Noninvasive positive-pressure ventilation in ALS Cough augmentation with mechanical insufflation/exsufflation in patients with neuromuscular weakness Inspiratory muscle training and the perception of dyspnea in Parkinson's disease. Expiratory training in multiple sclerosis. INSPIRATIonAL – INSPIRAtory muscle training in amyotrophic lateral sclerosis A clinical pilot study: High frequency chest wall oscillation airway clearance in patients with amyotrophic lateral sclerosis Effects of Mechanical Insufflation‐Exsufflation on Respiratory Parameters for Patients with Chronic Airway Secretion Encumbrance. Effect of Aerobic Training on Walking Capacity and Maximal Exercise Tolerance in Patients With Multiple Sclerosis: A Randomized Crossover Controlled Study High-frequency chest wall oscillation in ALS Breathing-enhanced upper extremity exercises for patients with multiple sclerosis Longitudinal Effects of Noninvasive Positive-Pressure Ventilation in Patients with Amyotrophic Lateral Sclerosis Supramaximal inflation improves lung compliance in subjects with amyotrophic lateral sclerosis. Cough augmentation in amyotrophic lateral sclerosis Effects of a short-term exercise training program on aerobic fitness, fatigue, health perception and activity level of subjects with multiple sclerosis Randomized Control Trial of Effects of a 10-Week Inspiratory Muscle Training Program on Measures of Pulmonary Function in Persons with Multiple Sclerosis Cough determinants in patients with neuromuscular disease Comparison of the influence of different rehabilitation programmes on clinical, spirometric and spiroergometric parameters in patients with multiple sclerosis Respiratory muscle weakness and respiratory muscle training in severely disabled multiple sclerosis patients. Diaphragm Training in Amyotrophic Lateral Sclerosis
Objective : To identify factors associated with tolerance and survival after noninvasive positive-pressure ventilation ( NIPPV ) and to investigate the influence of NIPPV on lung function in patients with ALS . Methods : NIPPV was offered to 71 patients with ALS in accordance with currently published guidelines . Effects of NIPPV on lung function and factors influencing tolerance and survival after NIPPV were studied . Results : Forty-four patients ( 61.9 % ; 95 % CI : 50.6 to 73.2 ) tolerated NIPPV ( NIPPV use ≥4 h/day ) and 27 ( 38.1 % ; 95 % CI : 26.8 to 49.4 ) were intolerant ( NIPPV use < 4 h/day ) . Patients with mild or moderate bulbar symptoms were more likely to tolerate NIPPV than those with severe impairment ( odds ratio = 6.09 , 95 % CI : 1.18 to 31.52 , p = 0.031 ) . After NIPPV introduction , a slower decline in forced vital capacity ( FVC ) was observed in tolerant vs intolerant patients ( p = 0.002 ) . The slope of FVC decline after NIPPV initiation ( risk ratio [ RR ] : 0.78 , 95 % CI : 0.65 to 0.94 , p = 0.01 ) together with NIPPV tolerance ( RR : 0.32 , 95 % CI : 0.13 to 0.78 , p = 0.013 ) were the only independent predictors of survival in the overall group of patients . In multivariate analysis , body mass index was the most powerful predictor of longer survival  after NIPPV in tolerant patients ( RR : 0.77 , 95 % CI : 0.61 to 0.96 , p = 0.022 ) . Conclusion : Survival  after noninvasive ventilation was independently related to ventilatory use ( ≥4 h/day ) and to the modifications of forced vital capacity decline after treatment initiation . The severity of bulbar impairment and the nutritional status of the ALS patients at the introduction of ventilation may predict tolerance and survival Adults and children with neuromuscular disease exhibit weak cough and are susceptible to recurrent chest infections , a major cause of morbidity and mortality . Mechanical insufflation/exsufflation may improve cough efficacy by increasing peak cough flow . It was hypothesised that mechanical insufflation/exsufflation would produce a greater increase in peak cough flow than other modes of cough augmentation . The acceptability of these interventions was also compared . Twenty-two patients aged 10–56 yrs ( median 21 yrs ) with neuromuscular disease and 19 age-matched controls were studied .  