Objective: To automatically study the pupillary light reflex in patients with hereditary transthyretin-associated amyloidosis (hATTR).
Methods: Prospective cross-sectional observational study in patients with hATTR with unilateral scalloped iris. Pupillary light reflex of scalloped iris eyes (21 eyes) were compared with non-scalloped iris eyes (21 eyes, paired eyes of the same patients) and also with a control group of 20 healthy eyes, using static and dynamic pupillometry with the Metrovision MonPack One.
Results: No patient presented evident neurological involvment of the cranial nerves. No significant differences were found in the pupillary diameters under standardized lighting conditions (static pupillometry) among groups. In dynamic pupillometry, the amplitude of contraction, the velocity of contraction and the velocity of dilation were statistically significantly lower in eyes with scalloped iris, comparing both with the contralateral non-scalloped iris eyes ( < 0.001 for all) and with eyes from healthy subjects ( < 0.05 for all).
Conclusion: A scalloped iris reflects a more advanced endocular hATTR and it is associated with an altered pupillary light reflex. Pupillometry may be a quick, simple, and portable test to objectively evaluate ocular amyloid deposition in hATTR eyes. Pupillary light reflex may not be reliable to evaluate neurological dysfunction in these patients.
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http://dx.doi.org/10.1177/1120672121997294 | DOI Listing |
Ann Med Surg (Lond)
February 2022
Pediatric Ophthalmology Department, Ho^pital 20, Aou^t 1953, Casablanca, Morocco.
Introduction: Gillespie syndrome (GS) is a rare genetic disorder that combines ocular and cerebral defects.It was first described in 1965, by Frederick D. Gillespie.
View Article and Find Full Text PDFEur J Ophthalmol
January 2022
Ophthalmology Department, Centro Hospitalar Universitário do Porto, Porto, Portugal.
Objective: To automatically study the pupillary light reflex in patients with hereditary transthyretin-associated amyloidosis (hATTR).
Methods: Prospective cross-sectional observational study in patients with hATTR with unilateral scalloped iris. Pupillary light reflex of scalloped iris eyes (21 eyes) were compared with non-scalloped iris eyes (21 eyes, paired eyes of the same patients) and also with a control group of 20 healthy eyes, using static and dynamic pupillometry with the Metrovision MonPack One.
Amyloid
March 2021
Ophthalmology Department, Centro Hospitalar Universitário do Porto, Porto, Portugal.
Background: Retinal angiopathy is a known ocular manifestation of hereditary transthyretin amyloidosis (ATTRv). Optical coherence tomography angiography (OCT-A) is a recent noninvasive imaging technique, used in other retinal vascular diseases. Our purpose was to analyse subclinical changes in retinal vessels, with OCT-A, in patients with ATTRv amyloidosis.
View Article and Find Full Text PDFAdv Exp Med Biol
July 2019
Department of Ophthalmology, Columbia University, Edward S. Harkness Eye Institute, NewYork-Presbyterian Hospital, New York, NY, USA.
Patients with late-onset retinal degeneration (LORD) usually present with nyctalopia in the fifth or sixth decade of life. The fundus shows yellowish-white, punctate deposits, usually progressive and giving rise to scalloped areas of retinal pigment epithelium (RPE) atrophy in the mid-periphery and posterior pole (Fig. 23.
View Article and Find Full Text PDFJ Ophthalmol
September 2018
Instituto de Ciências Biomédicas Abel Salazar, Universidade do Porto, Porto, Portugal.
Purpose: Assess the aqueous humor flare in transthyretin V30M amyloidosis patients (ATTRV30M).
Materials And Methods: This is a retrospective, cross-sectional, noninterventional comparative study including 28 ATTRV30M patients with a unilateral scalloped iris. For comparative analysis, the fellow eye, the nonscalloped iris eye, from each patient was used as control.
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