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Amyotrophic Lateral Sclerosis - Wiley Online Library

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Last Updated: 27 October 2022

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pTDP‐43 aggregates accumulate in non‐central nervous system tissues prior to symptom onset in amyotrophic lateral sclerosis: a case series linking archival surgical biopsies with clinical phenotypic data

We analyzed tissue from 13 people who went on to experience ALS, including sporadic ALS and C9orf72 hexanucleotide repeat expansion; The tissue collection contained 68 formalin-fixed paraffin embedded samples from 21 surgical samples isolated from 8 organ systems, which we investigated for evidence of phosphorylated TDPu201043 pathology. We found pTDP and dendritic cells within the lamina propria's macrophages and dendritic cells, as well as ganglion/neuronal and glial cells of the myenteric plexus. In all cases of non-u2010CNS pTDPu201043 pathology, aggregates were present prior to ALS diagnosis and in some cases preceded neurological symptom onset by more than ten years. These results indicate that patients with microscopically unexplained non-U2010CNS symptoms may have occult protein aggregation that could have been present for many years before neurological involvement.

Source link: https://onlinelibrary.wiley.com/doi/10.1002/cjp2.297

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions