Effect and impact of mechanical ventilation in myotonic dystrophy type 1: a prospective cohort study.

Few studies have assessed the impact of home ventilation in patients with myotonic dystrophy type 1 (DM1) and no specific recommendations are available. We assessed the survival associated with category of home ventilation adherence of patients with DM1 followed up at a home ventilation unit using a Cox proportional hazards model. 218 patients were included; those who refused or delayed their acceptance of non-invasive ventilation were at higher risk for severe events (invasive ventilation or death) (P=0.

Survival of patients with chronic respiratory failure on long-term oxygen therapy and or non-invasive ventilation at home.

Background & Aims: Chronic respiratory failure (CRF) is the common fate of respiratory diseases where systemic effects contribute to outcomes. In a prospective cohort of home-treated patients with CRF, we looked for predictors of long-term survival including respiratory, nutritional and inflammatory dimensions.

Methods: 637 stable outpatients with CRF, 397 men, 68 ± 11 years, on long-term oxygen therapy and/or non-invasive ventilation from 21 chest clinics were enrolled and followed over 53 ± 31 months.

Plasma exchange for Guillain-Barré syndrome.

Background: Guillain-Barré syndrome is an acute paralysing disease caused by peripheral nerve inflammation. This is an update of a review first published in 2001 and last updated in 2008.

Objectives: To assess the effects of plasma exchange for treating Guillain-Barré syndrome.

Preceding infections and anti-ganglioside antibody profiles assessed by a dot immunoassay in 306 French Guillain-Barré syndrome patients.

We describe by an in-house dot immunoassay, specific anti-ganglioside and sulfatide antibodies, by comparing the results from a large group of 134 infected French GBS patients and those from 172 noninfected French GBS and 142 control groups. A recent infection was identified in 134/306 (43.8%) GBS patients: Campylobacter jejuni (24.

Guillain-Barré syndrome following primary cytomegalovirus infection: a prospective cohort study.

Background: Little is known about the epidemiology and the prognostic factors of Guillain-Barré syndrome (GBS) following primary infection with cytomegalovirus (CMV-GBS).

Methods: We prospectively followed up 506 patients with cases of GBS who were admitted to our center from 1996 through 2006. We diagnosed 63 (12.

Hyperbaric oxygen therapy for acute domestic carbon monoxide poisoning: two randomized controlled trials.

Introduction: Although hyperbaric oxygen therapy (HBO) is broadly used for carbon monoxide (CO) poisoning, its efficacy and practical modalities remain controversial.

Objectives: To assess HBO in patients poisoned with CO.

Design: Two prospective randomized trial on two parallel groups.

Detection of Campylobacter jejuni by culture and real-time PCR in a French cohort of patients with Guillain-Barre syndrome.

Bacteriological culture and real-time PCR (RT-PCR) were used to detect Campylobacter jejuni in fecal samples from a French cohort of 237 patients with Guillain-Barré syndrome (GBS). We provide evidence that diverse serotypes and genotypes of C. jejuni are a major trigger of GBS in France.

Guillain-Barré syndrome and influenza virus infection.

Background: In Western countries, the cause of 60% of all Guillain-Barré syndrome (GBS) cases remains unidentified. The number of cases of unidentified cause peaks in winter, and these cases are commonly preceded by respiratory tract infection or influenza-like illness. We investigated the triggering role of influenza virus infection.

Automatic air-leak compensation in neuromuscular patients: a feasibility study.

Air leaks often result in alveolar hypoventilation in mechanically ventilated patients with neuromuscular disease. The primary objective of this study was to assess the feasibility, efficacy and tolerance of a ventilator equipped with an automated air-leak compensation system in a clinical situation. Fourteen neuromuscular patients with nocturnal air leaks during home ventilation were included in a prospective randomised crossover study.

[Acute carbon monoxide poisoning].

Carbon monoxide (CO) poisoning is still complicated by a high mortality and morbidity rate. Diagnosis can be obvious but is most of time difficult and sometimes remained unknown. It is usually based on clinical signs and must be confirmed by assessment of CO level in room air or in patient's expired breathing or blood and detection of a source.

Noninvasive ventilation as an alternative to endotracheal intubation during tracheotomy in advanced neuromuscular disease.

Objective: To compare conventional tracheotomy with endotracheal intubation to tracheotomy with noninvasive positive-pressure ventilation (NPPV) in advanced neuromuscular disease.

Methods: This was a retrospective study of a historical cohort of patients tracheotomized while sedated and intubated versus patients tracheotomized under NPPV and local anesthesia. We recorded previous intubation difficulties, complications (eg, aspiration pneumonia), and hospital stay.