Spirometry was performed and respiratory muscle strength measured . Peak cough flow was recorded during maximal unassisted coughs , followed in r and om order by coughs assisted by physiotherapy , noninvasive ventilation , insufflation and exsufflation , and exsufflation alone . Subjects rated strength of cough , distress and comfort on a visual analogue scale . In the neuromuscular disease group , mean±sd forced expiratory volume in one second was 0.8±0.6 L·s−1 , forced vital capacity 0.9±0.8 L , maximum inspiratory pressure 25±16 cmH2O , maximum expiratory pressure 26±22 cmH2O and unassisted peak cough flow 169±90 L·min−1 . The greatest increase in peak cough flow was observed with mechanical insufflation/exsufflation at 235±111 L·min−1 ( p<0.01 ) . All techniques showed similar patient acceptability . Mechanical insufflation/exsufflation produces a greater increase in peak cough flow than other st and ard cough augmentation techniques in adults and children with neuromuscular disease BACKGROUND Pulmonary and respiratory muscle function impairment are common in patients with Parkinson 's disease ( PD ) .  Inspiratory muscle training may improve strength , dyspnea and functional capacity in healthy subjects and in those with chronic obstructive pulmonary disease . This study investigated the effect of specific inspiratory muscle training ( SIMT ) on pulmonary functions , inspiratory muscle performance , dyspnea and quality of life , in patients with PD . PATIENTS AND METHODS Twenty patients with PD ( stage II and III Hoehn and Yahr scale ) were recruited for the study and were divided into two groups : ( a ) ten patients who received SIMT and ( b ) ten patients who received sham training , for three months . Pulmonary functions , the respiratory muscle strength and endurance , the perception of dyspnea ( POD ) and the quality of life were studied before and within one week after the training period . All subjects trained daily , six times a week , each session consisting of 1/2 hour , for 12 weeks . RESULTS Following the training period , there was a significant improvement , in the training group but not in the control group , in the following parameters : inspiratory muscle strength , ( PImax , increased from 62.0 + /- 8.2 to 78.0 + /- 7.5 cm of H2O ( p < 0.05 ) , inspiratory muscle endurance ( increased from 20.0 + /- 2.8 to 29.0 + /- 3.0 cm of H2O ( p < 0.05 ) , and the POD ( decreased from 17.9 + /- 3.2 to 14.0 + /- 2.4 units ( p < 0.05 ) . There was a close correlation between the increase in the inspiratory muscle performance and the decrease in the POD . CONCLUSIONS The inspiratory muscle performance may be improved by SIMT in patients with PD . This improvement is associated with a significant decrease in their POD OBJECTIVE To compare the effects of expiratory muscle training and sham training on respiratory muscle strength in patients with multiple sclerosis ( MS ) . DESIGN A r and omized control trial ; subjects were r and omly assigned to either expiratory muscle training or sham training . SETTING Training and measurement of respiratory muscle pressures were conducted in patients ' homes . Weekly home visits were conducted to assure compliance with the training protocol s and to obtain measurements . PATIENTS Twenty subjects with clinical ly definite MS and decreased expiratory muscle strength entered the study ; 10 subjects completed 3 months of expiratory training using a threshold training device and 5 subjects completed 3 months of sham training using the same device but without an expiratory training threshold load . MEASUREMENT Respiratory muscle strength was assessed at baseline and after 1 , 2 , and 3 months of training ; maximal inspiratory and expiratory pressures were used as measures of respiratory muscle strength . RESULTS There was a significant increase in expiratory muscle strength after 3 months of training when the expiratory training group was compared to the sham group ( p = .003 ) ; no significant change in inspiratory muscle strength was observed . CONCLUSIONS The results of this pilot study suggest that the strength of the expiratory muscles of persons with MS can be increased through respiratory muscle training targeted to the expiratory muscles . Further research is indicated to determine if increasing the strength of the expiratory muscles in MS has an effect on clinical outcomes in this patient population Respiratory impairment , due to respiratory muscle weakness , is a major cause of morbidity and mortality in patients with amyotrophic lateral sclerosis/motor neuron disease ( ALS/MND ) . Threshold loading may strengthen the inspiratory muscles and thereby improve patient prognosis . A phase II , double-blind , r and omized-controlled trial was undertaken to determine whether a 12-week inspiratory muscle training programme attenuated the decline in respiratory function and inspiratory muscle strength in patients with ALS/MND . Nine patients were r and omized to inspiratory muscle training and 10 to sham training . Primary endpoints were respiratory function ( forced vital capacity , vital capacity ) , lung volumes and inspiratory muscle strength . Patients were assessed before , during and immediately after a 12-week training period , and at eight weeks follow-up . While improvements in inspiratory muscle strength were observed in both treatment arms , there was a non-significant increase in maximum inspiratory pressure of 6.1 % in the experimental group compared to controls ( st and ard error of mean , 6.93 % ; 95 % confidence interval −8.58 −20.79 ; p=0.39 ) . The gains in inspiratory muscle strength were partially reversed during a period of training cessation . In conclusion , inspiratory muscle training may potentially strengthen the inspiratory muscles and slow the decline in respiratory function in patients with ALS/MND Respiratory complications are common in patients with amyotrophic lateral sclerosis ( ALS ) with respiratory failure representing the most common cause of death . Ineffective airway clearance result ant from deficient cough frequently contributes to these abnormalities . We sought to evaluate the effectiveness of high frequency chest wall oscillation ( HFCWO ) administered through the Vest ™ Airway Clearance System when added to st and ard care in preventing pulmonary complications and prolonging the time to death in patients with ALS . This is a single center study performed at the Penn State Milton S. Hershey Medical Center ( HMC ) . Nine patients with a diagnosis of ALS and concurrently receiving non‐invasive ventilatory support with bi‐level positive airway pressure ( BiPAP ) were recruited from the outpatient clinic at HMC . Four patients were r and omized to receive st and ard care and five patients to receive st and ard care plus the addition of HFCWO administered twice‐daily for 15 min duration . Longitudinal assessment s of oxyhemoglobin saturation , forced vital capacity ( FVC ) , and adverse events were obtained until time of death . Pulmonary complications of atelectasis , pneumonia , hospitalization for a respiratory‐related abnormality , and tracheostomy with mechanical ventilation were monitored throughout the study duration . No differences were observed between treatment groups in relation to the rate of decline in FVC . The addition of HFCWO airway clearance failed to improve time to death compared to st and ard treatment alone ( 340 days + /− 247 vs. 470 days + /− 241 ; p = 0.26 ) . The r and om allocation of HFCWO airway clearance to patients with ALS concomitantly receiving BiPAP failed to attain any significant clinical benefits in relation to either loss of lung function or mortality . This study does not exclude the potential benefit of HFCWO in select patients with ALS who have coexistent pulmonary diseases , pre‐existent mucus‐related pulmonary complications , or less severe levels of respiratory muscle weakness STUDY OBJECTIVES To analyze the physiologic effects and tolerance of mechanical insufflation-exsufflation ( MI-E ) for patients with chronic ventilatory failure of various etiologies . DESIGN Prospect i ve clinical trial . SETTING Rehabilitation unit of a university hospital . PATIENTS OR PARTICIPANTS Thirteen patients with amyotrophic lateral sclerosis ( ALS ) , 9 patients with severe COPD , and 7 patients with other neuromuscular disorders ( oNMDs ) with chronic airway secretion encumbrance and decreases in oxyhemoglobin saturation ( Spo(2 ) ) . INTERVENTIONS Pressures of MI-E of 15 cm H(2)O , 30 cm H(2)O , and 40 cm H(2)O were cycled to each patient , with 3 s for insufflation and 4 s for exsufflation . One application was six cycles at each pressure for a total of three applications . MEASUREMENTS AND RESULTS We continuously evaluated respiratory inductance plethysmography ( RIP ) and Spo(2 ) during every application . Peak cough flow ( PCF ) and dyspnea ( Borg Scale ) were also measured before the first and after the last application . The technique was well tolerated in all patient groups . Median Spo(2 ) improved significantly ( p < 0.005 ) in all patient groups . Median PCF improved significantly ( p < 0.005 ) in the ALS and oNMD groups from 170 to 200 L/min and from 180 to 220 L/min , respectively , and dyspnea improved significantly in the patients with oNMDs and patients with COPD from 3 to 1 and from 2 to 0.75 , respectively . Breathing pattern characteristics ( RIP ) did not deteriorate after MI-E in any patient groups . Inspiratory flow limitation significantly decreased at the highest MI-E pressures for the ALS group . CONCLUSIONS Our results confirm good tolerance and physiologic improvement in patients with restrictive disease and in patients with obstructive disease , suggesting that MI-E may be a potential complement to noninvasive ventilation for a wide variety of patient groups Background and Purpose