Retinal ganglion cell and microvascular density loss in hereditary spastic paraplegia

IF 1.9 4区医学 Q4 NEUROSCIENCES Restorative neurology and neuroscience Pub Date : 2024-01-06 DOI:10.3233/rnn-231380

Gabrielle N. Turski, Christopher A. Turski, Marcus Grobe-Einsler, Xenia Kobeleva, Jennifer S. Turski, Frank G. Holz, Robert P. Finger, Thomas Klockgether

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Abstract

Background:

Hereditary spastic paraplegia (HSP) is characterized by progressive degeneration of distal axons in the long corticospinal tracts. Loss of retinal cells and microvascular networks has neither been suspected nor investigated. We concurrently examined the retinal microvasculature and retinal layer morphology in patients with HSP to assess whether retinal features may portray disease and its progression.

Methods:

Fifteen patients with HSP and 30 healthy controls were included in this cross-sectional case-control study. Disease severity was assessed with the Spastic Paraplegia Rating Scale (SPRS). Severity of ataxia was determined by the Scale for the Assessment and Rating of Ataxia (SARA). Retinal microvasculature was measured by means of optical coherence tomography angiography (OCT-A) and morphology of retinal layers using structural OCT. Mixed-effects models were applied for data analysis.

Results:

HSP patients showed significantly reduced vessel density of the superficial vascular plexus (SVP), reduced ganglion cell layer (GCL) volume, reduced inner plexiform layer (IPL) volume and reduced temporal-inferior peripapillary retinal nerve fiber layer (pRNFL) thickness versus healthy controls. GCL volume reduction correlated significantly with the worsening of visual acuity and higher SARA scores.

Conclusions:

These findings demonstrate that, in HSP both cells and vascular networks of the retina are compromised. Assessment of the retinal GCL, IPL and SVP may aid in diagnosis and monitoring of disease progression as well as provide novel structural outcome measures for clinical trials.

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遗传性痉挛性截瘫的视网膜神经节细胞和微血管密度损失

摘要背景：遗传性痉挛性截瘫（HSP）的特征是长皮质脊髓束远端轴突进行性变性。视网膜细胞和微血管网络的丧失既未被怀疑也未被研究。我们同时检查了HSP患者的视网膜微血管和视网膜层形态，以评估视网膜特征是否可以反映疾病及其进展。用痉挛性截瘫评定量表（SPRS）评估疾病的严重程度。共济失调的严重程度由共济失调评估和分级量表（SARA）确定。视网膜微血管通过光学相干断层血管成像（OCT-A）进行测量，视网膜层的形态则通过结构性 OCT 进行测量。结果显示：与健康对照组相比，HSP 患者的浅层血管丛（SVP）血管密度明显降低，神经节细胞层（GCL）体积缩小，内层丛状层（IPL）体积缩小，颞侧-下腹部视网膜神经纤维层（pRNFL）厚度缩小。结论：这些研究结果表明，HSP 患者视网膜的细胞和血管网络均受到损害。对视网膜 GCL、IPL 和 SVP 的评估可能有助于诊断和监测疾病的进展，并为临床试验提供新的结构性结果测量。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Restorative neurology and neuroscience 医学-神经科学

CiteScore

5.40

自引率

3.60%

发文量

审稿时长

>12 weeks

期刊介绍： This interdisciplinary journal publishes papers relating to the plasticity and response of the nervous system to accidental or experimental injuries and their interventions, transplantation, neurodegenerative disorders and experimental strategies to improve regeneration or functional recovery and rehabilitation. Experimental and clinical research papers adopting fresh conceptual approaches are encouraged. The overriding criteria for publication are novelty, significant experimental or clinical relevance and interest to a multidisciplinary audience. Experiments on un-anesthetized animals should conform with the standards for the use of laboratory animals as established by the Institute of Laboratory Animal Resources, US National Academy of Sciences. Experiments in which paralytic agents are used must be justified. Patient identity should be concealed. All manuscripts are sent out for blind peer review to editorial board members or outside reviewers. Restorative Neurology and Neuroscience is a member of Neuroscience Peer Review Consortium.