Department of Neurology, Neurovascular Division, University of California, 505 Parnassus Avenue, Box 0114, San Francisco, California, 94143, USA.
Weill Institute for Neurosciences, University of California, San Francisco, California, USA.
BMC Neurol. 2021 May 21;21(1):205. doi: 10.1186/s12883-021-02228-2.
Heterogenous central nervous system (CNS) neurologic manifestations of polyarteritis nodosa (PAN) are underrecognized. We review three cases of patients with PAN that illustrate a range of nervous system pathology, including the classical mononeuritis multiplex as well as uncommon brain and spinal cord vascular manifestations.
Case 1 presented with mononeuritis multiplex and characteristic skin findings. Case 2 presented with thunderclap headache and myelopathy due to spinal artery aneurysm rupture. Both patients experienced disease remission upon treatment. Case 3 presented with headache and bulbar symptoms due to partially thrombosed intracranial aneurysms, followed by systemic manifestations related to visceral aneurysms. She demonstrated clinical improvement with treatment, was lost to follow-up, then clinically deteriorated and entered hospice care.
Although the peripheral manifestations of PAN are well-known, PAN association with CNS neurovascular disease is relatively underappreciated. Clinician awareness of the spectrum of neurologic disease is required to reduce diagnostic delay and promote prompt diagnosis and treatment with immunosuppressants.
结节性多动脉炎 (PAN) 的中枢神经系统 (CNS) 表现多样,容易被漏诊。我们回顾了三例 PAN 患者的病例,这些患者表现出多种神经系统病理改变,包括经典的多发性单神经炎以及不常见的脑和脊髓血管表现。
例 1 表现为多发性单神经炎和典型的皮肤表现。例 2 表现为霹雳性头痛和脊髓动脉动脉瘤破裂导致的脊髓病。这两位患者在治疗后均出现疾病缓解。例 3 表现为部分血栓形成的颅内动脉瘤导致的头痛和球部症状,随后出现与内脏动脉瘤相关的全身表现。她在治疗后临床症状有所改善,但随后失访,病情恶化并进入临终关怀。
尽管 PAN 的外周表现广为人知,但 PAN 与 CNS 神经血管疾病的关联相对被低估。临床医生需要意识到神经疾病的范围,以减少诊断延误,并促进及时诊断和免疫抑制剂治疗。