Prognostic role of slow vital capacity in amyotrophic lateral sclerosis

Calvo, Andrea; Vasta, Rosario; Moglia, Cristina; Matteoni, Enrico; Canosa, Antonio; Mattei, Alessio; Mancusa, Claudio La; Focaraccio, Luana; Mazzini, Letizia; Chiò, Adriano; D’Ovidio, Fabrizio; Manera, Umberto

doi:10.1007/s00415-020-09751-1

Cited by 25 publications

(33 citation statements)

References 37 publications

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“…due to air leakage or orthopnea), were allowed to perform a slow vital capacity (SVC) test in supine position. Literature has shown that the results of the SVC and FVC are very similar and interchangeable [22,23]. We, therefore, report the supine VC.…”

Section: Pulmonary Function Testmentioning

confidence: 88%

Using patient-reported symptoms of dyspnea for screening reduced respiratory function in patients with motor neuron diseases

et al. 2020

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Background Poor monitoring of respiratory function may lead to late initiation of non-invasive ventilation (NIV) in patients with motor neuron diseases (MND). Monitoring could be improved by remotely assessing hypoventilation symptoms between clinic visits. We aimed to determine which patient-reported hypoventilation symptoms are best for screening reduced respiratory function in patients with MND, and compared them to the respiratory domain of the amyotrophic lateral sclerosis functional rating scale (ALSFRS-R). Methods This prospective multi-center study included 100 patients with MND, who were able to perform a supine vital capacity test. Reduced respiratory function was defined as a predicted supine vital capacity ≤ 80%. We developed a 14-item hypoventilation symptom questionnaire (HYSQ) based on guidelines, expert opinion and think-aloud interviews with patients. Symptoms of the HYSQ were related to dyspnea, sleep quality, sleepiness/fatigue and pneumonia. The diagnostic performances of these symptoms and the ALSFRS-R respiratory domain were determined from the receiver operating characteristic (ROC) curves, area under the curve (AUC), sensitivity, specificity, predictive values and accuracy. Results Dyspnea-related symptoms (dyspnea while eating/talking, while lying flat and during light activity) were combined into the MND Dyspnea Scale (MND-DS). ROC curves showed that the MND-DS had the best diagnostic performance, with the highest AUC = 0.72, sensitivity = 75% and accuracy = 71%. Sleep-quality symptoms, sleepiness/fatigue-related symptoms and the ALSFRS-R respiratory domain showed weak diagnostic performance. Conclusion The diagnostic performance of the MND-DS was better than the respiratory domain of the ALSFRS-R for screening reduced respiratory function in patients with MND, and is, therefore, the preferred method for (remotely) monitoring respiratory function.

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Section: Pulmonary Function Testmentioning

confidence: 88%

Using patient-reported symptoms of dyspnea for screening reduced respiratory function in patients with motor neuron diseases

et al. 2020

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“…The identification of factors able to predict such a different evolution would be of crucial importance to improve clinical trials’ designs and for neurologists to better schedule patients’ interventions. Several clinical and demographic characteristics and lifestyle habits have been identified as ALS prognostic factors: age at diagnosis, diagnostic delay, onset type, cognitive status [3] respiratory functionality at diagnosis [4] early weight loss [5] and cigarette smoking [6]. Recently, some of these factors have been grouped in complex models in order to better predict ALS patients’ survival [7–9].…”

Section: Introductionmentioning

confidence: 99%

Neck flexor weakness at diagnosis predicts respiratory impairment in amyotrophic lateral sclerosis

Vasta

Torrieri

D’Ovidio

et al. 2020

Euro J of Neurology

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Background and purpose The purpose was to assess the prognostic role of neck muscle weakness at diagnosis in amyotrophic lateral sclerosis (ALS) patients with respect to survival and respiratory impairment. Methods A retrospective cohort study was conducted. All ALS patients seen in the Turin ALS Centre from 2007 to 2014 were included. Muscle strength at diagnosis was evaluated using the Medical Research Council (MRC) scale. Survival was considered as the time from diagnosis to death or tracheostomy; time to respiratory impairment was considered as the interval from diagnosis to the first event amongst an ALS Functional Rating Scale revised item 10 <4, forced vital capacity <70%, start of non‐invasive ventilation or tracheostomy. Time from diagnosis to dysarthria, dysphagia and walking impairment were considered as secondary outcomes. Cox proportional hazard regression models adjusted for sex, age at diagnosis, diagnostic delay, onset site, genetics status and the MRC scores of other muscle groups were used to assess the prognostic role of neck muscles. Results A total of 370 patients were included in the study. Fifty‐nine (15.9%) patients showed neck flexor weakness at diagnosis; MRC values were mostly in agreement for neck extensors. Neck flexors were the only muscles able to predict survival (hazard ratio 0.49, 95% confidence interval 0.28–0.86; p = 0.01). Furthermore, neck flexor normal strength decreased the risk of respiratory impairment (hazard ratio 0.46, 95% confidence interval 0.22–0.96; p = 0.04) but did not influence any secondary outcomes. Discussion Neck flexor weakness at diagnosis predicts survival and respiratory impairment in ALS. This result could be valuable for both planning of patients’ interventions and clinical trials’ design.

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“…A Esclerose Lateral Amiotrófica (ELA) é uma doença que se manifesta pela degeneração dos neurônios motores superiores e inferiores [1][2][3] . A função desses neurônios é transmitir mensagens para um músculo específico ou grupos musculares, uma vez que, com sua degradação, essas mensagens param de ser transmitidas, levando à paresia dos músculos estriados, causando disfagia progressiva, disartria, imobilidade, dependência completa e acometimentos respiratórios 2,4 .…”

Section: Introductionunclassified

“…Todos os pacientes com ELA apresentam ou apresentarão insuficiência respiratória, pois, em 97-98% dos casos, a função pulmonar diminui durante o curso da doença de forma progressiva 5 . A expectativa de vida média, após o diagnóstico, é de aproximadamente três a cinco anos 6 , a grande maioria evolui para óbito devido a acometimentos de ordem respiratória 4 .…”

Section: Introductionunclassified

“…Alguns estudos investigaram a evolução do curso clínico respiratório da doença. Calvo et al (2020) concluíram em seu recente estudo que a capacidade vital forçada (CVF) e capacidade vital lenta (CVL) podem ser utilizadas como preditores de sobrevida em pacientes com ELA 4 . Todavia são escassos na literatura estudos que investigaram as manifestações da doença em relação às questões respiratórias, tais como: hipoventilação alveolar e seus sintomas, além das capacidades e funções respiratórias.…”

Section: Introductionunclassified

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Perfil epidemiológico, função pulmonar e incidência de hipoventilação dos pacientes com Esclerose Lateral Amiotrófica de um hospital referência de uma capital brasileira: um estudo transversal retrospectivo

Polese¹,

Pessoa²

2020

Conexão Ci.

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Introdução: A Esclerose Lateral Amiotrófica (ELA) é uma doença que se manifesta pela degeneração dos neurônios motores superiores e inferiores. A degeneração dos neurônios provoca a perda de contração muscular voluntária e o controle dos movimentos, de forma importante sobre os músculos respiratórios, o que leva à uma expectativa de vida máxima de três a cinco anos após o início dos sintomas. As evidências sugerem que o percurso clínico da doença está altamente relacionado a funções e capacidades respiratórias. Objetivo: Traçar o perfil epidemiológico, função pulmonar e incidência de hipoventilação de pacientes portadores de ELA. Metodologia: Estudo transversal retrospectivo, com coletas de dados de prontuários dos pacientes que já vieram a óbito atendidos no setor Distrofias do Hospital Júlia Kubitschek de Belo Horizonte. Resultados: Foram coletados 46 prontuários de paciente com ELA com média de idade de 59,98±11,66 com 63,8% do sexo masculino e 84,8% com diagnóstico de ELA esporádica. O tempo de evolução da doença foi em média de 3,37±2,87 anos. Apresentaram em média capacidade vital de 1,19±0,57 litros e com presença de hipoventilação alveolar em 90,3% dos indivíduos. Conclusão: A grande maioria dos dados foram compatíveis com a literatura atual e com o curso clínico da doença, porém ainda são necessários mais estudos dessas variáveis para melhor caracterização desses pacientes.

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Prognostic role of slow vital capacity in amyotrophic lateral sclerosis

Cited by 25 publications

References 37 publications

Using patient-reported symptoms of dyspnea for screening reduced respiratory function in patients with motor neuron diseases

Using patient-reported symptoms of dyspnea for screening reduced respiratory function in patients with motor neuron diseases

Neck flexor weakness at diagnosis predicts respiratory impairment in amyotrophic lateral sclerosis

Perfil epidemiológico, função pulmonar e incidência de hipoventilação dos pacientes com Esclerose Lateral Amiotrófica de um hospital referência de uma capital brasileira: um estudo transversal retrospectivo

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