中国神经再生研究(英文版) ›› 2023, Vol. 18 ›› Issue (9): 1903-1907.doi: 10.4103/1673-5374.367833

• 综述:退行性病与再生 • 上一篇    下一篇

多系统萎缩的肛门外括约肌肌电图:诊断意义、临床相关性和对预后的新见解

  

  • 出版日期:2023-09-15 发布日期:2023-03-06

External anal sphincter electromyography in multiple system atrophy: implications for diagnosis, clinical correlations, and novel insights into prognosis

Massimiliano Todisco1, 2, 3, *, Giuseppe Cosentino1, 3, Enrico Alfonsi1   

  1. 1Translational Neurophysiology Research Unit, IRCCS Mondino Foundation, Pavia, Italy; 2Movement Disorders Research Center, IRCCS Mondino Foundation, Pavia, Italy; 3Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy
  • Online:2023-09-15 Published:2023-03-06
  • Contact: Massimiliano Todisco, MD, massimiliano.todisco@mondino.it.
  • Supported by:
    This work was supported by the Italian Ministry of Health (‘Ricerca Corrente’ 2020–2021) (to MT).

摘要: https://orcid.org/0000-0002-6527-3558 (Massimiliano Todisco)

Abstract: Multiple system atrophy is a sporadic, progressive, adult-onset, neurodegenerative disorder characterized by autonomic dysfunction symptoms, parkinsonian features, and cerebellar signs in various combinations. An early diagnosis of multiple system atrophy is of utmost importance for the proper prevention and management of its potentially fatal complications leading to the poor prognosis of these patients. The current diagnostic criteria incorporate several clinical red flags and magnetic resonance imaging markers supporting diagnosis of multiple system atrophy. Nonetheless, especially in the early disease stage, it can be challenging to differentiate multiple system atrophy from mimic disorders, in particular Parkinson’s disease. Electromyography of the external anal sphincter represents a useful neurophysiological tool for differential diagnosis since it can provide indirect evidence of Onuf’s nucleus degeneration, which is a pathological hallmark of multiple system atrophy. However, the diagnostic value of external anal sphincter electromyography has been a matter of debate for three decades due to controversial reports in the literature. In this review, after a brief overview of the electrophysiological methodology, we first aimed to critically analyze the available knowledge on the diagnostic role of external anal sphincter electromyography. We discussed the conflicting evidence on the clinical correlations of neurogenic abnormalities found at external anal sphincter electromyography. Finally, we reported recent prognostic findings of a novel classification of electromyography patterns of the external anal sphincter that could pave the way toward the implementation of this neurophysiological technique for survival prediction in patients with multiple system atrophy.

Key words: bowel dysfunction, differential diagnosis, dysautonomia, electrophysiology, multiple system atrophy, Onuf’s nucleus degeneration, parkinsonism, Parkinson’s disease, prognostic prediction, urogenital symptoms