Spinal cord cross sign: a potential marker for hereditary spastic paraplegia type 5.

IF 2.4 3区医学 Q2 CLINICAL NEUROLOGY

Neuroradiology Pub Date : 2025-04-01 Epub Date: 2025-01-24 DOI:10.1007/s00234-025-03543-y

Fan Zhang, Jianping Hu, Zebin Xiao, Chenlin Lin, Zhuoting Huang, Ning Wang, Ying Liu

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引用次数: 0

Abstract

Purpose: Spastic paraplegia type 5 (SPG5) is a rare neurodegenerative disease diagnosed primarily through genetic testing.We identified a specific spinal cord sign on conventional MR imaging to help narrow the scope of genetic screening.

Methods: In 25 patients with SPG5 and 21 healthy controls (HCs), the spinal cord cross sign was evaluated on T2*-weighted imaging. The morphological and signal characteristics of the dorsal column (DC), ventral funiculi (VF), dorsal horn (DH), ventral horn (VH), and intermediate zone (IMZ) were assessed. Differences in fractional anisotropy (FA) values within specific regions between HC and SPG5 were tested using Student's t-test. Spearman correlation was used to evaluate associations between cross-sign scores, FA values, and clinical indicators.

Results: The cross sign was detected in the cervical spinal cord of all SPG5 patients. The occurrence of T2 hyperintensity in the DC, VF and IMZ was 100%,100% and 88%,respectively. Bilateral VH morphology was normal in 14.4% of cases, blurred in 49.6%, and absent in 36%.Bilateral DH morphology was normal in 13.6%, blurred in 56%, and absent in 30.4%. FA values were reduced in these spinal cord regions. Cross-sign scores were negatively correlated with FA values in both grey (r = -0.70~-0.37) and white matter (r = -0.78~-0.70). Cross-sign scores were positively correlated with Spastic Paraplegia Rating Scale (r = 0.57) and disease duration (r = 0.42).

Conclusion: The spinal cord cross sign was a potential imaging marker for SPG5. Cross-sign scores were associated with disease duration and severity in SPG5 patients.

Trial registration: A Registered Cohort Study on Spastic Paraplegia,NCT04006418 Registered 1 July 2019, https://clinicaltrials.gov/study/NCT04006418 .

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脊髓交叉征象：遗传性痉挛性截瘫5型的潜在标志。

目的：痉挛性截瘫5型（SPG5）是一种罕见的神经退行性疾病，主要通过基因检测诊断。我们在常规磁共振成像上识别出一种特定的脊髓征象，以帮助缩小遗传筛查的范围。方法：对25例SPG5患者和21例健康对照（hc）进行T2*加权成像评价脊髓交叉征象。评估背柱（DC）、腓索腹侧（VF）、背角（DH）、腹角（VH）和中间区（IMZ）的形态学和信号特征。采用学生t检验检验HC和SPG5在特定区域内分数各向异性（FA）值的差异。Spearman相关性用于评价交叉征象评分、FA值和临床指标之间的相关性。结果：SPG5患者颈脊髓均可见交叉征。T2高信号在DC、VF和IMZ的发生率分别为100%、100%和88%。14.4%的双侧VH形态正常，49.6%的双侧VH形态模糊，36%的双侧VH形态缺失。双侧DH形态正常的占13.6%，模糊的占56%，缺失的占30.4%。这些脊髓区域的FA值降低。交叉符号评分与灰质FA值（r = -0.70~-0.37）和白质FA值（r = -0.78~-0.70）呈负相关。交叉符号评分与痉挛性截瘫评定量表（r = 0.57）和病程（r = 0.42）呈正相关。结论：脊髓交叉征是SPG5的潜在影像学标志。SPG5患者的交叉体征评分与疾病持续时间和严重程度相关。试验注册：一项已注册的痉挛性截瘫队列研究，NCT04006418于2019年7月1日注册，https://clinicaltrials.gov/study/NCT04006418。

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

Neuroradiology 医学-核医学

CiteScore

5.30

自引率

3.60%

发文量

214

审稿时长

4-8 weeks

期刊介绍： Neuroradiology aims to provide state-of-the-art medical and scientific information in the fields of Neuroradiology, Neurosciences, Neurology, Psychiatry, Neurosurgery, and related medical specialities. Neuroradiology as the official Journal of the European Society of Neuroradiology receives submissions from all parts of the world and publishes peer-reviewed original research, comprehensive reviews, educational papers, opinion papers, and short reports on exceptional clinical observations and new technical developments in the field of Neuroimaging and Neurointervention. The journal has subsections for Diagnostic and Interventional Neuroradiology, Advanced Neuroimaging, Paediatric Neuroradiology, Head-Neck-ENT Radiology, Spine Neuroradiology, and for submissions from Japan. Neuroradiology aims to provide new knowledge about and insights into the function and pathology of the human nervous system that may help to better diagnose and treat nervous system diseases. Neuroradiology is a member of the Committee on Publication Ethics (COPE) and follows the COPE core practices. Neuroradiology prefers articles that are free of bias, self-critical regarding limitations, transparent and clear in describing study participants, methods, and statistics, and short in presenting results. Before peer-review all submissions are automatically checked by iThenticate to assess for potential overlap in prior publication.