Discontiguous or Contiguous Spread Patterns Affect the Functional Staging in Patients With Sporadic Amyotrophic Lateral Sclerosis

Front Neurol. 2019 May 22:10:523. doi: 10.3389/fneur.2019.00523. eCollection 2019.

Abstract

Objective: This study aimed to investigate whether the spread pattern affects functional staging in amyotrophic lateral sclerosis (ALS). We examined the spreading patterns of disease following symptom onset and the affected regions in ALS using electromyography. Methods: This study reviewed the medical records of 103 patients with sporadic ALS in the Second Hospital of Hebei Medical University from 2012 to 2017. According to the clinical manifestation and the distribution of the affected regions on electromyography, spread patterns were classified as discontiguous or contiguous. The patients were graded according to the ALS-Milano-Torino staging (MITOS) system. Results: The clinical spread patterns were contiguous in 91.5% of patients and discontiguous in 8.5% of patients. The electrophysiological spread patterns were contiguous in 87.4% of patients and discontiguous in 12.6% of patients. Sex, age, or delay in diagnosis did not affect the clinical or electrophysiological spread patterns. No significant correlation was observed between the clinical classification and the ALS-MITOS grade, but the electrophysiological spread was significantly correlated with the ALS-MITOS. Conclusion: This study provides evidence that not all ALS patients show contiguous clinical or electrophysiological spread patterns. The electrophysiological spread pattern can affect the functional staging in ALS patients.

Keywords: Amyotrophic lateral sclerosis; electrophysiology; functional staging; prion-like mechanism; spread pattern.