Evaluation of Pulmonary Function Test in Patients with Amyotrophic lateral sclerosis and its correlation with the symptoms of the disease

Document Type: Original Article

Authors

1 Pulmonologist, Lung Diseases Research Center, Mashhad University of Medical Sciences, Mashhad, Iran

2 Community Medicine Specialist, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran

3 Neurologist, Department of Neurology Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran

Abstract

Introduction: Amyotrophic lateral sclerosis (ALS) is a neurogenic progressive disease that leads to muscle atrophy. The purpose of this study was to evaluate pulmonary function test (PFT) in patients with ALS and its correlation with ASL symptoms.
Materials and Methods: This cross-sectional study was performed on 32 ALS patients at Ghaem Hospital, Mashhad, Iran. All patients filled out a demographic form and underwent body plethysmography to determine forced vital capacity (FVC), forced expiratory volume in 1 sec (FEV1), and FEV1/FVC indexes based on their gender and age. Blood samples were also collected to analyze atrial blood gas (ABG) and the levels of oxygen and carbon dioxide. Finally, the data were analyzed by using SPSS20 software.
 
Results: The mean age of the patients was 61.66±13.6 years. The prevalence of ALS was higher in females than in males. The study of the symptoms of the disease (87.1%) of the patients in the study was motor disorder, (0.31%) swallowing disorder, (48.0%) cough and shortness of breath and (40.0%) speech impairment. The results showed that there was a significant relationship between hypercarbia and night oxygen saturation , which the hypercarbia abundance was higher among patients whose night oxygen saturation was SO2 ˂90. But there was no significant relationship between hypercarbia and hypoxemia with symptoms of the disease.. Other results showed that the FEV1 test with swallowing disorder (P = 0.01) and cough and shortness of breath (P = 0.02) the results of FVC test with swallowing disorder (P = 0.01) and cough and shortness of breath (P = 0.02) and Also, there was a significant relationship between FEV1 / FVC test with swallowing disorder (P = 0.01) and cough and shortness of breath (P = 0.01) so that, With the normalization of the Pulmonary Function Test and the improvement of the patients , the symptoms of the disease also decreased.
Conclusions: Overall, the results indicate that early detection of pulmonary involvement in patients with ALS can lead to interventions such as oxygen therapy and reduce symptoms and help improve their quality of life.

Keywords


1. Gruis KL, Lechtzin N. Respiratory therapies for amyotrophic lateral sclerosis: a primer. Muscle Nerve. 2012; 46:313-31.

2. van der Gr aaff MM, Sage CA, Caan MW, Akkerman EM, Lavini C, Majoie CB, et al. Upper and extra-motoneuron involvement in early motoneuron disease: a diffusion tensor imaging study. Brain. 2011; 134:1211-28.

3.Brooks BR, Miller RG, Swash M, Munsat TL. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2000; 1(5):293-9.

4.Moosavi-Movahedi AA, Golchin AR, Nazari KK, Chamani J, Saboury AA, Bathaie SZ, Tangestani-Nejad S. Microcalorimetry, energetics and binding studies of DNA–dimethyltin dichloride complexes. Thermochim Acta. 2004; 414:233-41.

5.Haidet-Phillips AM, Hester ME, Miranda CJ, Meyer K, Braun L, Frakes A, et al. Astrocytes from familial and sporadic ALS patients are toxic to motor neurons. Nat Biotechnol. 2011; 29:824-8.

6. Noh EJ, Park MI, Park SJ, Moon W, Jung HJ. A case of amyotrophic lateral sclerosis presented as oropharyngeal Dysphagia. J Neurogastroenterol Motil. 2010; 16:319-22.

7.Galvin M, Gaffney R, Corr B, Mays I, Hardiman O. From first symptoms to diagnosis of amyotrophic lateral sclerosis: perspectives of an Irish informal caregiver cohort-a thematic analysis. BMJ Open. 2017; 7:e014985.

8. Nicholson TT, Smith SB, Siddique T, Sufit R, Ajroud-Driss S, Coleman JM 3rd, et al. Respiratory pattern and tidal volumes differ for pressure support and volume-assured pressure support in amyotrophic lateral sclerosis. Ann Am Thorac Soc. 2017; 14:1139-46.

9.Chamani J, Heshmati M. Mechanism for stabilization of the molten globule state of papain by sodium n-alkyl sulfates: spectroscopic and calorimetric approaches. J Colloid Interface Sci. 2008; 322:119-27.

10.Tobin K, Gilthorpe MS, Rooney J, Heverin M, Vajda A, Staines A, et al. Age-period-cohort analysis of trends in amyotrophic lateral sclerosis incidence. J Neurol. 2016; 263:1919-26.

11. Ingre C, Roos PM, Piehl F, Kamel F, Fang F. Risk factors for amyotrophic lateral sclerosis. Clin Epidemiol. 2015; 7:181-93.

12. Mehta J, Kamdar V, Dumesic D. Phenotypic expression of polycystic ovary syndrome in South Asian women. Obstet Gynecol Surv. 2013; 68:228-34.

13. Prell T, Ringer TM, Wullenkord K, Garrison P, Gunkel A, Stubendorff B, et al. Assessment of pulmonary function in amyotrophic lateral sclerosis: when can polygraphy help evaluate the need for non-invasive ventilation? J Neurol Neurosurg Psychiatry. 2016; 87:1022-6.

14.Zolfagharzadeh M, Pirouzi M, Asoodeh A, Saberi MR, Chamani J. A comparison investigation of DNP-binding effects to HSA and HTF by spectroscopic and molecular modeling techniques? J Biomol Struct Dyn. 2014; 32:1936-1952.

15. Jackson CE, Rosenfeld J, Moore DH, Bryan WW, Barohn RJ, Wrench M, et al. A preliminary evaluation of a prospective study of pulmonary function studies and symptoms of hypoventilation in ALS/MND patients. J Neurol Sci. 2001; 191:75-8.

16. Radunovic A, Annane D, Rafiq MK, Brassington R, Mustfa N. Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev. 2017; 10:CD004427.

17. Javad Mousavi SA, Zamani B, Shahabi Shahmiri S, Rohani M, Shahidi GA, Mostafapour E, et al. Pulmonary function tests in patients with amyotrophic lateral sclerosis and the association between these tests and survival. Iran J Neurol. 2014; 13:131-7.

18. Chandrasoma B, Balfe D, Naik T, Elsayegh A, Lewis M, Mosenifar Z. Pulmonary function in patients with amyotrophic lateral sclerosis at disease onset. Monaldi Arch Chest Dis. 2012; 77:129-33.

19. Sanei H, Asoodeh A, Hamedakbari-Tusi S, Chamani J. Multi-spectroscopic investigations of aspirin and colchicine interactions with human hemoglobin: binary and ternary systems. J Solution Chem. 2011; 40:1905-31.

20. Perez T. Amyotrophic lateral sclerosis (ALS): evaluation of respiratory function. Rev Neurol (Paris). 2006; 162:4S188-94.

21. Traxinger K, Kelly C, Johnson BA, Lyles RH, Glass JD. Prognosis and epidemiology of amyotrophic lateral sclerosis. Neurol Clin Pract. 2013; 3:313-20.