The clinical assessment of amyotrophic lateral sclerosis patients' prognosis by ZNF512B gene, neck flexor muscle power score and body mass index (BMI)

BMC Neurol. 2018 Dec 19;18(1):211. doi: 10.1186/s12883-018-1219-9.

Abstract

Background: 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.

Methods: ALS 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.

Results: ALS 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).

Conclusion: The 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.

Keywords: Amyotrophic lateral sclerosis; BMI; Neck flexor muscle power score; Prognosis; Risk allele; Single nucleotide polymorphism; Zinc finger protein 512B gene.

MeSH terms

  • Aged
  • Alleles
  • Amyotrophic Lateral Sclerosis / genetics*
  • Body Mass Index
  • Carrier Proteins / genetics*
  • Disease Progression
  • Female
  • Humans
  • Male
  • Middle Aged
  • Muscle Strength / genetics*
  • Muscle, Skeletal
  • Neck
  • Polymorphism, Single Nucleotide
  • Prognosis

Substances

  • Carrier Proteins
  • ZNF512B protein, human