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Progressive brachial plexus enlargement in hereditary transthyretin amyloidosis.

Alessandro SalvalaggioDaniele CoraciLaura ObiciMario CacciavillaniMarco LuigettiAnna MazzeoFrancesca PastorelliMarina GrandisTiziana CavallaroGiulia BisogniAlessandro LozzaChiara GemelliLuca GentileMassimo RussoMario ErmaniGian Maria FabriziRosaria PlasmatiFederica De NapoliMarta CampagnoloFrancesca CastellaniFabrizio SalviSilvia FenuGrazia DevigiliDavide PareysonRoberto GasparottiClaudio RapezziCarlo MartinoliLuca PaduaChiara Briani
Published in: Journal of neurology (2021)
Axonal polyneuropathy is the main feature of hereditary transthyretin amyloidosis (ATTRv). Nerve morphological abnormalities have been reported, but longitudinal changes have never been assessed. We performed a prospective widespread nerve ultrasound evaluation and nerve cross-sectional area (CSA) was compared with baseline data in both ATTRv patients and pre-symptomatic carriers. Thirty-eight subjects were evaluated (mean follow-up 17.1 months), among them 21 had polyneuropathy while 17 were pre-symptomatic carriers. CSA significantly increased at brachial plexus in both groups (p = 0.008 and p = 0.012) pointing to progressive brachial plexus enlargement as a longitudinal biomarker of both disease progression and disease occurrence in pre-symptomatic carriers.
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