Highlights • Nocturnal hypercapnia may be present in daytime normocapnic neuromuscular patients. • Nocturnal hypercapnia seems to predict mechanical ventilation in follow-up. • Several cut-offs have ...been proposed to define nocturnal hypoventilation. • Peak TcCO2 should be the preferred criterion for nocturnal hypoventilation.
Abstract Background/objectives Muscular dystrophies are genetic muscle disorders, in which heart involvement and chronic respiratory impairment affect survival. Cardiac conduction disturbances ...require implantable cardiac pacemaker. Implantable defibrillators may also be necessary to prevent cardiac sudden death. The safety and risk of cardiac electronic devices' implantation are not known in patients with muscular dystrophy. We aimed to assess the risks related to cardiac implantable electronic devices (CIED) in muscular dystrophy patients ventilated by tracheostomy. Methods We reviewed all medical charts of neuromuscular patients and identified all CIED implantations of pacemakers (PM) or defibrillators (ICD) in patients ventilated using tracheostomy. Results Twelve device implantations were included, performed in 9 patients (5 DMD, 1 Becker muscular dystrophy and 3 DM1). Mean age was 39.9 years ± 13.0. All patients were wheel-chair bound and tracheotomized. Six pacemakers (PM) and 6 cardiac resynchronization (CRT) devices, including 2 defibrillators (CRT-D) were implanted. Following device implantation, two patients had a pneumothorax and one died from severe heart failure after an unsuccessful CRT implant attempt. Follow-up lasted up to 8 years (mean 2.6 ± 2.9 years), during which one patient presented a PM pocket infection, requiring PM explantation and epicardial reimplantation. Conclusion We found a high prevalence of early complications (16.6% pneumothorax) after CIED implantation and an acceptable long-term infectious risk (8.3%). These results highlight the feasibility of CIED implantation in tracheotomized patients with muscular dystrophies and the need for a particular caution in the management of these patients during invasive procedures. ClinicalTrials.gov (identifier: NCT02501083 ).
Highlights • Compliance with non-invasive ventilation is poor in patients with no subjective symptoms of hypercapnia. • Cessation is more frequent in the case of excessive leaks, ventilator ...dysfunction, or high body mass index. • Cessation is less frequent for patients with a professional occupation.
Background The objective was to determine whether optoelectronic plethysmography (OEP) can detect asymmetric ventilation related to unilateral or asymmetric diaphragmatic weakness, suggesting ...usefulness as a diagnostic tool. Methods Thirteen patients with suspected asymmetric diaphragmatic weakness based on dyspnea and hemidiaphragm elevation on the chest radiograph were studied as well as three patients with maltase acid deficiency (a cause of symmetrical diaphragmatic weakness). The transdiaphragmatic pressure response to unilateral magnetic stimulation (lateral twitch transdiaphragmatic pressure latPdiTw) and the diaphragm compound muscle action potentials (CMAPs) elicited by transcutaneous electrical stimulation of each phrenic nerve as well as OEP were performed. Results The CMAPs and latPdiTw showed unilateral or predominantly unilateral diaphragmatic weakness in nine of the 13 patients. By OEP, the affected side of the thorax and abdomen contributed < 45% of the inspiratory capacity in each of these nine patients, whereas no asymmetry was noted in the other four patients or in the three patients with maltase acid deficiency. All patients preferred OEP over CMAP or latPdiTw. Conclusions OEP detected asymmetric ventilation in all patients diagnosed with unilateral diaphragm weakness and in no patients without this diagnosis. Thus, OEP is an effective noninvasive alternative that is preferred by the patients over CMAP response and latPdiTw.