Physical deconditioning is involved in the impaired exercise tolerance of patients with multiple sclerosis ( MS ) , but data on the effects of aerobic training ( AT ) in this population are scanty . The purpose of this study was to compare the effects of an 8-week AT program on exercise capacity — in terms of walking capacity and maximum exercise tolerance , as well as its effects on fatigue and health-related quality of life — as compared with neurological rehabilitation ( NR ) in subjects with MS . Subjects and Methods Nineteen subjects ( 14 female , 5 male ; mean age [X̄±SD]=41±8 years ) with mild to moderate disability secondary to MS participated in a r and omized crossover controlled study . Eleven subjects ( 8 female , 3 male ; mean age [X̄±SD]=44±6 years ) completed the study . Results After AT , but not NR , the subjects ’ walking distances and speeds during a self-paced walk were significantly improved , as were their maximum work rate , peak oxygen uptake , and oxygen pulse during cardiopulmonary exercise tests . The increases in peak oxygen uptake and maximum work rate , but not in walking capacity , were significantly higher after AT , as compared with after NR . Additionally , the subjects who were most disabled tended to benefit more from AT . There were no differences between AT and NR in effects on fatigue , and the results showed that AT may have partially affected health-related quality of life . Discussion and Conclusion The results suggest that AT is more effective than NR in improving maximum exercise tolerance and walking capacity in people with mild to moderate disability secondary to MS Objectives : To evaluate changes in respiratory function in patients with ALS after using high-frequency chest wall oscillation ( HFCWO ) . Methods : This was a 12-week r and omized , controlled trial of HFCWO in patients with probable or definite ALS , an Amyotrophic Lateral Sclerosis Functional Rating Scale respiratory subscale score ≤11 and ≥5 , and forced vital capacity ( FVC ) ≥40 % predicted . Results : We enrolled 46 patients ( 58.0 ± 9.8 years ; 21 men , 25 women ) ; 22 used  HFCWO and 24 were untreated . Thirty-five completed the trial : 19 used HFCWO and 16 untreated . HFCWO users had less breathlessness ( p = 0.021 ) and coughed more at night ( p = 0.048 ) at 12 weeks compared to baseline . At 12 weeks , HFCWO users reported a decline in breathlessness ( p = 0.048 ) ; nonusers reported more noise when breathing ( p = 0.027 ) . There were no significant differences in FVC change , peak expiratory flow , capnography , oxygen saturation , fatigue , or transitional dyspnea index . When patients with FVC between 40 and 70 % predicted were analyzed , FVC showed a significant mean decrease in untreated patients but not in HFCWO patients ; HFCWO patients had significantly less increased fatigue and breathlessness . Satisfaction with HFCWO was 79 % . Conclusion : High-frequency chest wall oscillation was well tolerated , considered helpful by a majority of patients , and decreased symptoms of breathlessness . In patients with impaired breathing , high-frequency chest wall oscillation decreased fatigue and showed a trend toward slowing the decline of forced vital capacity Objective : To explore the effectiveness of breathing-enhanced upper extremity exercises on the respiratory function of patients with multiple sclerosis . Design : R and omized controlled study of six-week duration . Subjects : Forty patients with multiple sclerosis ( age 39.2 ± 7 years ; Kurtzke Exp and ed Disability Status Scale scores : 4.51 ± 1.55 ) r and omly divided into two groups . Methods : The training group followed a six-week home training programme design ed to strengthen accessory respiratory muscles . Controls performed no exercises . All subjects su bmi tted to baseline and post-training tests of spirometry , respiratory muscle strength and 6-minute walking . They were also assessed with pulmonary dysfunction and exertion fatigue indices . Results : Spirometry revealed clear improvement in forced expiratory volume in 1 second ( FEV 1 ) ( + 13 % , P = 0.003 ) result ing in higher FEV1/FVC ( forced vital capacity ) ( + 8.5 % , P = 0.03 ) . Maximal inspiratory pressure ( P Imax ) increased by + 7.1 % but not significantly . Maximal expiratory pressure ( P Emax ) and FVC were significantly higher ( by + 7.1 % , P = 0.0066 and + 4.8 % , P = 0.036 respectively ) with respect to baseline measures . Pulmonary dysfunction was reduced ( —9 % , P = 0.002 ) while 6-minute walking distance was longer ( + 16 % , P = 0.029 ) at equal exertion fatigue level . Conclusions : The programme improved most pulmonary performance measures