Mann Richard H
Podiatry, Realm Labs, LLC, Boca Raton, USA.
Cureus. 2023 Jun 16;15(6):e40511. doi: 10.7759/cureus.40511. eCollection 2023 Jun.
Homogenates of brain tissue from the frontal cortex at autopsy in patients with amyotrophic lateral sclerosis (ALS) showed dramatically reduced levels of the enzyme thiamine pyrophosphatase (TPPase), the enzyme responsible for the conversion of thiamine pyrophosphate (TPP) to thiamine monophosphate (TMP). Additionally, free thiamine (vitamin B1) and TMP levels have been shown to be significantly reduced in the plasma and cerebral spinal fluid (CSF) of patients with ALS. These findings suggest that there is impaired thiamine metabolism in patients with ALS. Impaired thiamine metabolism decreases adenosine triphosphate (ATP) production and is a well-established cause of neurodegeneration. Decreased levels of TPPase, resulting in decreased levels of TMP in the cells of the frontal cortex, might account for the focal neurodegenerative changes observed in motor neurons in ALS. Benfotiamine, a safe, lipid-soluble, highly absorbable thiamine analogue, significantly raises free thiamine, TMP, and TPP levels in the blood. A case in which benfotiamine may have positively impacted the symptoms of a patient with ALS is presented. The use of benfotiamine in patients with ALS appears to be a promising therapeutic option. Considering the severity and the lack of satisfactory treatment options associated with this disease, more research on the effects of benfotiamine on the course of ALS is urgently needed.
对肌萎缩侧索硬化症（ALS）患者尸检时获取的额叶皮质脑组织匀浆显示，硫胺素焦磷酸酶（TPPase）水平显著降低，该酶负责将硫胺素焦磷酸（TPP）转化为硫胺素单磷酸（TMP）。此外，已证实ALS患者血浆和脑脊液（CSF）中的游离硫胺素（维生素B1）和TMP水平显著降低。这些发现表明ALS患者存在硫胺素代谢受损。硫胺素代谢受损会减少三磷酸腺苷（ATP）的产生，是神经退行性变的一个公认原因。TPPase水平降低，导致额叶皮质细胞中TMP水平降低，可能是ALS运动神经元中观察到的局灶性神经退行性变的原因。苯磷硫胺是一种安全、脂溶性、高吸收性的硫胺素类似物，可显著提高血液中的游离硫胺素、TMP和TPP水平。本文介绍了一例苯磷硫胺可能对一名ALS患者症状产生积极影响的病例。在ALS患者中使用苯磷硫胺似乎是一种有前景的治疗选择。鉴于该疾病的严重性以及缺乏令人满意的治疗方案，迫切需要更多关于苯磷硫胺对ALS病程影响的研究。
