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Biomedical subjects

P Desbordes

Publications and source records attributed to P Desbordes.

15 recordsLinked to original sources

A French cluster of Creutzfeldt-Jakob disease: a molecular analysis.

We report the molecular and phenotypic analysis of a French cluster of three cases of Creutzfeldt-Jakob disease (CJD), two of them occurring in 1998 in the same village and the other in 1995 in a neighboring village. Analyses of the occurrence of these events in a close area with less than 3000 inhabitants over the 1992-1999 notification period confirmed that they are rare. This could be explained either by a common source of contamination or by the coincidental occurrence of either sporadic or genetic CJD. We applied genetic analysis and brain PrPres typing to explore these CJD cases. The three patients did not carry any mutation in their prion protein gene coding sequence. All were homozygous for methionine at the polymorphic codon 129. Brain tissue was available from two cases that died in 1998. The two patients showed different PrPres profiles on Western blot and distinct clinico-pathological features. These findings do not support the conclusion that in these three cases, CJD was acquired from a unique source of contamination and suggest that concurrent occurrence of sporadic CJD accounted for this CJD cluster.

Aged↗

[Cerebral embolism of cardiac origin].

Embolisms of cardiac origin represent approximately 15 p. cent of the causes of cerebral vascular accidents. This article considers the difficult diagnostic and therapeutic problems raised by cerebral embolisms. First: arguments permitting to suspect this mechanism in the absence of an obvious cardiac cause or in the presence of a high incidence heart disease but low embolic potential. The main etiologies are then reviewed emphasizing the evolution of their respective frequencies, then a program of additional investigative examinations is suggested. Finally the indication of anticoagulants is discussed according to neurological and cardiologic criteria.

Heart Diseases↗

Brain water and aging.

Edematous reactions surrounding brain lesions are less extensive in old patients. There also is a general tendency of the aging brain to be vulnerable to osmotic stress, to yield space, and to collapse. In order to elucidate these clinical phenomena, brain sodium, potassium and water, brain osmolarity, and Na+-K+-ATPase activity were studied in old and young rats following three experimental aggressions: cold induced vasogenic edema, osmotically induced edema, and osmotically induced dehydration. This study supports the hypothesis that: (a) extracellular edema is slightly smaller in the aged brain, but cellular swelling is relatively greater and (b) that protective adaptation of brain volume to acute osmotic changes is less efficient and slower in the aged brain.

Adaptation, Physiological↗

[Indications for the long-term electrocardiogram and echocardiogram after a cerebral ischemia complication].

Ninety patients admitted to a neurology unit for cerebral ischaemic accident without evidence of embolism from the heart were examined by ambulatory electrocardiographic monitoring (AEM) and by echocardiography assessable in 76 cases. On the basis of a standard cardiac evaluation (SCE) including physical examination, chest radiography and electrocardiography, the patients were divided into 3 groups: 42 with normal SCE (group I), 19 with isolated arterial hypertension (group II) and 36 with patent cardiac disease (group III). Significant abnormalities were detected by AEM in 10.5% of group I patients, 18.5% of group II patients and 33% of group III patients. A structural cardiopathy was discovered at echocardiography in 33%, 62% and 91% respectively of patients in these 3 groups. The greatest number of mitral valve prolapses (7/9 cases) was found in group I. The main echocardiographic abnormality detected in group II was dilatation of the left cardiac cavities: atrium more than 38 mm in 8 cases, ventricle more than 50 mm in 3 cases. Comparisons between the two explorations showed that arrhythmias were often associated with dilatation of the left cardiac cavities (39%) and much less frequently with mitral valve prolapse (11%) and myocardial hypertrophy (16%). Our results suggest that when examinations cannot be routinely performed in hospital patients with a cerebral ischaemic accident echocardiography should be reserved to patients with heart disease or hypertension, and AEM to those with heart disease or hypertension and with dilatation of the left cardiac cavities.

Adult↗

Brain water and aging.

Brain water content and Na/K-ATPase activity have been compared in two groups of rats aged 60 +/- 5 days (group I) and 630 +/- 10 days (group II). In group II control animals, brain water content (p less than 0.001) and Na/K-ATPase activity (p less than 0.02) were found to be significantly reduced. Following a cryogenic lesion of the brain, the increase in water content was larger in group I (not significant), whereas impairment of Na/K-ATPase activity was much more pronounced in group II (p less than 0.001).

Aging↗

Cavernous sinus syndrome due to lymphoma.

In both the cases described, painful ophthalmoplegia was the first indication of infiltration of the cavernous sinus by a lymphosarcoma. The onset of symptoms and the course of the disease were different in the two cases. CT scan which has been the crucial investigation for detecting lymphomas in the cavernous sinus, was normal in the early stages. Symptomatic remission could be obtained with treatment, although the prognosis remained poor.

Aged↗

Chronic inflammatory demyelinating polyneuropathy: immunopathological and ultrastructural study of peripheral nerve biopsy in 42 cases.

The authors recently reexamined the peripheral nerve biopsies from 42 patients with chronic inflammatory demyelinating polyneuropathy (CIDP). There were 27 males and 15 females, aged from 9 to 84 years, and 13 had relapses. No patient had vasculitis, monoclonal gammopathy, tumor, diabetes mellitus, Lyme disease, familial neuropathy, HIV, or any other immune deficiency. In the endoneurium, perivascular inflammatory cell infiltrates were present in only one case, but scattered histiocytes marked by KP1 on paraffin-embedded fragments were present in every case and there were no T-lymphocytes. At ultrastructural examination macrophage-associated demyelination was observed in 17 cases, of which 6 had relapses separated by intervals of several months or years. Axonal lesions without associated primary demyelination were observed in 4 cases and 3 of these had relapses. Thirty-two patients had mixed lesions of demyelination and axonal involvement. This study confirms other recent data indicating that in all cases of CIDP, macrophages are present in the endoneurium. Macrophage-associated demyelination is the characteristic feature of demyelinating forms. On the other hand, isolated primary axonal forms, which have been known since 1989, are relatively frequent and prone to relapses.

Adolescent↗

Eating seizures.

An 18-year old man had a cluster of three seizures in a few hours and then remained seizure-free without medication; his mother has experienced seizures induced by movement. A 29-year-old man has had recurrent seizures for six years. In both cases, seizures were partial motor seizures induced by chewing. They are considered to be a particular variety of movement-induced seizures triggered by proprioceptive afferents of muscular origin. This explanation does not account for the great majority of the 63 published cases of seizures precipitated by eating. Eating seizures represent a heterogeneous group of seizures with discrete electroclinical signs and mechanisms. The term "eating seizures" should be preferred to the term "eating epilepsy."

Adolescent↗