PDF(Pubmed)
Abstract：
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
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的进行性变性。视网膜细胞和微血管网络的丢失既未被怀疑也未被调查。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描绘疾病及其进展。
方法：本横断面病例对照研究包括15例HSP患者和30例健康对照。用痉挛型截瘫评定量表（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评级量表(SARA)确定。通过光学相干断层扫描血管造影（OCT-A）和使用结构OCT的视网膜层形态来测量视网膜微血管。应用混合效应模型进行数据分析。
结果：HSP患者显示浅表血管丛（SVP）的血管密度显着降低，神经节细胞层（GCL）体积减少，与健康对照组相比，内网状层（IPL）体积减少，颞下乳头周围视网膜神经纤维层（pRNFL）厚度减少。GCL体积减少与视力恶化和较高的SARA评分显着相关。
结论：这些研究结果表明，在HSP中，视网膜的细胞和血管网络均受损。视网膜GCL的评估，IPL和SVP可能有助于诊断和监测疾病进展，并为临床试验提供新的结构结果指标。
方法：本横断面病例对照研究包括15例HSP患者和30例健康对照。用痉挛型截瘫评定量表（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评级量表(SARA)确定。通过光学相干断层扫描血管造影（OCT-A）和使用结构OCT的视网膜层形态来测量视网膜微血管。应用混合效应模型进行数据分析。
结果：HSP患者显示浅表血管丛（SVP）的血管密度显着降低，神经节细胞层（GCL）体积减少，与健康对照组相比，内网状层（IPL）体积减少，颞下乳头周围视网膜神经纤维层（pRNFL）厚度减少。GCL体积减少与视力恶化和较高的SARA评分显着相关。
结论：这些研究结果表明，在HSP中，视网膜的细胞和血管网络均受损。视网膜GCL的评估，IPL和SVP可能有助于诊断和监测疾病进展，并为临床试验提供新的结构结果指标。
