Purpose: Corneal neurotization (CN) is a novel, potentially curative surgical procedure for the treatment of neurothophic keratopathy (NK). Patients with severe NK can present with corneal opacification requiring optical keratoplasty, which would likely fail without a proper trophic support of corneal nerves in the recipient cornea.
Methods: This is a pilot study on 4 patients undergoing keratoplasty after CN.
Electromyographic evaluation is a reliable tool for confirming facial palsy and assessing its severity. It allows differentiating facial paresis and paralysis, and further distinguishes acute palsies, still showing muscle fibrillations, from chronic cases. This article aims to show that EMG fibrillations might represent a better criterion to differentiate acute and chronic palsies than the standard 18-24 months' cut-off usually employed for classification and treatment purposes.
View Article and Find Full Text PDFBackground: Hereditary transthyretin (ATTRv, v for variant) amyloidosis with polyneuropathy is a rare disease caused by mutations in the transthyretin gene. In ATTRv amyloidosis, multisystem extracellular deposits of amyloid cause tissue and organ dysfunction. Patisiran is a small interfering RNA molecule drug that reduces circulating levels of mutant and wild-type TTR proteins.
View Article and Find Full Text PDFThe therapeutic advance in hereditary transthyretin amyloidosis (ATTRv amyloidosis) requires quantitative biomarkers of nerve involvement in order to foster early diagnosis and monitor therapy response. We aimed at quantitatively assessing Magnetic Resonance Neurography (MRN) and Diffusion Tensor Imaging (DTI) properties of the sciatic nerve in subjects with ATTRv-amyloidosis-polyneuropathy (ATTRv-PN) and pre-symptomatic carriers (ATTRv-C). Twenty subjects with pathogenic variants of the TTR gene (mean age 62.
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