Abstract

BackgroundAssessment on the prognosis of amyotrophic lateral sclerosis (ALS) is becoming a focus of research in recent years since there is no effective treatment. The aim of the research is to explore the major factors involving in prognosis of ALS patients through long-term follow-up.MethodsALS patients’ DNA extracted from peripheral blood white cells were detected for the risk allele by single nucleotide polymorphism (SNP) analysis. Neck flexor muscle score and body mass index (BMI) were recorded during Medical Research Council follow-up using manual muscle testing method.ResultsALS patients with risk alleles (C) deteriorated rapidly with poor clinical outcome. It seemed that the higher neck flexor muscle strength score in ALS patients with the longer survival time but without significant correlation (p > 0.05). The lower the basal body mass index, the shorter the survival time and the faster deterioration (p < 0.05). The patients with body mass index less than 22.04 seemed to have short survival time than those with BMI more than 22.04 (p < 0.05), however, the speed of deterioration in two groups of patients had no significant difference (p > 0.05).ConclusionThe risk (C) allele of the SNP (rs2275294) in the ZNF512B gene, cervical flexor muscle power and body weight index might have clinical potential for ALS prognostication, since these indicators is so simple to perform that they might be very suitable for primary clinics and even community medical institutions to carry out.

Highlights

  • Assessment on the prognosis of amyotrophic lateral sclerosis (ALS) is becoming a focus of research in recent years since there is no effective treatment

  • Enrollment criteria: All of the subjects was collected from Northeast Territory of China during 2012~2016 who were diagnosed as ALS according to both clinical symptoms, signs and electromyography (EMG) positive manifestation

  • The diagnosis was confirmed by two neurologist who had been engaged in clinical practice against ALS for years

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Summary

Introduction

Assessment on the prognosis of amyotrophic lateral sclerosis (ALS) is becoming a focus of research in recent years since there is no effective treatment. The aim of the research is to explore the major factors involving in prognosis of ALS patients through long-term follow-up. As a fatal progressive neurodegenerative disease, there is not any effective treatment for amyotrophic lateral sclerosis (ALS), the average survival duration might merely be 2 to 5 years. Since no advance could be expected in the predictable future years, the evaluation of its prognosis has become a more and more.

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