Abstract

BackgroundKnowledge of prognostic factors related to the survival of Motor Neuron Diseases (MND) remains scarce in Southeast Asia. PurposeTo determine potential prognostic factors for survival, need for feeding and ventilation support in MND patients in a multi-racial Asian population. MethodsOne 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). ResultsMean 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 ± 12.95 years; Non-Malay: 60.39 ± 10.38 years, 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 ± 24.06 months, P = 0.03). 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. ConclusionsBulbar onset was a significant risk predictor for survival. Slower disease progression correlated with better outcomes. Age of onset may differ among ethnic groups. Male patients are more likely to develop Amyotrophic Lateral Sclerosis (ALS) and have shorter survival duration.

Highlights

  • Motor neuron diseases (MND) are a group of neurodegenerative disorders which include amyotrophic lateral sclerosis (ALS), progressive bulbar palsy (PBP), primary lateral sclerosis (PLS) and progressive muscular atrophy (PMA)

  • Bulbar onset was a significant risk predictor for both the need of feeding support (HR=3.87, p

  • 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

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Summary

Methods

One hundred and four MND patients from the Singapore General Hospital (SGH) between January 2004 and December 2017 were reviewed. 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
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