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Nutritional Consequences of Amyotrophic Lateral Sclerosis

  • Rup TandanEmail author
  • Waqar Waheed
  • Connor Scagnelli
Living reference work entry
First Online:

Abstract

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by degeneration of the motor neurons in the brain, brain stem, and spinal cord. Clinically, there are dysphagia and dysarthria from bulbar involvement and muscle weakness with atrophy that result in profound and progressive weakness, respiratory failure, and death within 3 to 4 years from onset. Malnutrition is present in a substantial proportion of patients, with energy or caloric deficiency predominating. The malnutrition is of multifactorial origin, resulting not only from decreased energy intake but also from increased caloric expenditure related to physical activity and hypermetabolism. Several techniques detect malnutrition, ranging from simple anthropometry to the estimation of body composition to define loss of muscle and body fat, indirect calorimetry to demonstrate increased resting energy expenditure, and doubly labeled water methodology to show increased total daily energy expenditure. Malnutrition has several adverse consequences, including accelerated disease progression and early death. This chapter describes an algorithmic approach to nutritional care in ALS, preferably undertaken through regular visits to a multidisciplinary clinic with staffed experienced healthcare providers. This chapter also provides recommendations for reliably estimating energy intake and expenditure needs of patients to ensure energy balance.

Keywords

Amyotrophic lateral sclerosis Basal metabolic rate Body composition Body mass index Dysphagia Energy expenditure Energy intake Fat mass Fat-free mass Harris-Benedict equation Hypermetabolism Indirect calorimetry Non-exercise activity thermogenesis Percutaneous endoscopic gastrostomy Resting energy expenditure Weight loss 

List of Abbreviations

AAN

American Academy of Neurology

AgRP

Agouti-related peptide

ALS

Amyotrophic lateral sclerosis

ALSFRS-R

Amyotrophic lateral sclerosis functional rating scale-revised

ALS-FTD

Amyotrophic lateral sclerosis-frontotemporal dementia

ALS-P

ALS-plus (cognitive and behavioral changes insufficient for diagnosis of FTD)

AMA

Arm muscle area

APOE

Apolipoprotein E

ATPase

Adenosine triphosphatase

BIA

Bioelectrical impedance analysis

BIS

Bioelectrical impedance spectroscopy

BMI

Body mass index

BMR

Basal metabolic rate

CI

Confidence interval

cREE

Calculated resting energy expenditure

DEXA

Dual-energy x-ray absorptiometry

DLW

Doubly labeled water

ECF

Extracellular fluid

EFNS

European Federation of Neurological Societies

ETF

Enteral tube feeding

FALS

Familial amyotrophic lateral sclerosis

FFM

Fat-free mass

FM

Fat mass

FUS

Fused in sarcoma

FVC

Forced vital capacity

GNRI

Geriatric nutritional risk index

H-B

Harris-Benedict

HR

Hazard ratio

ICF

Intracellular fluid

K+

Potassium

MAC

Mid-arm circumference

MAMC

Mid-arm muscle circumference

mREE

Measured resting energy expenditure

MV

Mechanical ventilation

Na+

Sodium

NEAT

Non-exercise activity thermogenesis

NGT

Nasogastric tube

NIV

Noninvasive ventilation

PA

Phase angle

PCMS

Protein-caloric malnutrition score

PEG

Percutaneous endoscopic gastrostomy

PN

Parenteral nutrition

POMC

Proopiomelanocortin

QOL

Quality of life

RDA

Recommended daily allowance

REE

Resting energy expenditure

RIG

Radiologically inserted gastrostomy

RR

Relative risk

SALS

Sporadic amyotrophic lateral sclerosis

SOD

Superoxide dismutase

T3

Triiodothyronine

T4

Thyroxine

TDEE

Total daily energy expenditure

TDP

Transactive response DNA-binding protein

TSF

Triceps skinfold

UK

United Kingdom

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Authors and Affiliations

  1. 1.Department of Neurological SciencesRobert Larner, MD College of Medicine and University of Vermont Medical CenterBurlingtonUSA

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