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Respiratory and Nutritional Support in Amyotrophic Lateral Sclerosis

  • NEUROMUSCULAR DISORDERS (SA RUDNICKI, SECTION EDITOR)
  • Published:
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Opinion statement

Amyotrophic lateral sclerosis (ALS) is an uncommon and almost invariably fatal neurodegenerative disease. There is no known cure for ALS, and only one disease-modifying therapy is currently approved. In the absence of robust pharmacologic treatment options, the value of nutritional and respiratory support in the management of the disease should not be underestimated. The primary causes of morbidity and mortality in ALS are complications from dysphagia, leading to malnutrition and respiratory insufficiency, and these require focused therapeutic attention. This article reviews the current evidence for nutritional and respiratory support in the management of ALS patients.

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Abbreviations

AAN:

American Academy of Neurology

ALS:

Amyotrophic lateral sclerosis

BMI:

Body mass index

DPS:

Diaphragmatic pacing stimulators

EN:

Enteral nutrition

FVC:

Forced vital capacity

HFCWO:

High-frequency chest wall oscillation

IPAP:

Inspiratory airway pressure

MIE:

Mechanical insufflation/exsufflation

MIP:

Maximal inspiratory pressure

MIF:

Maximal mouth-inspiratory force

NIPPV:

Noninvasive positive pressure ventilation

NIV:

Noninvasive ventilation

PCEF:

Peak cough expiratory flow

Pdi:

Transdiaphragmatic pressure

PEG:

Percutaneous endoscopic gastrostomy

PN:

Parenteral nutrition

RIG:

Radiologically inserted gastrostomy

SNIP:

Sniff nasal inspiratory pressure

SVC:

Slow vital capacity

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Conflict of Interest

Namita A. Goyal and Tahseen Mozaffar declare that they have no conflict of interest.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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  1. Department of Neurology, University of California, Irvine, CA, USA

    Namita A. Goyal MD & Tahseen Mozaffar MD

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  1. Namita A. Goyal MD
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Correspondence to Namita A. Goyal MD.

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This article is part of the Topical Collection on Neuromuscular Disorders

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Goyal, N.A., Mozaffar, T. Respiratory and Nutritional Support in Amyotrophic Lateral Sclerosis. Curr Treat Options Neurol 16, 270 (2014). https://doi.org/10.1007/s11940-013-0270-5

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