Bipap improves survival and rate of pulmonary function decline in patients with ALS

Mourão

et al. 2008

Arq. Neuro-Psiquiatr.

-Objective:To establish correlations between nutritional, functional and respiratory indices of patients with amyotrophic lateral sclerosis (ALS). Method: Twenty patients (13 appendicular -GA and 7 bulbar -GB) were included in the multidisciplinary study at the Neurological Clinic Ambulatory of the University of Campinas Hospital. Results: Among the GA type significant correlation was observed between maximal inspiratory (MIP) and expiratory (MEP) pressure (r= -0.76), MEP and pulse oxymetry (r=0.58), MIP and percent weight loss (%WL; r=0.59), and between MIP, total and subscale respiratory scores (ALSFRS-R) with %WL. With regard to the GB, correlation was found between MEP and body mass index (BMI) (r=0.97). In both GA and GB correlations were noticed between the BMI and the variables mass (kg), fat (%), arm and wrist circumference (cm), and tricipital, subscapular and supra-iliac skinfolds (mm), as well as the arm muscle circumference (cm) and fatty arm muscular area (mm 2 ). Conclusion: It is suggested that the application of simple anthropometric measurements could be useful in routine monitoring of patients with ALS.KEY WORDS: amyotrophic lateral sclerosis, nutritional support, respiratory tests, ALSFRS scale. esclerose lateral amiotrófica: correlações dos indicadores da avaliação nutricional, funcional e respiratóriaResumo -Objetivo: Correlacionar os indicadores utilizados na avaliação nutricional, funcional e respiratória de indivíduos com esclerose lateral amiotrófica (ELA). Método: Vinte pacientes (13 apendiculares -GA e 7 bulbares -GB) foram incluídos no estudo usando parâmetros nutricionais, respiratórios e escala funcional (ALSFRS-R). Resultados: Entre os pacientes do GA, as correlações observadas foram: pressão inspiratória máxima (PI max ) e expiratória máxima (PE max ) (r= -0,76); PE max e oximetria de pulso (r=0,58); PI max e porcentagem de perda de peso (%PP) (r=0,59); e entre PI max , escore ALSFRS-R com %PP. No GB, houve correlação entre MEP e índice de massa corporal (IMC) (r=0,97). Em GA e GB, observaram-se correlação entre IMC e as variáveis: massa, gordura (%), circunferência braquial e punho, pregas cutâneas tricipital, subescapular e supra-ilíaca, circunferência muscular do braço (cm), área muscular gordurosa do braço (mm 2 ). Conclusão: Sugere-se a aplicação deste conjunto de medidas durante a evolução clínica de indivíduos com ELA. PALAVRAS-CHAVE: esclerose lateral amiotrófica, suporte nutricional, testes respiratórios, escala ALSFRS-R.

Section: Discussionmentioning

confidence: 99%

Amyotrophic lateral sclerosis: combined nutritional, respiratory and functional assessment

Mourão

et al. 2008

Arq. Neuro-Psiquiatr.

“…Inexsufflation and use of high frequency chest wall oscillation are increasingly common and have been shown to improve ventilation [126][127][128][129]. Physical techniques such as breath stacking and assisted cough, as well as medication management (bronchodilators, anticholinergic agents), have become increasingly common in aggressive multidisciplinary treatment settings.…”

Section: Respiratory Therapiesmentioning

confidence: 99%

Challenges in the Understanding and Treatment of Amyotrophic Lateral Sclerosis/Motor Neuron Disease

Rosenfeld¹,

Strong

2015

Neurotherapeutics

With the acceleration in our understanding of ALS and the related motor neuron disease has come even greater challenges in reconciling all of the proposed pathogenic mechanisms and how this will translate into impactful treatments. Fundamental issues such as diagnostic definition(s) of the disease spectrum, relevant biomarkers, the impact of multiple novel genetic mutations and the significant effect of symptomatic treatments on disease progression are all areas of active investigation. In this review, we will focus on these key issues and highlight the challenges that confront both clinicians and basic science researchers.

“…ABOUSSOUAN et al [14] also found that prognosis was improved in patients who could tolerate NIV. More recently, KLEOPA et al [15] have shown improved survival and a slower rate of decline in vital capacity (VC) in ALS/MND patients who were started on NIV once VC had fallen below 50% of the predicted value. Further research has shown an increase in energy and vitality in patients after starting NIV, despite a continuing fall in physical strength [16].…”

Section: Motor Neurone Disease/amyotrophic Lateral Sclerosismentioning

confidence: 99%

Home ventilation: Table 1—

Simonds¹

2003

Eur Respir J

127

Home ventilation is a growth area. Rapid expansion during the 1990s was stimulated by the development of noninvasive ventilation (NIV)viaa mask and the recognition that an increased number of patient groups can benefit. Although patients receiving NIV in the home outnumber those receiving invasive ventilationviatracheostomy, there is substantial variation in practice between European countries.Evidence that individuals who develop ventilatory failure as a consequence of chest wall disease or stable neuromuscular disease such as old poliomyelitis benefit from nocturnal NIV is overwhelming. Patients with progressive neuromuscular disease such as Duchenne muscular dystrophy and amyotrophic lateral sclerosis can also derive prolongation of life, palliation of symptoms and an improvement in quality of life.Home ventilation in chronic obstructive pulmonary disease (COPD) patients remains controversial. Multicentric randomised controlled trials of long-term oxygen therapy (LTOT)versusNIV plus LTOT in COPD have produced mixed results, although certain subgroups,e.g.those with recurrent infective exacerbations requiring short-term NIV, patients aged >65 yrs, and those with uncontrolled hypercapnia on LTOT or symptomatic nocturnal hypoventilation, may benefit.At the other end of the age spectrum, children as young as a few months can be successfully treated with noninvasive ventilation. Most work on paediatric home ventilation centres on children with congenital neuromuscular disease. Pressure preset bilevel ventilators are now the dominant form of ventilator in adults and children. Discharge planning is vital for the home ventilator patient and a sensible risk management strategy should be in place.