title: Pattern of Respiratory Deterioration in Sporadic Amyotrophic Lateral Sclerosis According to Onset Lesion by Using Respiratory Function Tests.
abstract: Most amyotrophic lateral sclerosis (ALS) patients show focal onset of upper and lower motor neuron signs and spread of symptoms to other regions or the other side clinically. Progression patterns of sporadic ALS are unclear. The aim of this study was to evaluate the pattern of respiratory deterioration in sporadic ALS according to the onset site by using respiratory function tests. Study participants included 63 (42 cervical-onset [C-ALS] and 21 lumbosacral-onset [L-ALS]) ALS patients and 31 healthy controls. We compared respiratory function test parameters among the 3 groups. Age was 57.4±9.6 (mean±SD), 60.8±9, and 60.5±7 years, and there were 28, 15, and 20 male participants, in the C-ALS, L-ALS, and control groups, respectively. Disease duration did not differ between C-ALS and L-ALS patients. Sniff nasal inspiratory pressure (SNIP) was significantly low in C-ALS patients compared with controls. Maximal expiratory pressure (MEP) and forced vital capacity percent predicted (FVC% predicted) were significantly low in C-ALS and L-ALS patients compared with controls. Maximal inspiratory pressure to maximal expiratory pressure (MIP:MEP) ratio did not differ among the 3 groups. Eighteen C-ALS and 5 L-ALS patients were followed up. ΔMIP, ΔMEP, ΔSNIP, ΔPEF, and ΔFVC% predicted were higher in C-ALS than L-ALS patients without statistical significance. Fourteen C-ALS (77.8%) and 3 L-ALS (60%) patients showed a constant MIP:MEP ratio above or below 1 from the first to the last evaluation. Our results suggest that vulnerability of motor neurons in sporadic ALS might follow a topographic gradient.
authors.affiliation: Department of Neurology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seoul 03080, Korea.
authors.forename: Dong-Gun
authors.initials: DG
authors.lastname: Kim
authors.affiliation: Department of Neurology, Seoul National University College of Medicine, Seoul Metropolitan Government Boramae Medical Center, Seoul 03080, Korea.
authors.forename: Yoon-Ho
authors.initials: YH
authors.lastname: Hong
authors.affiliation: Department of Neurology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul 03080, Korea.
authors.forename: Je-Young
authors.initials: JY
authors.lastname: Shin
authors.affiliation: Department of Neurology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul 03080, Korea.
authors.forename: Kwang-Woo
authors.initials: KW
authors.lastname: Lee
authors.affiliation: Department of Neurology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seoul 03080, Korea.
authors.forename: Kyung Seok
authors.initials: KS
authors.lastname: Park
authors.affiliation: Wide River Institute of Immunology, Department of Microbiology and Immunology, Department of Biomedical Sciences, Seoul National University College of Medicine, Seoul 03080, Korea.
authors.forename: Seung-Yong
authors.initials: SY
authors.lastname: Seong
authors.affiliation: Department of Neurology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul 03080, Korea.
authors.forename: Jung-Joon
authors.initials: JJ
authors.lastname: Sung
keywords: Amyotrophic lateral sclerosis; Maximal expiratory pressure; Maximal inspiratory pressure; Progression; Respiratory function test