Could PLS represent a UMN-predominant ALS syndrome?

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Revue neurologique Pub Date : 2024-05-22 DOI:10.1016/j.neurol.2024.04.006

P Corcia, P Couratier, C Ingre

引用次数: 0

Abstract

Primary lateral sclerosis (PLS) is a motor neuron condition marked by pure upper motor neuron (UMN) degeneration. PLS represents around 3% of all motor neuron diseases. Classically the prognosis of PLS is less severe than those of amyotrophic lateral sclerosis (ALS). This explains the necessity to distinguish both conditions as early as possible. The key hallmark between the two diseases is the involvement of the lower motor neuron (LMN) system which is classically considered spared in PLS contrary to ALS. Although it seemed clinically easy to distinguish PLS from ALS with the aid of clinical and complementary examinations, there is a large body of evidence highlighting that the LMN system might be impaired in PLS. This led us to suggest that PLS might be considered as an almost pure UMN ALS phenotype.

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PLS 是否代表一种 UMN 显性 ALS 综合征？

原发性侧索硬化症（PLS）是一种以单纯上运动神经元（UMN）变性为特征的运动神经元疾病。原发性侧索硬化症约占所有运动神经元疾病的 3%。与肌萎缩侧索硬化症（ALS）相比，原发性肌萎缩侧索硬化症的预后通常较轻。因此，有必要尽早区分这两种疾病。这两种疾病的主要特征是下运动神经元（LMN）系统受累，与 ALS 相反，下运动神经元系统在 PLS 中通常被认为是不受影响的。虽然在临床上，借助临床和辅助检查似乎很容易将 PLS 与 ALS 区分开来，但有大量证据表明，LMN 系统可能在 PLS 中受损。这使我们认为，PLS 可被视为一种几乎纯粹的 UMN ALS 表型。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Revue neurologique 医学-临床神经学

CiteScore

4.80

自引率

0.00%

发文量

598

审稿时长

55 days

期刊介绍： The first issue of the Revue Neurologique, featuring an original article by Jean-Martin Charcot, was published on February 28th, 1893. Six years later, the French Society of Neurology (SFN) adopted this journal as its official publication in the year of its foundation, 1899. The Revue Neurologique was published throughout the 20th century without interruption and is indexed in all international databases (including Current Contents, Pubmed, Scopus). Ten annual issues provide original peer-reviewed clinical and research articles, and review articles giving up-to-date insights in all areas of neurology. The Revue Neurologique also publishes guidelines and recommendations. The Revue Neurologique publishes original articles, brief reports, general reviews, editorials, and letters to the editor as well as correspondence concerning articles previously published in the journal in the correspondence column.