The Impact of Age on Outcome of Embryonal and Alveolar Rhabdomyosarcoma Patients.:A Multicenter Study

Background: The prognosis of rhabdomyosarcoma (RMS) in children and adolescents has improved since the introduction of multi-agent chemotherapy. However, outcome data of adults with RMS are scarce. This multicenter retrospective study investigated the effect of age on outcome of RMS. Patients and Methods: Data were collected from three Dutch University Medical Centers between 1977-2009. The effect of age and clinical prognostic factors on relapse-free and disease-specific survival (DSS) were analyzed. Results: Age as a continuous variable predicted poor survival in multivariate analysis. Five-year DSS was highest for non-metastatic embryonal RMS, followed by non-metastatic alveolar RMS and was poor in metastatic disease. Higher age correlated with unfavorable histological subtype (alveolar RMS) and with metastatic disease at presentation in embryonal RMS. In non-metastatic embryonal RMS and in all alveolar RMS, higher age was an adverse prognostic factor of outcome. Conclusion: This study indicates that age is a negative predictor of survival in patients with embryonal and alveolar RMS

Encéphalopathies métaboliques

International audienceMetabolic encephalopathies (ME) are a common cause of admission to emergency rooms, to hospitalization wards or to intensive care units. They could account for 10 to 20% of causes of comatose states in ICU and could be associated to a poor outcome especially in older patients. Nevertheless, they are often reversible and are associated with a favorable outcome when diagnosed and rapidly treated. They correspond to an altered brain functioning secondary to the deficiency of a substance that is mandatory for the normal brain functioning or to the accumulation of a substance that can be either endogenous or exogenous. It preferably occurs in co-morbid patients, complicating its diagnosis and its management. Altered brain functioning, going from mild neuropsychological impairment to coma, movement disorders especially myoclonus and the absence of any obvious differential diagnosis are highly suggestive of the diagnosis. Whereas some biological samplings and brain MRI are essential to rule out differential diagnosis, some others, such as electroencephalogram, may be able to propose important clues in favor of the diagnosis. Once simple symptomatic measures are introduced, the treatment consists mainly in the correction of the cause. Specific treatment options are only seldom available for ME; this is the case for hepatic encephalopathy and some drug-induced encephalopathies. We will successively describe in this review the main pathophysiological mechanisms, the main causes, favoring circumstances of ME, the differential diagnosis to rule out and the etiological work-up for the diagnosis. Finally, a diagnostic and therapeutic strategy for the care of patients with ME will be proposed.Les encéphalopathies métaboliques (EMtb) sont des causes fréquentes de recours au système de soins, via les urgences, l'hospitalisation et même la réanimation. Elles pourraient représenter jusqu'à 10 à 20 % des causes de coma en réanimation et compromettre le pronostic vital, notamment chez le sujet âgé. L'EMtb correspond à une altération du fonctionnement cérébral due à un déficit en une substance indispensable au métabolisme normal ou à l'accumulation d'une substance toxique que celle-ci soit endogène ou exogène. Elle survient préférentiellement chez des patients présentant des comorbidités, complexifiant ainsi son diagnostic et sa prise en charge. Son diagnostic clinique repose sur la survenue subaiguë de troubles des fonctions supérieures et de mouvements anormaux, myoclonies principalement, en l'absence de signe de localisation neurologique. Indispensables pour éliminer les diagnostics différentiels, certains examens complémentaires sont parfois en mesure d'apporter des arguments positifs en faveur du diagnostic. C'est le cas pour certains dosages biologiques, et l'électroencéphalogramme. Une fois les mesures symptomatiques simples mises en oeuvre à l'évocation du diagnostic, le traitement repose souvent sur le traitement de la cause. Seules certaines encéphalopathies métaboliques dont l'encéphalopathie hépatique bénéficient de prises en charge spécifiques. Nous décrirons successivement les principaux mécanismes physiopathologiques et les étiologies les plus fréquentes d'EMtb, les circonstances favorisantes, la présentation clinique, les diagnostics différentiels à éliminer, et les examens complémentaires utiles au diagnostic. Nous proposerons enfin une stratégie de prise en charge de l'EMtb

PNES Epidemiology: what is known, what is new?

International audienc

Nonsystemic vasculitic neuropathy: Presentation and long-term outcome from a French cohort of 50 patients.

International audienc

The Impact of Age on Outcome of Embryonal and Alveolar Rhabdomyosarcoma Patients.: A Multicenter Study

Background: The prognosis of rhabdomyosarcoma (RMS) in children and adolescents has improved since the introduction of multi-agent chemotherapy. However, outcome data of adults with RMS are scarce. This multicenter retrospective study investigated the effect of age on outcome of RMS. Patients and Methods: Data were collected from three Dutch University Medical Centers between 1977-2009. The effect of age and clinical prognostic factors on relapse-free and disease-specific survival (DSS) were analyzed. Results: Age as a continuous variable predicted poor survival in multivariate analysis. Five-year DSS was highest for non-metastatic embryonal RMS, followed by non-metastatic alveolar RMS and was poor in metastatic disease. Higher age correlated with unfavorable histological subtype (alveolar RMS) and with metastatic disease at presentation in embryonal RMS. In non-metastatic embryonal RMS and in all alveolar RMS, higher age was an adverse prognostic factor of outcome. Conclusion: This study indicates that age is a negative predictor of survival in patients with embryonal and alveolar RMS