Abstract:

Objective To analyze the clinical characteristics and epidemiological status of amyotrophic lateral sclerosis (ALS). Methods A total of 110 ALS patients (62 males and 48 females) with a median age of 60 years of onset, were collected. The basic information, site of onset, diagnostic process, modified amyotrophic lateral sclerosis function score (ALSFRS-R), rate of disease progression (ΔFS), electrophysiology and pulmonary function were analyzed comprehensively. The patients were followed up by outpatient or telephone every 3 to 6 months to record changes of their condition, the application of non-invasive positive pressure ventilation (NIPPV), gastric tube, percutaneous gastrostomy (PEG) and the treatment of riluzole. Results Among 110 patients, limb onset accounted for 90 patients (81.8%). The delay diagnosis was 12 (7, 20) months. The misdiagnosis interval was 11 (7, 17) months, and the misdiagnosis rate was 81.3% (74 cases). The most misdiagnosed cases were cervical spondylosis and lumbar spondylosis. The ALSFRS-R score was 39 (36, 45) points, and the ΔFS was 0.6 (0.3, 1.0) points/month. The proportion of confirmed and suspected diagnosis was higher in patients with ball onset than that in patients with limb onset (90% vs. 83%, χ²=2.098, P<0.05). Compared with patients of rapid disease progression, the age of onset was younger in patients with moderate and slow disease progression. Compared with patients of slow disease progression, patients of rapid and moderate disease progression had shorter diagnostic delay. Results of EMG in 90 (81.8%) patients indicated that lower motor neuron lesion segment was greater than or equal to the clinically involved segment. The first pulmonary function examination indicated that 50% (45 cases) patients had restrictive ventilation dysfunction, and the forced vital capacity (FVC)<70% accounted for 17.8% (16 cases). After discharge, the treatment rate of riluzole was 85.6%, and the application rate of NIPPV and PEG was low. During the follow-up, 50 patients (45.5%) died, and the median survival time was 44.0 months. Median survival time was shorter in patients with onset age ≥60 years than that in patients under 60 years (36 months vs. 50 months, P<0.05). Survival time was shorter in patients with rapid disease progression than that in patients with moderate and slow disease progression (27 months vs. 32 months vs. 65 months, P<0.01). Conclusion A full and correct understanding of clinical features of ALS is helpful to make a reasonable diagnosis and treatment plan for patients and improve the prognosis.

Key words: amyotrophic lateral sclerosis, clinical characteristics, neuroelectrophysiological examination, survival