Abstract Purpose Arterial blood gas measurement is frequently performed in critically ill patients to diagnose and monitor acute respiratory failure. At a given metabolic rate, carbon dioxide partial ...pressure (Pa co2 ) is entirely determined by CO2 elimination through ventilation. Transcutaneous partial pressure of carbon dioxide (PtcCO2 ) monitoring permits a noninvasive and continuous estimation of arterial CO2 tension (Pa co2 ). The accuracy of PtcCO2 , however, has not been well studied. To assess the accuracy of different CO2 monitoring methods, we compared PtcCO2 and end-tidal CO2 concentration (EtCO2 ) to Pa co2 measurements in nonintubated intensive care unit (ICU) patients with acute respiratory failure. Methods During a 2-month period, we conducted a prospective observational cohort study in 25 consecutive nonintubated and spontaneously breathing patients admitted to our ICU. Arterial blood gases were measured at study inclusion, 30, 60, and 120 minutes later. At each sampling time, EtCO2 was continuously monitored using a Philips Smart Capnoline Plus, and PtcCO2 was measured using was measured using SenTec device. The aim of the study was to assess agreement between PtcCO2 and Pa co2 and between EtCO2 and Pa co2 in nonintubated ICU patients with acute respiratory failure. Bland-Altman techniques and Pearson correlation coefficients were used. The differences over time (at 30, 60, and 120 minutes) between Pa co2 and EtCO2 and between PtcCO2 and Pa co2 were evaluated using 1-way analysis of variance. Results Transcutaneous partial pressure of carbon dioxide and Pa co2 were well correlated ( R = 0.97), whereas the correlation between EtCO2 and Pa co2 was poor ( R = 0.62) probably due to the presence of an alveolar dead space in a few patients, most notably in the group with chronic obstructive pulmonary disease. The difference over time remained stable for both Pa co2 vs EtCO2 (analysis of variance; P = .88) and Pa co2 vs PtcCO2 ( P = .93). Conclusion We found large differences between EtCO2 and Pa co2 in spontaneously breathing nonintubated ICU patients admitted for acute respiratory failure. Our study argues against the use of EtCO2 monitoring in such patients but raises the possibility that PtcCO2 measurement may provide reasonable estimates of Pa co2.
Abstract Background : Myotonic muscular dystrophy type 1 (MMD1) is the most common form of adult MD, with a mean prevalence of 1 in 8000. Excessive daytime sleepiness (ie, hypersomnia) is a common ...complication of MMD1. Objective : The aim of this study was to evaluate the efficacy and tolerability of modafinil for the treatment of hypersomnia in adults with MMD1. Methods : This multicenter, prospective, randomized, double-blind, placebo-controlled study consisted of a prerandomization period (90 to 2 days before randomization) and a 4-week randomization period in which patients were assigned to receive either active treatment (modafinil 300 mg/d) or placebo. The study was conducted at 3 clinics in France between February 2000 and June 2002. Adult patients aged ≥18 years, with genetically proven MMD1, an Epworth Sleepiness Scale (ESS) score >10, and a mean latency to sleep onset ≤8 minutes measured by the Multiple Sleep Latency Test (MSLT) were eligible. The primary efficacy end point was the Maintenance of Wakefulness Test (MWT) score at 4 weeks. Secondary end points included the mean MSLT score and scores from the ESS, physician's assessment of the therapeutic effect and the patient's global self-assessment via visual analog scale, the 17-item Hamilton Depression Rating Scale, and the Short Form Health Survey (SF-36) quality-of-life assessment. Results : A total of 28 patients (15 men, 13 women; mean SD age, 40 12.7 years range, 18–69 years; 100% white; modafinil group, 13; placebo group, 15) completed the study without protocol violations. Of the 28 patients with MMD1 included in the analysis, 21 had adult-onset MMD1. At 4 weeks, the mean MWT score was 16.4 (3.3) minutes in the modafinil group and 15.8 (3.8) minutes in the placebo group ( P = NS). At the end of the randomization period, there were no significant between-group differences in any secondary outcome. A total of 8 patients (4 in each group) reported ≥1 adverse event, including digestive, neurologic, and skin symptoms. Weight loss was reported in 1 patient (2 kg). Conclusion : In this small study conducted in an adult population with MMD1 and a high prevalence of hyper-somnia, modafinil had no significant effects on daytime somnolence measured using objective MWTs.
To characterize electromyographic abnormalities according to symptoms (asymptomatic, fatigue, pseudobotulism) reported 1 month after botulinum toxin injection.
Retrospective, single-center study ...comparing single-fiber electromyography (SFEMG) in the extensor digitorum communis (EDC) or orbicularis oculi (OO) muscles.
Hospital.
