Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis: Drug, nutritional, and respiratory therapies (an evidence-based review)

Miller, Robert G.; Jackson, Carlayne E.; Kasarskis, Edward J.; England, J. D.; Forshew, Dalles A.; Johnston, Wendy; Kalra, Sanjay; Katz, José; Mitsumoto, Hiroshi; Rosenfeld, Jeffrey; Shoesmith, Christen; Strong, Michael J.; Woolley, Susan

doi:10.1212/wnl.0b013e3181bc0141

Cited by 690 publications

(632 citation statements)

References 68 publications

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“…Patient acceptance is often better when the intervention is initially used as a supplement to oral caloric intake and not the sole source of nutrition. Patient and family education on the benefits of percutaneous gastrostomy often results in an improved sense of autonomy, confidence, and quality of life [115][116][117]. Patients and families relate that there is a sense of not being able to affect any significant change in disease course heightened by a fear of choking and the caregiver burden.…”

Section: Percutaneous Endoscopic Gastrostomymentioning

confidence: 99%

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

Rosenfeld¹,

Strong

2015

Neurotherapeutics

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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.

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Section: Percutaneous Endoscopic Gastrostomymentioning

confidence: 99%

“…The timing of this intervention is, however, critical as it introduces an important source of variability when considering treatment trials where functional capacity, quality of life, and survival are relevant endpoints [107,122,123].…”

Section: Percutaneous Endoscopic Gastrostomymentioning

confidence: 99%

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

Rosenfeld¹,

Strong

2015

Neurotherapeutics

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“…All subjects received therapeutic procedures (multidisciplinary care, scheduled clinical assessments, nutritional support, psychological management, neurological treatment, and sialorrhoea treatment) in accordance with expert guidelines. 13 Upon NIV initiation, spirometry was assessed with a pneumotachograph spirometer (MS 2000, C Schatzman, Madrid, Spain) in accordance with European Respiratory Society guidelines and suggested values, 14 and P Imax and maximum expiratory pressure were measured (Electrometer 78.905a, Hewlett-Packard, Palo Alto, California) according to the Black and Hyatt technique. 15 Cough peak flow, maximum insufflation capacity, and manually and mechanically assisted cough peak flows were assessed with a sealed oronasal mask (King Systems, Noblesville, Indiana) and a pneumotachograph spirometer (MS 2000) as described previously.…”

Section: Clinical and Functional Impairment Assessmentmentioning

confidence: 99%

Tolerance of Volume Control Noninvasive Ventilation in Subjects With Amyotrophic Lateral Sclerosis

et al. 2015

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BACKGROUND: Noninvasive ventilation (NIV) tolerance has been identified as an independent predictor of survival in amyotrophic lateral sclerosis (ALS). Volume control continuous mandatory ventilation (VC-CMV) NIV has been associated with poor tolerance. The aim of this study was to determine the tolerance of subjects with ALS to VC-CMV NIV. METHODS: This was a prospective study involving subjects with ALS who were treated with VC-CMV NIV. Respiratory and functional parameters were recorded when the subjects began ventilatory support. NIV tolerance was evaluated after 3 months. RESULTS: Eighty-seven subjects with ALS were included. After 3 months, 80 subjects (92%) remained tolerant of NIV. Tolerant subjects presented greater survival (median 22.0 months, 95% CI 14.78 -29.21) than intolerant subjects (median 6.0 months, 95% CI 0.86 -11.13) (P ‫؍‬ .03). The variables that best predicted NIV tolerance were mechanically assisted cough peak flow (P ‫؍‬ .01) and percentage of time spent with S pO 2 < 90% at night while on NIV (P ‫؍‬ .03) CONCLUSIONS: VC-CMV NIV provides high rates of NIV tolerance in subjects with ALS. Mechanically assisted cough peak flow and percentage of time spent with S pO 2 < 90% at night while using NIV are the 2 factors associated with tolerance of VC-CMV NIV in subjects with ALS.

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“…The American Association of Neurology recommends performing vaccination with both pneumovax and influenza for ALS patients 27 . As the respiratory mucosa contributes a constant flow of serous and mucoid fluids, mucolitics like guaifenesin or N-acetilcysteine and/or an anticholinergic bronchodilator like ipratropium and/or theophylline, or even furosemide, may add some benefit in clearing secretions, but there are no controlled studies on ALS patients…”

Section: Vaccination Drugs and Other Therapiesmentioning

confidence: 99%

Current issues in the respiratory care of patients with amyotrophic lateral sclerosis

Orsini

Lopes

Menezes

et al. 2015

Arq. Neuro-Psiquiatr.

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SHORT OVERVIEW OF ALSAmyotrophic lateral sclerosis (ALS) is characterized by progressive degeneration of both cortical and alpha motor neurons of the final common pathway. Despite being a progressive degenerative process which primarily affects motor systems, ALS is now recognized to involve several non-motor systems and in long survivors affects many subcortical structures. Many hypotheses have been formulated about what causes ALS, including chemical exposures, occupational exposure, military service, infectious agents, nutritional intake, physical activity, and trauma. Worldwide, ALS affects white males aged > 60 years more often than any other group 1 . In the past three decades new practice parameters to the clinical care in ALS were developed and several clinical trials were performed². As ALS remains as an incurable, progressive and fatal condition, the treatment strategies are focused in improving the quality of life 3 . The only effective pharmacological treatment is Riluzole, a putative glutamate release blocker linked to modestly prolonged survival 2 . Other therapeutic measures concerning respiratory support are outlined in this paper. PULMONARY FUNCTION IN ALS Respiratory muscle strengthProgressive weakness remains the foremost characteristic of ALS. However, while some ALS patients progress rapidly, others have slow progression of muscle weakness; ABSTRACTAmyotrophic lateral sclerosis is a progressive neuromuscular disease, resulting in respiratory muscle weakness, reduced pulmonary volumes, ineffective cough, secretion retention, and respiratory failure. Measures as vital capacity, maximal inspiratory and expiratory pressures, sniff nasal inspiratory pressure, cough peak flow and pulse oximetry are recommended to monitor the respiratory function. The patients should be followed up by a multidisciplinary team, focused in improving the quality of life and deal with the respiratory symptoms. The respiratory care approach includes airway clearance techniques, mechanically assisted cough and noninvasive mechanical ventilation. Vaccination and respiratory pharmacological support are also recommended. To date, there is no enough evidence supporting the inspiratory muscle training and diaphragmatic pacing.Keywords: amyotrophic lateral sclerosis, respiratory function, rehabilitation. RESUMOEsclerose lateral amiotrófica é uma doença neuromuscular progressiva que resulta em fraqueza muscular, redução dos volumes pulmonares, tosse ineficaz, retenção de secreção e insuficiência respiratória. Medidas como a capacidade vital, pressão inspiratória e pressão expiratória máximas, pressão inspiratória máxima nasal, pico de fluxo de tosse e oximetria de pulso são recomendados para monitorar a função respiratória. Os pacientes devem ser acompanhados por uma equipe multidisciplinar, buscando melhorias na qualidade de vida e melhores estratégias para lidar com os sintomas respiratórios. A abordagem de cuidados respiratórios inclui técnicas de desobstrução das vias respiratórias, tosse assistida mecanicamente e ventilaç...

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Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis: Drug, nutritional, and respiratory therapies (an evidence-based review)

Cited by 690 publications

References 68 publications

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

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

Tolerance of Volume Control Noninvasive Ventilation in Subjects With Amyotrophic Lateral Sclerosis

Current issues in the respiratory care of patients with amyotrophic lateral sclerosis

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