ophthalmoplegia. One suffered from typical Kearns-Sayre syndrome and the other from isolated external ophthalmoplegia. None of these patients had affected relatives. The detection of mitochondrial DNA deletion in external ophthalmoplegia and their site and size support previously reported data.

Blotting, Southern↗
PubMed1991

Increased growth of myoblasts from hypertrophic muscles in syringomyelia.

Proliferation and differentiation of myoblasts from hypertrophic muscles were studied "in vitro" in two cases of syringomyelia with muscle hypertrophy (MH). Their myoblast growth was compared with that of muscle cells sampled on the contralateral side in the same patients and in control subjects. The effect of a circulating factor was tested using patient sera in place of fetal calf and horse sera. The results showed that MH cells were morphologically abnormal (giant and granular). MH myoblasts proliferated more rapidly than contralateral and normal myoblasts, their fusion was accelerated and the resulting myotubes synthesized higher levels of protein. MH sera increased these effects. Serum factors are therefore likely to be involved in "in vivo" muscle hypertrophy. These findings suggest that the pathogenesis of muscle hypertrophy in syringomyelia involves acquired abnormalities due to molecules released in response to neural lesions.

Adult↗