[Neurological manifestations of amyloidoses]
Related Experiment Video
Updated: Nov 5, 2026

Interactions with and Membrane Permeabilization of Brain Mitochondria by Amyloid Fibrils
Published on: September 28, 2019
Clara C Meny1, Hans H Jung1,2
1Klinik für Neurologie, Universitätsspital Zürich, Zürich, Schweiz.
Introduction:
Amyloidoses are a heterogeneous group of disorders characterized by the extracellular deposition of misfolded proteins as insoluble fibrils. Neurological involvement is among the most clinically relevant manifestations, particularly in hereditary transthyretin amyloidosis (hATTR). The neurological phenotype commonly includes a length-dependent sensorimotor and autonomic polyneuropathy, leptomeningeal and central nervous system involvement. The differential diagnosis is broad and encompasses inflammatory and hereditary neuropathies as well as systemic causes of polyneuropathy. A detailed family history, genetic testing, and histopathological confirmation of amyloid deposition are central to establishing the diagnosis. Disease-modifying therapies - especially TTR stabilizers and RNA-silencing agents - have markedly improved the prognosis of hATTR neuropathy, highlighting the critical importance of early recognition and timely treatment initiation.
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