Prognostic factors of key outcomes for motor neuron disease in a multiracial Asian population

Abstract Objective To determine potential prognostic factors for survival, need for feeding and ventilation support in motor neuron diseases (MND) patients in a multi-racial Asian population. Methods One hundred and four MND patients from the Singapore General Hospital (SGH) between January 2004 and December 2017 were reviewed. All relevant clinical data, demographic information were collected. Kaplan-Meier and cox regression model were performed to identify potential prognostic factors for crucial outcomes (survival, need for feeding support and ventilation support). Results Mean age of onset was 59.54± 10.91 years, Mean age of onset in Malays was significantly younger than that of other ethnic groups (Malay: 54.18±12.95years; Non-Malay: 60.39±10.38years, p=0.035). Fifty six of the male and 33of the female were diagnosed with ALS (90.3% vs 78.6% p=0.048). Mean overall survival duration from symptom onset was significantly longer in female than male patients (female: 39.2± 29.04 months; male: 29.4± 24.06months, P=0.03). Bulbar onset was a significant risk predictor for both the need of feeding support (HR=3.87, p<0.001) and respiratory aid (HR=3.43, p=0.01).A longer interval from symptom onset to diagnosis was correlated with a longer time to the need for feeding support (HR=0.94, p=0.002) and respiratory aid (HR=0.9, p=0.009). In the univariable cox regression analysis, need for respiratory support (HR=2.1, p=0.03) indicated poor survival outcome significantly, while MND subtypes other than ALS (HR=0.25, p=0.008) and slower disease progression, including longer duration from symptom onset to second symptom (HR=0.97, p=0.005), to feeding support (HR=0.95, p<0.001), to ventilation support (HR=0.96, p=0.004), significantly suggested lower risk of death. In the multivariable cox regression model, bulbar onset (aHR=5.28, p=0.035) correlated with poor survival outcome while longer duration from onset to second symptom (aHR=0.96, P=0.037) indicated better survival. Conclusions Bulbar onset was a significant risk predictor for survival, need of feeding support and ventilation aid. Slower disease progression correlated with better outcomes. Age of onset may differ among ethnic groups. Male patients are more likely to develop ALS and have shorter survival duration.