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Neuropathology of central nervous system involvement in TTR amyloidosis by Ricardo Taipa; Luísa Sousa; Miguel Pinto; Inês Reis; Aurora Rodrigues; Pedro Oliveira; Manuel Melo-Pires; Teresa Coelho is a Medicine article available to read on EtoBox.

What is Neuropathology of central nervous system involvement in TTR amyloidosis about?

Hereditary transthyretin amyloidosis (ATTRv) is a systemic disease caused by the accumulation of misfolded transthyretin (TTR). It usually presents with an adult-onset progressive axonal peripheral neuropathy and cardiomyopathy. In the central nervous system (CNS), variant TTR is produced by the choroid plexus and accumulates in the leptomeninges. CNS symptoms have been increasingly recognized in this population, including transient focal neurological episodes and stroke, particularly in patients with the V30M mutation and longstanding disease. The prevalence, pathophysiology, and progression of CNS involvement remain to be clarified. The present work explores if there is a recognizable sequence of CNS TTR deposition in ATTRv. We studied the topographical and severity distribution of TTR deposition in 16 patients with ATTRv, aged 27-69 years and with a mean disease duration of 10.9 years (range: 3-29). Our results suggest that CNS pathological involvement in V30M ATTRv occurs early in the disease course, probably starting in pre-symptomatic phases, and follows a distinct sequence. Leptomeninges and subarachnoid meningeal vessels are affected earlier, then followed by perforating co

Who reads Neuropathology of central nervous system involvement in TTR amyloidosis?

Author

Ricardo Taipa; Luísa Sousa; Miguel Pinto; Inês Reis; Aurora Rodrigues; Pedro Oliveira; Manuel Melo-Pires; Teresa Coelho

Publisher

Springer Science and Business Media LLC

Published

2022

Language

EN

Field

Medicine (Life Sciences)