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Respiratory and Nutritional Support in Amyotrophic Lateral Sclerosis

  • Namita A. Goyal
  • Tahseen Mozaffar
NEUROMUSCULAR DISORDERS (SA RUDNICKI, SECTION EDITOR)
Part of the following topical collections:
  1. Topical Collection on Neuromuscular Disorders

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.

Keywords

Amyotrophic lateral sclerosis ALS Respiratory support Nutritional support Respiratory insufficiency Noninvasive ventilation Percutaneous endoscopic gastrostomy Enteral nutrition Diaphragmatic pacing 

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

Notes

Compliance with Ethics Guidelines

Conflict of Interest

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

Human and Animal Rights and Informed Consent

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

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Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Department of NeurologyUniversity of CaliforniaIrvineUSA

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