[Assessment of medication prescribing in a geriatric follow-up care and rehabilitation].

The aim of the study was to analyse the prescriptions and identify the factors associated with inappropriate prescribing (IP) and polypharmacy. Polypharmacy and IP concerned 80% of the 54 patients included in the study upon admission, and 78% upon discharge without a statistically significant reduction. A critical analysis of prescriptions would help to reduce inappropriate prescribing and polypharmacy in elderly patients.

[Physicians and advance directives in geriatric setting].

Advance directives (AD) are not sufficiently applied in geriatric setting despite their widespread dissemination since the publication of Claeys-Leonetti law. The aim of this study was to analyze geriatricians' knowledge of the concept of AD and to evaluate their practices in the process of patient information and AD collection.

Should patients with asymptomatic pompe disease be treated? A nationwide study in France.

Introduction: Acid α-glucosidase deficiency, that is, Pompe disease, is a glycogenosis for which enzyme replacement therapy (ERT) is available. It is not known whether patients diagnosed at an asymptomatic stage should be treated to prevent progression of the disease.

Methods: We investigated 7 patients with asymptomatic Pompe disease identified from the French Pompe registry.

The French Pompe registry. Baseline characteristics of a cohort of 126 patients with adult Pompe disease.

Pompe disease is a rare autosomal recessive muscle lysosomal glycogenosis, characterised by limb-girdle muscle weakness and frequent respiratory involvement. The French Pompe registry was created in 2004 with the initial aim of studying the natural history of French patients with adult Pompe disease. Since the marketing in 2006 of enzyme replacement therapy (alglucosidase alfa, Myozyme(®)), the French Pompe registry has also been used to prospectively gather the biological and clinical follow-up data of all adult patients currently treated in France.

Attitudes and expectations of patients with neuromuscular diseases about their participation in a clinical trial.

Aim: This study aimed to gain a better understanding of the psychological impact of participating in a clinical trial for patients with Pompe disease (Acid Maltase Deficiency). Attitudes and expectations of adult patients with neuromuscular diseases regarding medical trials are as yet unreported. In order to learn about the psychological consequences of participating in a clinical trial, we conducted a prospective assessment of patients with late-onset Pompe Disease, a rare genetic condition, for which no treatment had been available before.

Whole-body muscle MRI in 20 patients suffering from late onset Pompe disease: Involvement patterns.

To describe muscle involvement on whole-body MRI scans in adult patients at different stages of late-onset Pompe disease. Twenty patients aged 37 to 75 were examined. Five were bedridden and required ventilatory support.

Rigid spine syndrome revealing late-onset Pompe disease.

The authors describe a 50-year-old man who was evaluated for a rigid spine syndrome with onset at age 15, and subsequent walking difficulties. Cardiac and pulmonary functions were normal. Deltoid biopsy revealed the presence of small vacuoles and increased glycogen with Periodic Acid Schiff staining in a limited number of fibers.