Thesis (M.Sc.) - Cairo University - Faculty of Medicine - Department of Neurophysiology

Background: Respiratory and bulbar dysfunctions are the leading causes of death in amyotrophic lateral sclerosis disease (ALS).The function of the diaphragm as the primary muscle of respiration can be assessed by phrenic nerve conduction studies and diaphragm ultrasound. In addition, the findings of the tongue ultrasound can be used to represent the bulbar function. Subjects and methods: Eleven patients with definite ALS; 8 males and 3 females with mean age of 44.55 ± 8.90 years participated in the study. All patients were subjected to bilateral phrenic nerve conduction studies, ultrasound of the diaphragm and ultrasound examination of the tongue. Results: The amplitude of the CMAP of the phrenic nerve was reduced in 54.4% of patients.The diaphragm excursion was abnormal in 9.09% of patients and the respiratory thickening ratio was normal in all patients.This showed a statistically significant difference between phrenic nerve amplitude and diaphragm ultrasound (p value=0.022). The phrenic nerve amplitude was significantly correlated with the revised ALS functional rating scale (ALSFRS-R) respiratory sub-score (p=0.019) while diaphragm ultrasound was not.Tongue echo intensity showed a sensitivity of 63.6%

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