and had clinical significance . Its sustained application may prevent respiratory complications frequently observed in the later stages of multiple sclerosis Butz M , Wollinsky KH , Wiedemuth-Catrinescu U , Sperfeld A , Winter S , Mehrkens HH , Ludolph AC , Schreiber H : Longitudinal effects of noninvasive positive-pressure ventilation in patients with amyotrophic lateral sclerosis . Am J Phys Med Rehabil 2003;82:597–604 . Objective To evaluate the duration of benefit on symptoms , quality of life , and survival derived from the use of noninvasive positive-pressure ventilation by patients with amyotrophic lateral sclerosis . Design In this prospect i ve , cohort study , 30 of 36 consecutively referred symptomatic patients tolerated nightly noninvasive positive-pressure ventilation  and undertook pulmonary function testing and 12 symptom and quality -of-life instruments  concerning sleep quality , daytime sleepiness ,  physical fatigue  ,  mental fatigue  , and  depression  that were administered during a 10-mo period . Results With treatment , there was a significant improvement in the majority of patients in sleep quality , daytime sleepiness , physical fatigue , and depression ; however , significant improvements lasted for up to 10 mo only in sleep quality . Partial pressure of arterial oxygen , partial pressure of arterial carbon dioxide , and oxyhemoglobin saturation remained stable or even improved for up to 7 mo during use of part-time noninvasive positive-pressure ventilation . A total of 14 patients had survival prolonged by continuous dependence on noninvasive positive-pressure ventilation . Conclusions Noninvasive positive-pressure ventilation provides a long-lasting benefit on symptoms and quality of life indicators for amyotrophic lateral sclerosis patients and should be offered to all patients with symptoms of sleep disordered breathing or inspiratory muscle dysfunction . It can also prolong tracheostomy-free survival RATIONALE Lung compliance has been found to be low in patients with chronic diaphragmatic weakness or paralysis but has not been well-studied in patients with amyotrophic lateral sclerosis ( ALS ) .  Noninvasive positive-pressure ventilation ( NPPV ) prolongs survival in ALS patients but may also have additional beneficial effects . OBJECTIVES This study evaluated static expiratory lung compliance ( CL ) in subjects with ALS and determined the effect of lung inflation with supramaximal inflation on CL . DESIGN This was a prospect i ve trial comparing CL before and after supramaximal lung inflation via mouthpiece-delivered positive pressure . SETTING A single university medical center with an multidisciplinary ALS center . PARTICIPANTS Fourteen subjects with ALS were compared to 4 healthy volunteers . INTERVENTIONS Subjects underwent a battery of pulmonary function tests including for CL . Then they used positive pressure administered via a mouthpiece set to 10 cm H2O above their maximal static recoil pressure for 5 min . The CL measurement was then repeated . RESULTS The mean ( + /- SD ) baseline CL was reduced ( 164.1 + /- 82.1 mL/cm H2O ) in subjects with ALS and was significantly lower than that in healthy volunteers ( 237.5 mL/cm H2O ; p = 0.04 ) . CL increased significantly in subjects with evidence of diaphragm weakness ( change in CL , 11.3 + /- 16.7 mL/cm H2O ; p = 0.03 ) . Healthy volunteers did not have an increase in CL . CONCLUSIONS Patients with ALS and diaphragmatic weakness have reduced CL , and brief supramaximal inflation increases CL . These findings suggest that atelectasis or increased alveolar surface forces are present in ALS patients and that these patients will have increased work of breathing . Some of the beneficial effects demonstrated with NPPV therapy may be through its effects on CL and the work of breathing Cough flows and pressures were measured during cough augmentation in healthy subjects and patients with bulbar and nonbulbar amyotrophic lateral sclerosis . Manual assistance increased flow 11 % in bulbar ( p < 0.01 ) and 13 % in nonbulbar ( p < 0.001 ) patients . Mechanical insufflation-exsufflation increased flow 17 % in healthy subjects ( p < 0.05 ) , 26 % ( p < 0.001 ) in bulbar , and 28 % ( p < 0.001 ) in nonbulbar patients . The greatest improvements were in patients with the weakest coughs . Patient group and level of weakness influenced the effect of augmentation Multiple sclerosis ( MS ) patients of an inpatient rehabilitation program have been r and omly assigned to an exercise training ( MS-ET ) or nontraining group ( MS-NI ) . Before and after 4 weeks of aerobic exercise training , a grade d maximal exercise test with measurement of gas exchange and a lung function test was administered to all 26 patients fulfilling the inclusion criteria .  