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对肌萎缩侧索硬化症患者样本中的黑质纹状体系统进行特征描述。

Characterization of the nigroestriatal system in a sample of patients with amyotrophic lateral sclerosis.

机构信息

Hospital Simon Bolívar, Departamento de Neurologia, Bogotá, Colômbia.

Irmandade Santa Casa de Misericórdia de Porto Alegre, Departamento de Neurologia, Porto Alegre, RS, Brasil.

出版信息

Arq Neuropsiquiatr. 2022 Aug;80(8):806-811. doi: 10.1055/s-0042-1755282. Epub 2022 Oct 17.

DOI:10.1055/s-0042-1755282
PMID:36252589
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9703885/
Abstract

BACKGROUND

The coexistence of amyotrophic lateral sclerosis (ALS) with clinical forms of Parkinson disease (PD), although uncommon, is found to a greater degree than one would expect by chance. The pathological mechanisms of ALS and PD are still not fully understood, and the coexistence of these two diseases suggests that they could share mechanisms in common.

OBJECTIVE

Here we present a sample of patients with clinically definitive or probable ALS who were evaluated with single-photon emission computed tomography SPECT/TRODAT and compared with non-ALS controls.

METHODS

Patients with clinically definite or probable ALS were assessed with the amyotrophic lateral sclerosis functional rating scale (ALSFRS) to define severity and had their demographic data collected. The TRODAT results of patients with ALS were compared with those of patients with a diagnosis of PD with less than 10 years of duration, and with patients with a diagnosis of others movement disorders not associated with neurodegenerative diseases.

RESULTS

A total of 75% of patients with ALS had TRODAT results below the levels considered normal; that was also true for 25% of the patients in the control group without neurodegenerative disease, and for 100% of the patients in the PD group. A statistically significant difference was found between patients with ALS and the control group without neurodegenerative disease in the TRODAT values < 0.05.

CONCLUSIONS

Our study fits with the neuropathological and functional evidence that demonstrates the existence of nigrostriatal dysfunction in patients with ALS. Further research to better understand the role of these changes in the pathophysiological process of ALS needs to be performed.

摘要

背景

肌萎缩侧索硬化症(ALS)与临床帕金森病(PD)形式共存虽然不常见,但比预期的偶然程度更高。ALS 和 PD 的病理机制仍不完全清楚,这两种疾病的共存表明它们可能有共同的机制。

目的

我们在这里介绍了一组经临床确诊或可能的 ALS 患者,他们接受了单光子发射计算机断层扫描 SPECT/TRODAT 评估,并与非 ALS 对照组进行了比较。

方法

用肌萎缩侧索硬化功能评定量表(ALSFRS)评估临床确诊或可能的 ALS 患者,以确定严重程度,并收集他们的人口统计学数据。将 ALS 患者的 TRODAT 结果与 PD 诊断不到 10 年的患者和与神经退行性疾病无关的其他运动障碍患者的结果进行比较。

结果

75%的 ALS 患者 TRODAT 结果低于正常值;在无神经退行性疾病的对照组中也有 25%的患者如此,在 PD 组中则有 100%的患者如此。ALS 患者与无神经退行性疾病的对照组之间的 TRODAT 值存在显著差异(<0.05)。

结论

我们的研究符合神经病理学和功能证据,证明 ALS 患者存在黑质纹状体功能障碍。需要进一步研究以更好地了解这些变化在 ALS 病理生理过程中的作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9855/9703885/9f225d5584a7/0004-282X-anp-80-08-0806-gf02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9855/9703885/581d33ae0993/0004-282X-anp-80-08-0806-gf01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9855/9703885/9f225d5584a7/0004-282X-anp-80-08-0806-gf02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9855/9703885/581d33ae0993/0004-282X-anp-80-08-0806-gf01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9855/9703885/9f225d5584a7/0004-282X-anp-80-08-0806-gf02.jpg

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