中国神经再生研究(英文版) ›› 2023, Vol. 18 ›› Issue (8): 1728-1729.doi: 10.4103/1673-5374.363193

• 观点:退行性病与再生 • 上一篇    下一篇

在多发性硬化症中重新利用组胺能药物

  

  1. 细胞神经生物学单位,CNR-系统分析与计算机科学研究所,圣卢西亚基金会,意大利
  • 出版日期:2023-08-15 发布日期:2023-02-23

Amyotrophic lateral sclerosis disease burden: doing better at getting better

Cinzia Volonté*, Susanna Amadio   

  1. National Research Council-Institute for Systems Analysis and Computer Science “Antonio Ruberti”, Cellular Neurobiology Unit, IRCCS Fondazione Santa Lucia, Rome, Italy (Volonté C) 
    Cellular Neurobiology Unit, IRCCS Fondazione Santa Lucia, Rome, Italy (Amadio S) 
  • Online:2023-08-15 Published:2023-02-23
  • Contact: Cinzia Volonté, PhD, cinzia.volonte@cnr.it.
  • Supported by:
    The present work was supported by FATALSDRUG Project (Progetti di Ricerca@CNR) from National Research Council, Italy (to CV).

摘要: https://orcid.org/0000-0001-7362-8307 (Cinzia Volonté)

Abstract: Amyotrophic lateral sclerosis (ALS) is classified as a multigenic, multifactorial, and heterogeneous neurodegenerative/neuroinflammatory disease that slays especially upper and lower motor neurons controlling voluntary muscle activity. After the insurgence that is characterized by typical symptoms such as weakness in the limbs and muscle twitches, the disease rapidly evolves into progressive muscle atrophy, paralysis, and lastly death occurring by respiratory failure usually within 2–4 years of diagnosis. ALS is now understood as a multisystem and broad-spectrum motor neuron disease largely variable in presentation and outcomes, also showing extra-motor deficits such as extrapyramidal, thalamic, cerebellar, and sensory nerve abnormalities, in addition to comorbid cognitive-behavioral instabilities and psychiatric symptoms. Approximately 15% of ALS patients are also reported suffering from frontotemporal dementia. Concomitant immunological irregularities and gut dysbiosis are often observed. The autonomic nervous system is also involved in the disease, since patients die of sudden death when they lose their ability to compensate for cardiorespiratory arrest. It clearly emerges that a disease like ALS impacts multiple systems throughout the body, and it is not just a neurological disorder.