Altered pupillary light responses are associated with the severity of autonomic symptoms in patients with Fabry disease

Abstract Symptoms of autonomic dysfunction are common in Fabry disease. In this study we aimed to evaluate alterations in the pupillary response to white light stimulation in patients with Fabry disease and their association with the severity of autonomic symptoms. Fourteen consecutive patients with...

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Autores principales:	Gulfidan Bitirgen, Kultigin Turkmen, Nazmi Zengin, Rayaz A. Malik
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/ac4a59b167a04010a05dce7f307bb442
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spelling	oai:doaj.org-article:ac4a59b167a04010a05dce7f307bb4422021-12-02T14:27:53ZAltered pupillary light responses are associated with the severity of autonomic symptoms in patients with Fabry disease10.1038/s41598-021-87589-x2045-2322https://doaj.org/article/ac4a59b167a04010a05dce7f307bb4422021-04-01T00:00:00Zhttps://doi.org/10.1038/s41598-021-87589-xhttps://doaj.org/toc/2045-2322Abstract Symptoms of autonomic dysfunction are common in Fabry disease. In this study we aimed to evaluate alterations in the pupillary response to white light stimulation in patients with Fabry disease and their association with the severity of autonomic symptoms. Fourteen consecutive patients with Fabry disease and 14 healthy control participants were enrolled in this cross-sectional study. The Mainz Severity Score Index (MSSI) was used to measure the severity of Fabry disease and the Composite Autonomic Symptom Scale 31 (COMPASS 31) questionnaire was used to evaluate the severity of autonomic symptoms. The pupil light responses were assessed with an infrared dynamic pupillometry unit. There were significant reductions in the amplitude (P = 0.048) and duration (P = 0.048) of pupil contraction, and the latency of pupil dilation (P = 0.048) in patients with Fabry disease compared to control subjects. The total weighted COMPASS 31 score correlated with MSSI (r = 0.592; P = 0.026) and the duration of pupil dilation (ρ = 0.561; P = 0.037). The pupillomotor weighted sub-score of the COMPASS 31 correlated inversely with the duration of pupil contraction (r = − 0.600; P = 0.023) and latency of pupil dilation (ρ = − 0.541; P = 0.046), and directly with the duration of pupil dilation (ρ = 0.877; P < 0.001) and MSSI (r = 0.533; P = 0.049). In conclusion, abnormal pupillary function is demonstrated in patients with Fabry disease, which is associated with the severity of autonomic symptoms.Gulfidan BitirgenKultigin TurkmenNazmi ZenginRayaz A. MalikNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 11, Iss 1, Pp 1-8 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Gulfidan Bitirgen Kultigin Turkmen Nazmi Zengin Rayaz A. Malik Altered pupillary light responses are associated with the severity of autonomic symptoms in patients with Fabry disease
description	Abstract Symptoms of autonomic dysfunction are common in Fabry disease. In this study we aimed to evaluate alterations in the pupillary response to white light stimulation in patients with Fabry disease and their association with the severity of autonomic symptoms. Fourteen consecutive patients with Fabry disease and 14 healthy control participants were enrolled in this cross-sectional study. The Mainz Severity Score Index (MSSI) was used to measure the severity of Fabry disease and the Composite Autonomic Symptom Scale 31 (COMPASS 31) questionnaire was used to evaluate the severity of autonomic symptoms. The pupil light responses were assessed with an infrared dynamic pupillometry unit. There were significant reductions in the amplitude (P = 0.048) and duration (P = 0.048) of pupil contraction, and the latency of pupil dilation (P = 0.048) in patients with Fabry disease compared to control subjects. The total weighted COMPASS 31 score correlated with MSSI (r = 0.592; P = 0.026) and the duration of pupil dilation (ρ = 0.561; P = 0.037). The pupillomotor weighted sub-score of the COMPASS 31 correlated inversely with the duration of pupil contraction (r = − 0.600; P = 0.023) and latency of pupil dilation (ρ = − 0.541; P = 0.046), and directly with the duration of pupil dilation (ρ = 0.877; P < 0.001) and MSSI (r = 0.533; P = 0.049). In conclusion, abnormal pupillary function is demonstrated in patients with Fabry disease, which is associated with the severity of autonomic symptoms.
format	article
author	Gulfidan Bitirgen Kultigin Turkmen Nazmi Zengin Rayaz A. Malik
author_facet	Gulfidan Bitirgen Kultigin Turkmen Nazmi Zengin Rayaz A. Malik
author_sort	Gulfidan Bitirgen
title	Altered pupillary light responses are associated with the severity of autonomic symptoms in patients with Fabry disease
title_short	Altered pupillary light responses are associated with the severity of autonomic symptoms in patients with Fabry disease
title_full	Altered pupillary light responses are associated with the severity of autonomic symptoms in patients with Fabry disease
title_fullStr	Altered pupillary light responses are associated with the severity of autonomic symptoms in patients with Fabry disease
title_full_unstemmed	Altered pupillary light responses are associated with the severity of autonomic symptoms in patients with Fabry disease
title_sort	altered pupillary light responses are associated with the severity of autonomic symptoms in patients with fabry disease
publisher	Nature Portfolio
publishDate	2021
url	https://doaj.org/article/ac4a59b167a04010a05dce7f307bb442
work_keys_str_mv	AT gulfidanbitirgen alteredpupillarylightresponsesareassociatedwiththeseverityofautonomicsymptomsinpatientswithfabrydisease AT kultiginturkmen alteredpupillarylightresponsesareassociatedwiththeseverityofautonomicsymptomsinpatientswithfabrydisease AT nazmizengin alteredpupillarylightresponsesareassociatedwiththeseverityofautonomicsymptomsinpatientswithfabrydisease AT rayazamalik alteredpupillarylightresponsesareassociatedwiththeseverityofautonomicsymptomsinpatientswithfabrydisease
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Altered pupillary light responses are associated with the severity of autonomic symptoms in patients with Fabry disease

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