Hand versus mouth for call-bell activation by DMD and Becker patients.

Severe hand dysfunction is common in patients with Duchenne muscular dystrophy (DMD) and may preclude the use of conventional call-bells. We prospectively evaluated a call-bell with two hand-controlled interfaces (push-button and key-pinch) and two mouth-controlled interfaces (sip-or-puff) in 32 consecutive DMD and Becker patients. Patients called intentionally 348 times, using the sip-or-puff device 237 times and the hand-controlled interfaces 147 times.

Immunotherapy for Guillain-Barré syndrome: a systematic review.

Guillain-Barré syndrome (GBS) is an acute inflammatory disorder of the peripheral nervous system thought to be due to autoimmunity for which immunotherapy is usually prescribed. To provide the best evidence on which to base clinical practice, we systematically reviewed the results of randomized trials of immunotherapy for GBS. We searched the Cochrane Library, MEDLINE and EMBASE in July 2006 and used the methods of the Cochrane Neuromuscular Disease Group to extract and synthesize data.

Breathing-swallowing interaction in neuromuscular patients: a physiological evaluation.

Rationale: Malnutrition and aspiration are major problems in patients with neuromuscular disease. Because impaired swallowing contributes to malnutrition, means of improving swallowing are needed.

Objectives: To investigate interactions between breathing and swallowing in neuromuscular disorders and to evaluate the impact of mechanical ventilation (MV) on swallowing in tracheostomized patients.

Clinical and electrophysiological predictors of respiratory failure in Guillain-Barré syndrome: a prospective study.

Background: Respiratory failure is the most serious short-term complication of Guillain-Barré syndrome and can require invasive mechanical ventilation in 20-30% of patients. We sought to identify clinical and electrophysiological predictors of respiratory failure in the disease.

Methods: We prospectively assessed electrophysiological data and clinical factors, including identified predictors of delay between disease onset and admission, inability to lift head, and vital capacity, in patients admitted with Guillain-Barré syndrome.

Prognosis and risk factors of early onset pneumonia in ventilated patients with Guillain-Barré syndrome.

Objective: Invasive mechanical ventilation is required in 30% of patients with Guillain-Barré syndrome (GBS) and is associated with pneumonia and increased mortality. Our objective was to determine the incidence, characteristics, outcomes, and risk factors of pneumonia in GBS patients receiving mechanical ventilation.

Design And Setting: Study of a prospective database in an intensive care unit of a university hospital.

Guillain-Barré syndrome, greater Paris area.

We studied 263 cases of Guillain-Barré syndrome from 1996 to 2001, 40% of which were associated with a known causative agent, mainly Campylobacter jejuni (22%) or cytomegalovirus (15%). The cases with no known agent (60%) peaked in winter, and half were preceded by respiratory infection, influenza-like syndrome, or gastrointestinal illness.

Assessing inspiratory muscle strength by sniff nasal inspiratory pressure.

The sniff nasal inspiratory pressure (SNIP) consists in the measurement of pressure through an occluded nostril during sniffs performed through the controlateral nostril. It is an accurate and noninvasive approximation of esophageal pressure swing during sniff maneuvers. However SNIP can underestimate esophageal pressure swing in subjects with nasal obstruction, patients with chronic obstructive pulmonary disease and severe neuromuscular patients.

Pressure support versus assisted controlled noninvasive ventilation in neuromuscular disease.

Introduction: Noninvasive ventilation (NIV) is being increasingly used in patients with chronic neuromuscular disorders, but the optimal ventilation mode remains unknown. We compared physiological short-term effects of assist/controlled ventilation (ACV) and two pressure-limited modes (pressure-support ventilation [PSV] and assist pressure-controlled ventilation [ACPV]) in patients with neuromuscular disease who needed NIV.

Methods: Tidal volume was 10 to 12 mL/kg.

Respiratory dysfunction in Guillain-Barré Syndrome.

Guillain-Barré Syndrome is the leading cause of nontraumatic acute paralysis in industrialized countries. About 30% of patients have respiratory failure requiring intensive care unit (ICU) admission and invasive mechanical ventilation. Progressive weakness of both the inspiratory and the expiratory muscles is the mechanism leading to respiratory failure.

[Guillain-Barré syndrome].

The annual incidence of Guillain-Barré Syndrome is 1.5 per 100000 people. It has an associated mortality of about 5%, with ten percent of patients severely disabled one year after the first onset of neurological signs.