Marsh Elisabeth B, Zeiler Steven R, Levy Michael, Llinas Rafael H, Urrutia Victor C
Department of Neurology, Johns Hopkins University, Baltimore, MD, USA.
Neurologist. 2012 Jul;18(4):233-8. doi: 10.1097/NRL.0b013e31825c6d23.
Primary central nervous system vasculitis (PCNSV) is a rare inflammatory arteriopathy confined to the brain, spinal cord, and leptomeninges. Because of its nonspecific presentation and difficulties in making a positive diagnosis, empiric treatment is often instituted.
We report a case series of 5 patients who were admitted or transferred to the Johns Hopkins Hospital with a clinical history and magnetic resonance imaging findings suggestive of PCNSV. Four patients had received at least 1 course of immunosuppression with high-dose intravenous (IV) corticosteroids and/or a corticosteroid-sparing agent. Each underwent an extensive workup including 4-vessel cerebral angiography and, in the majority of cases, brain biopsy to evaluate for mimics of PCNSV. In each of the 5 cases, an alternative diagnosis was found.
We propose a cautious, multistep approach to the diagnosis of PCNSV, which takes into account more common diagnoses and avoids the pitfalls of empiric treatment.
原发性中枢神经系统血管炎（PCNSV）是一种罕见的炎症性动脉病，局限于脑、脊髓和软脑膜。由于其表现不具特异性且难以做出肯定诊断，常进行经验性治疗。
我们报告了一组5例患者的病例系列，这些患者因临床病史和磁共振成像结果提示PCNSV而被收治或转至约翰霍普金斯医院。4例患者接受了至少1个疗程的免疫抑制治疗，使用大剂量静脉注射（IV）糖皮质激素和/或糖皮质激素替代药物。每例患者都接受了全面检查，包括四血管脑血管造影，并且在大多数病例中进行了脑活检，以评估是否为PCNSV的模仿疾病。在这5例病例中，每例都发现了另一种诊断。
我们提出了一种谨慎的、多步骤的PCNSV诊断方法，该方法考虑了更常见的诊断，并避免了经验性治疗的陷阱。