Activity level , fatigue and health perception were measured by means of question naires . Twenty-six healthy persons served as control group and were matched in respect of age , gender and activity level . Training intervention consisted of 5 × 30 min sessions per week of bicycle exercise with individualised intensity . Compared with baseline , the MS training group demonstrated a significant rightward placement of the aerobic threshold ( AT ) ( VO2 + 13 % ; work rate [WR])+11 % ) , an improvement of health perception ( vitality+46 % ; social interaction+36 % ) , an increase of activity level ( + 17 % ) and a tendency to less fatigue . No changes were observed for the MS-NI group and the control groups . Maximal aerobic capacity and lung function were not changed by either training or nontraining in all four groups . Overall compliance to the training program was quite low ( 65 % ) , whereas incidence of symptom exacerbation by physical activity has been lower than expected ( 6 % ) Pulmonary impairments have long been recognized as major causes of morbidity and mortality in individuals with advanced multiple sclerosis ( MS ) . This study was design ed to determine if a 10-week home exercise inspiratory training program in community-dwelling persons with MS improves pulmonary muscle strength and endurance .  Forty-six ambulatory individuals with clinical ly diagnosed MS [ Exp and ed Disability Status Scale ( EDSS ) 2.0–6.5 , intervention group mean = 3.96 and control group mean = 3.36 ] were r and omly assigned to an intervention group that received 10 weeks of inspiratory muscle strength training ( IMT ) or a nontreatment control group .  Twenty-one subjects in the control group and 20 subjects in the intervention group completed the study . The intervention group demonstrated significantly greater improvement than the control group in maximal inspiratory pressure ( P < 0.001 ) . When compared to the control group , no significant differences were noted for maximal expiratory pressure or maximal ventilation volume after training in the intervention group . Baseline and postexercise training comparison of secondary pulmonary expiratory outcomes were significant in the intervention group for forced expiratory volume at one second ( FEV1 ) ( P = 0.014 ) , forced vital capacity ( FVC ) ( P = 0.041 ) , and midexpiratory flow rate(FEF25–75 % ) ( P = 0.011 ) . No significant changes were noted for the control group . Thus , IMT significantly increased inspiratory muscle strength and result ed in generalized improvements in expiratory pulmonary function in persons with MS who have minimal to moderate disability . Future studies are needed that focus on the long-term effects of IMT with increased resistance and the impact it has on increasing pulmonary function and functional performance Neuromuscular disease leads to cough impairment . Cough augmentation can be achieved by mechanical insufflation ( MI ) or manually assisted coughing ( MAC ) . Many studies have compared these two methods , but few have evaluated them in combination . In 155 neuromuscular patients , we assessed determinants of peak cough flow ( PCF ) using stepwise correlation . Maximal inspiratory capacity contributed 44 % of the variance ( p<0.001 ) , expiratory reserve volume 13 % , and maximal expiratory pressure 2 % . Thus , augmenting inspiration seems crucial . However , parameters dependent on expiratory muscles independently influence PCF . We measured vital capacity and PCF in 10 neuromuscular patients during cough augmentation by MI , MAC , or both . MI or MAC significantly improved VC and PCF ( p<0.01 ) as compared to the basal condition and VC and PCF were higher during MI plus MAC than during MAC or MI alone ( p<0.01 ) . In conclusion , combining MAC and MI is useful for improving cough in neuromuscular patients Purpose The aim of this study was to compare the effect of four different programmes on spiroergometric , spirometric and clinical parameters in multiple sclerosis ( MS ) patients . Methods One hundred and twelve MS patients were divided into four groups . The first group underwent neurophysiologically based physiotherapy , the second aerobic training , the third combined therapy ( neurophysiologically based physiotherapy and aerobic training ) and the fourth did not change any habits .  