Objective To investigate the clinical features of motor neuron diseases (MND).Methods A retrospective analysis was performed on the clinical data of 95 MND patients to investigate the features of onset, patterns of progression, and related examinations. The obtained data were summarized and analyzed to increase early diagnosis rate.Results Of the 95 patients, 73 (76.8%) had amyotrophic lateral sclerosis (ALS), 1 (1.1%) had primary lateral sclerosis, 13 (13.7%) had progressive muscular atrophy, and 8 (8.4%) had progressive bulbar palsy. The mean age of onset was 48.85±11.02 years. As to the initial symptoms, 44 cases (46.3%) had upper limb weakness or amyotrophia, 24 cases (25.3%) had lower limb weakness or amyotrophia, 10 cases (10.5%) had limb weakness or amyotrophia, and 17 cases (17.9%) had bulbar paralysis. The male-to-female ratio was 1.26：1. Females were significantly more likely to develop bulbar paralysis or have bulbar paralysis as the initial symptom than males (P<0.05).Conclusions MND is more common in men than in women. The onset is most seen in cervical myotomes. ALS is the most common type of MND. Electrophysiological examination is of great significance for the diagnosis of MND and should be carried out according to the diagnostic criteria for differential diagnosis.