Source: International Congress 2015 – Lung function: exploring the boundaries of the respiratory system
Disease area: Airway diseases

Abstract

Introduction Amyotrophic lateral sclerosis (ALS) is an incurable and highly disabling progressive neurodegenerative disease. The muscle weaknesses encompass all skeletal muscles, including the respiratory muscles, leading to reduced lung volumes and flows.Aims and objectives Study respiratory muscle strength and lung function in a cross-sectional population based study of ALS patients with different phenotypes treated at Haukeland University Hospital, Bergen, Norway.Methods ALS was sub-classified according to bulbar involvement and severity was scored. Forced vital capacity (FVC) and peak expiratory flow (PEF) was performed both seated and supine. Maximal inspiratory (MIP) and expiratory (MEP) pressure, and sniff nasal inspiratory pressure (SNIP) were measured seated.Results

	ALS without bulbar symptoms (n=6) Mean(SD)	ALS with bulbar symptoms (n=14) Mean(SD)	t-test p-value
Gender: Male/Female	6/0	7/7	-
Age, years	65.8(9.2)	69.9(9.4)	.385
Body Mass Index, kg/cm2	23.5(1.8)	23.6(5.1)	.935
ALSFRS-r, 0-48	39.0(7.5)	35.6(8.9)	.433
Bulbar Impairment Scale, 0-8	8.0(0)	5.0(2.27)	.005*
SEATED:
FVC, l	3.38(0.93)	2.34(0.91)	.034*
FVC, % of predicted	73.5(18.8)	64.5(23.7)	.428
PEF, l/min	461.7(140.3)	234.6(109.1)	.001*
MIP, cmH2O	54.2(18.9)	38.6(20.5)	.129
MEP, cmH2O	80.2(32.1)	37.6(18.3)	.001*
SNIP, cmH2O	47.7(22.2)	33.6(13.8)	.126
SUPINE:
FVC, l	2.97(0.50)	2.36(1.20)	.353
FVC, % of predicted	63.5(20.1)	66.3(24.8)	.845
PEF, l/min	395.8(167.7)	233.1(126.1)	.069

 

Conclusions ALS patients had decreased lung function and respiratory muscle strength, and those with bulbar involvement had lower lung volumes, and generated lower pressure and flow during expiration.
*=statistic significant. ALSFRS-r=ALS Functional rating scale-revised

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Citations should be made in the following way:
Astrid Sandnes (Bergen, Norway), Astrid Sandnes, Tiina Andersen, Maria Vollsæter, Ove Fondenes, Ola Røksund, Thomas Halvorsen, Hege Clemm. Lung function and respiratory muscle strength in amyotrophic lateral sclerosis. Eur Respir J 2015; 46: Suppl. 59, 961

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