Swallowıng and respıratıon relatıonshıp ın amyotrophıc lateral sclerosıs

Amyotrophic lateral sclerosis (ALS) is progressive neurodegenerative disease effect upper and lower motor neurons. Difficulty in swallowing and respiratory muscle weakness is the most important factors in prognosis of ALS. Our purpose in this study is investigating relationship breathing and swallowing dysfunction in ALS patients. The patients diagnosed with ALS by El Escorial diagnostic criteria were included to the study. ALS functional rating scale scoring was used for every included ALS patients. Conventional brain MRI, MR Spectroscopy, Diffusion Tensor MR was performed to patients. Motor evoked potentials were recorded for examination of the corticospinal tract involvement. Pulmonary functions were evaluated with sniff nasal pressure (SNIP) test that defined as an alternative for forced vital capacity (FVC). Swallowing function of patients was checked with dysphagia limit accompanied by EMG. Laryngeal movement sensor, superficial submental EMG activity, air flow sensor, chest movement sensor and diaphragm EMG were used to evaluate relationship swallowing and breathing. Swallowing parameters such as A-C, A-O, O-2, dyspagia limit in addition to respiration parameters such as the duration of the inspiration and expiration respiratory phases with or without swallowing as well as swallowing apnea was measured. Twenty seven patients were included to study. 20 cases (74,7%) were men, 7 cases were women. Patients' age ranged from 30 to 75 years and the mean age was 53±12 years. The average inclusion time after diagnosis were 24,7±27,3 months. In 5 cases disease had started with bulbar signs and dysphagia limits and respiratory function tests were abnormal in these cases. In 22 cases (81%) disease had started from limbs. Dysphagia limit was abnormal in 13 patients (< 15 cc). Abnormality related with respiratory function was detected in 9 of the dysphagic 13 patients. Dysphagia limit also correlated with Forced Vital Capacity and SNIP. Respiratory and swallowing function tests were normal in 11 ALS patients. All cases could swallow during the expiration period of the respiration. Twenty cases could swallow during the inspiration. Dysphagic seven patients could not swallow during the inspiration period of the respiration. Even if all of the cases whose have no swallowing difficulty could swallow during the inspiration period of respiration, but 53% of the cases whose have swallowing difficulty could not swallow during the inspiration of the respiration period. Dysphagia limits of all cases who were not able to swallow during the inspiration period were lower especially than 5 cc. There was statistically significant negative correlation between Forced vital capacity and prolongation of the inspiration interval due to swallowing. We could not find any correlation between dysphagia limit and swallowing associated respiration phases (neither with swallowing nor without swallowing). According to comparison of nine cases that had dysphagia and abnormal respiratory function and eleven cases that had not dysphagia and abnormal respiratory function, there was no significant correlation between respiratory phase's duration (with/ without swallowing) and swallowing apnea duration. Brain MRI detected T2 intensity increase of the corticospinal tract in 2 cases. MEP abnormalities related with corticospinal tract involvement was found in 19/24 patients. There wasn't significant difference of the FA parameters in the MR tractography between patients and normal healthy subjects. In this study it is noticed that TMS is more sensitive than MR imaging for detecting upper motor neuron involvement in ALS cases. Moreover there was not a significant association in terms of TMS abnormality with dysphagia occurrence. Swallowing and respiration functions are determining the prognoses of the ALS patients. Determining of the dysphagia, and relieving of this big problem, "dysphagia limit" may be considered as assistance for clinical practice