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瞳孔光反応の変化はファブリー病患者の自律神経症状の重症度と関連している
Altered pupillary light responses are associated with the severity of autonomic symptoms in patients with Fabry disease.
PMID: 33854145 PMCID: PMC8046772. DOI: 10.1038/s41598-021-87589-x.
抄録
ファブリー病では、自律神経失調症の症状がよく見られる。本研究では、ファブリー病患者における白色光刺激に対する瞳孔反応の変化と、自律神経症状の重症度との関連性を評価することを目的とした。この横断的研究には、連続した14人のファブリー病患者と14人の健常対照者が登録された。ファブリー病の重症度の測定にはMainz Severity Score Index（MSSI）を，自律神経症状の重症度の評価にはComposite Autonomic Symptom Scale 31（COMPASS 31）問診票を用いた．瞳孔の光反応は，赤外線ダイナミック瞳孔測定装置を用いて評価した。ファブリー病患者の瞳孔収縮の振幅（P=0.048）と持続時間（P=0.048）、および瞳孔拡張の潜時（P=0.048）は、対照群に比べて有意に減少した。加重COMPASS 31スコアの合計は、MSSI（r=0.592、P=0.026）および瞳孔散大時間（ρ=0.561、P=0.037）と相関した。COMPASS 31のpupillomotor weighted sub-scoreは、瞳孔収縮時間（r=-0.600; P=0.023）および瞳孔拡張潜時（ρ=-0.541; P=0.046）と逆相関し、瞳孔拡張時間（ρ=0.877; P<0.001）およびMSSI（r=0.533; P=0.049）と直接相関した。以上、ファブリー病患者では瞳孔機能の異常が認められ、それが自律神経症状の重さと関連していることがわかりました。
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.