Four groups of adults treated for spasticity or neurologic bladder hyperactivity (N=55): control group (asymptomatic patients: n=17), fatigue group (unusual fatigue with no weakness: n=15), pseudobotulism group (muscle weakness and/or visual disturbance: n=20), and botulism group (from intensive care unit of the same hospital: n=3).
Not applicable.
Mean jitter, percentage of pathologic fibers, and percentage of blocked fibers were compared between groups.
SFEMG was abnormal for 17.6% of control patients and 75% of patients in the pseudobotulism group. There were no differences between the control and fatigue groups. Mean jitter, percentage of pathologic fibers, and percentage of blocked fibers of the EDC muscle were significantly higher in the pseudobotulism group than in the fatigue and control groups. There were no differences between groups for the OO muscle. The SFEMG results in the botulism group were qualitatively similar to those of the pseudobotulism group.
SFEMG of the EDC muscle confirmed diffusion of the toxin into muscles distant from the injection site in the pseudobotulism group. SFEMG in the OO muscle is not useful for the diagnosis of diffusion. No major signs of diffusion of botulinum toxin type A were found away from the injection site in patients with fatigue but no motor weakness. Such fatigue may be related to other mechanisms.
Abstract Bensmail D, Marquer A, Roche N, Godard A-L, Lofaso F, Quera-Salva M-A. Pilot study assessing the impact of intrathecal baclofen administration mode on sleep-related respiratory parameters. ...Objective To assess the impact of intrathecal baclofen (ITB) mode of administration on sleep and sleep-related breathing events in severely disabled patients with severe spasticity. Design Open prospective trial. Setting Physical medicine and rehabilitation department. Participants Patients (N=11) treated with ITB pump for severe spasticity. Intervention Assessment of patients' sleep before and after ITB pump implantation, and comparison of polysomnography results after continuous or bolus mode of administration of ITB. Main Outcome Measures Polysomnography and sleep-related breathing events. Results ITB reduced periodic limb movements and increased the respiratory disturbance index (RDI) and central apneas in our population of patients. This study showed that ITB mode of administration may affect sleep-disordered breathing. Indeed, we observed a significant increase of respiratory events in the bolus condition (RDI and central apneas). In contrast, continuous infusion did not induce a significant modification of sleep-disordered breathing. When a sleep apnea syndrome was preexisting, it was generally severely worsened by the bolus mode of administration. Conclusions These results indicate that sleep function and sleep-related respiratory events should be assessed before ITB pump implantation. It is probably better to use a continuous mode of infusion if patients have preexisting sleep-disordered breathing.
Abstract Laffont I, Guillon B, Fermanian C, Pouillot S, Even-Schneider A, Boyer F, Ruquet M, Aegerter P, Dizien O, Lofaso F. Evaluation of a stair-climbing power wheelchair in 25 people with ...tetraplegia. Objective To compare the performance of a power wheelchair with stair-climbing capability (TopChair) and a conventional power wheelchair (Storm3). Design A single-center, open-label study. Setting A physical medicine and rehabilitation hospital. Participants Patients (N=25) who required power wheelchairs because of severe impairments affecting the upper and lower limbs. Interventions Indoor and outdoor driving trials with both devices. Curb-clearing and stair-climbing with TopChair. Main Outcome Measures Trial duration and Quebec User Evaluation of Satisfaction with Assistive Technology (QUEST) tool; number of failures during driving trials and ability to climb curbs and stairs. Results All 25 participants successfully completed the outdoor and indoor trials with both wheelchairs. Although differences in times to trial completion were statistically significant, they were less than 10%. QUEST scores were significantly better with the Storm3 than the TopChair for weight ( P =.001), dimension ( P =.006), and effectiveness ( P =.04). Of the 25 participants, 23 cleared a 20-cm curb without help, and 20 climbed up and down 6 steps. Most participants felt these specific capabilities of the TopChair—for example, curb clearing and stair climbing—were easy to use (22/25 for curb, 21/25 for stairs) and helpful (24/25 and 23/25). A few participants felt insecure (4/25 and 6/25, respectively). Conclusions The TopChair is a promising mobility device that enables stair and curb climbing and warrants further study.