Seventeen patients did not finish the study . Patients were examined on impairment ( Exp and ed Disability Status Scale ) , disability ( Barthel Index ) , h and icap ( Environment Status Scale ) , quality of life ( Multiple Sclerosis Quality of Life ) , fatigue ( Modified Fatigue Impact Scale ) , depression ( Beck Depression Inventory Score ) , respiratory function ( spirometric parameters on spirometry ) and physical fitness ( spiroergometric parameters on a bicycle ergometer ) . Results The patients who participated in one of our training programmes showed a significant improvement of the examined parameters in comparison to those who did not change their present habits . Each of the four training programmes had a different impact on the parameters , which means that each of them had a different effect . The neurophysiologically based physiotherapy had the greatest impact on impairment , and the aerobic training on spirometric and spiroergometric parameters . All methods ( the neurophysiologically based physiotherapy , the aerobic training and the combined programme ) had an impact on fatigue OBJECTIVE To evaluate the contribution of respiratory muscle weakness ( part 1 ) and respiratory muscle training ( part 2 ) to pulmonary function , cough efficacy , and functional status in patients with advanced multiple sclerosis ( MS ) . DESIGN Survey ( part 1 ) and r and omized controlled trial ( part 2 ) . SETTING Rehabilitation center for MS . PATIENTS Twenty-eight bedridden or wheelchair-bound MS patients ( part 1 ) ; 18 patients were r and omly assigned to a training group ( n = 9 ) or a control group ( n = 9 ) ( part 2 ) . INTERVENTION The training group ( part 2 ) performed three series of 15 contractions against an expiratory resistance ( 60 % maximum expiratory pressure [ PEmax ] ) two times a day , whereas the control group performed breathing exercises to enhance maximal inspirations . MAIN OUTCOME MEASURES Forced vital capacity ( FVC ) , inspiratory , and expiratory muscle strength ( PImax and PEmax ) , neck flexion force ( NFF ) , cough efficacy by means of the Pulmonary Index ( PI ) , and functional status by means of the Extended Disability Status Scale ( EDSS ) . RESULTS Part 1 revealed a significantly reduced FVC ( 43 % + /- 26 % predicted ) , PEmax ( 18 % + /- 8 % predicted ) , and PImax ( 27 % + /- 11 % predicted ) , whereas NFF was only mildly reduced ( 93 % + /- 26 % predicted ) . The PI ( median score , 10 ) and EDSS ( median score , 8.5 ) were severely reduced . PEmax was significantly correlated to FVC , EDSS , and PI ( r = .77 , -.79 , and -.47 , respectively ) . In stepwise multiple regression analysis . PEmax was the only factor contributing to the explained variance in FVC ( R2 = .60 ) , whereas body weight ( R2 = .41 ) was the only factor for the PI . In part 2 , changes in PImax and PEmax tended to be higher in the training group ( p = .06 and p = .07 , respectively ) . The PI was significantly improved after 3 months of training compared with the control group ( p < .05 ) . After 6 months , the PI remained significantly better in the training group . CONCLUSIONS Expiratory muscle strength was significantly reduced and related to FVC , cough efficacy , and functional status . Expiratory muscle training tended to enhance inspiratory and expiratory muscle strength . In addition , subjectively and objective ly rated cough efficacy improved significantly and lasted for 3 months after training cessation There is a continued need for therapies to improve respiratory function and quality of life in patients with amyotrophic lateral sclerosis . In this pilot trial , we studied 8 subjects with amyotrophic lateral sclerosis and respiratory involvement . We measured respiratory function and quality of life at baseline . We then taught subjects diaphragm training , a method of breath control design ed to improve respiratory muscle strength and efficiency . We repeated measures of respiratory function and quality of life 6 and 12 weeks later . There was no significant improvement in any outcome measure after instituting diaphragm training . There was a nonsignificant trend toward a slower rate of decline in respiratory function in those subjects who mastered the technique ; however , only half the subjects were able to successfully change their pattern of breathing . A larger study of this technique is warranted ; however , in determining the sample size for a larger study , the difficulty for patients in mastering the technique must be taken into consideration
BACKGROUND Respiratory failure and dysfunction are common problems in many neurodegenerative conditions .
Although physiotherapists manage these problems , it is not known which treatments have been studied and their efficacy .
OBJECTIVE To review evidence on the management of respiratory problems in people with neurodegenerative conditions using the PRISMA approach .