PCNSV With Spinal Cord Involvement: A 40-Year Single-Center Study.

IF 8.9 1区医学 Q1 CLINICAL NEUROLOGY Stroke Pub Date : 2025-05-01 Epub Date: 2025-03-20 DOI:10.1161/STROKEAHA.124.049547

Carlo Salvarani, Robert D Brown, Teresa J H Christianson, John Huston, Caterina Giannini, Gene G Hunder

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Abstract

Background: Spinal cord involvement is infrequently reported in primary central nervous system vasculitis (PCNSV). This study describes the clinical findings, therapeutic responses, and outcomes of spinal cord involvement in a large cohort of patients with PCNSV.

Methods: We retrospectively studied 216 consecutive patients with PCNSV evaluated at the Mayo Clinic from 1983 to 2023. Spinal cord involvement was documented by histological examination or imaging. The clinical, laboratory, radiological, and pathological findings, along with management and outcomes, were compared between patients with and without spinal cord involvement.

Results: Of 216 patients, 10 (4.6%) had spinal cord involvement, with cerebral involvement in 9 cases. One patient (0.5%) had isolated spinal cord vasculitis. Histological evidence of vasculitis was found in all 10, with necrotizing vasculitis in 5 (50%). Magnetic resonance imaging showed thoracic abnormalities in 8 patients, cervical spine involvement in 2, conus medullaris involvement in 3, and cauda equina enhancement in 4. Compared with the 206 patients without spinal cord involvement, those with spinal cord vasculitis were more likely to present with paraparesis/tetraparesis (P<0.001) and necrotizing vasculitis (P=0.01) and less likely with hemiparesis (P=0.006) and granulomatous vasculitis (P=0.03). Patients with spinal cord involvement were more likely to have at least 1 relapse (P<0.001) or more (P<0.001). No differences between the 2 groups were observed regarding long-term remission, therapy response, and high disability scores (modified Rankin Scale score, 4-6) or death at last follow-up. Spinal cord involvement was associated with meningeal enhancement on brain imaging (odds ratio, 10.50) and the presence of lymphoma (odds ratio, 6.49), specifically Hodgkin lymphoma diagnosed simultaneously with PCNSV. Spinal involvement was negatively associated with increasing age (odds ratio, 0.64) and cerebral infarction on imaging (odds ratio, 0.08).

Conclusions: Spinal cord involvement defines a distinct subset of patients with PCNSV.

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原发性中枢神经系统血管炎伴脊髓受累：一项40年的单中心研究

背景：原发性中枢神经系统血管炎（PCNSV）累及脊髓的报道并不多见。本研究描述了一大批PCNSV患者的临床表现、治疗反应和脊髓受累的结果。方法：我们回顾性研究了1983年至2023年在梅奥诊所接受评估的216例连续PCNSV患者。脊髓受累通过组织学检查或影像学证实。对有无脊髓受累患者的临床、实验室、放射学和病理结果以及治疗和预后进行比较。结果：216例患者中，脊髓受累10例（4.6%），脑受累9例。1例（0.5%）有孤立性脊髓血管炎。所有10例患者均有血管炎的组织学证据，其中5例（50%）为坏死性血管炎。磁共振成像显示胸部异常8例，颈椎受累2例，髓圆锥受累3例，马尾增强4例。与未受累脊髓的206例患者相比，有脊髓血管炎的患者出现麻痹/四肢麻痹的可能性更大（PP=0.01），出现偏瘫（P=0.006）和肉芽肿性血管炎的可能性更小（P=0.03）。脊髓受累的患者更有可能有至少1次复发(结论：脊髓受累定义了PCNSV患者的一个独特亚群。

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

Stroke 医学-临床神经学

CiteScore

13.40

自引率

6.00%

发文量

2021

审稿时长

3 months

期刊介绍： Stroke is a monthly publication that collates reports of clinical and basic investigation of any aspect of the cerebral circulation and its diseases. The publication covers a wide range of disciplines including anesthesiology, critical care medicine, epidemiology, internal medicine, neurology, neuro-ophthalmology, neuropathology, neuropsychology, neurosurgery, nuclear medicine, nursing, radiology, rehabilitation, speech pathology, vascular physiology, and vascular surgery. The audience of Stroke includes neurologists, basic scientists, cardiologists, vascular surgeons, internists, interventionalists, neurosurgeons, nurses, and physiatrists. Stroke is indexed in Biological Abstracts, BIOSIS, CAB Abstracts, Chemical Abstracts, CINAHL, Current Contents, Embase, MEDLINE, and Science Citation Index Expanded.