Time of symptoms beyond the bulbar region predicts survival in bulbar onset amyotrophic lateral sclerosis.

Abstract

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease. Spreading pattern and time interval of spreading are getting more and more attention. The aim of present study was to investigate spreading pattern in bulbar onset ALS patients and to explore the relationship between time interval of spreading and survival. ALS patients with bulbar onset diagnosed at Chinese PLA General Hospital from January 2015 to December 2018 were recruited. Clinical features including gender, onset age, diagnostic delay, the second involved region (SIR), time of symptoms beyond the bulbar region, forced vital capacity (FVC), ALSFRS-R score, electromyography results, and survival time were retrospectively collected. A total of 96 bulbar onset ALS patients were collected. Overall patients showed female predominance. Median age at onset was 56years. Median diagnostic delay was 8.5months. Median time of symptoms beyond the bulbar region (TBBR) was 7months. Median ALSFRS-R score at baseline was 40. Fifty-six (58.3%) patients' SIR were upper limb, 6 (6.3%) patients' SIR were lower limb, 3 (3.1%) patients' SIR were upper and lower limbs, and 5 (5.2%) patients' SIR were thoracic region. Twenty-six (27.1%) patients did not report SIR. The median survival time of patients with TBBR ≥ 7months was significantly longer than that with TBBR < 7month. Multivariate Cox regression showed that onset age and TBBR were prognostic factors. In bulbar onset ALS patients, cervical region is the second most common SIR. TBBR is an independent prognostic factor.