Silberstein P, Lawrence R, Pryor D, Shnier R
Department of Neurology, The St George Hospital, Gray Street, Kogarah NSW 2217, Australia.
J Clin Neurosci. 2002 Nov;9(6):689-90. doi: 10.1054/jocn.2002.1129.
A 37 year old man with a 2 year history of progressive cognitive decline, unilateral tinnitus and deafness presented with complex partial seizures and a fever. On examination there was a sluggish right pupillary response but no other abnormal findings. Serum and CSF syphilis serology were both strongly positive. High dose intravenous penicillin therapy was complicated by a severe Jarisch-Herxheimer reaction (JHR) characterised by fever, obtundation, fluctuating upper motor neuron signs and complex visual and auditory hallucinations. These symptoms resolved over three days and the course of penicillin was completed. At discharge the patient's cognitive functioning was unchanged from the pretreatment state. He made gradual improvement over the following months but remains unable to live alone or work. Clinical, pathologic and radiologic findings of neurosyphilis are reviewed, as is the JHR, a self-limiting, systemic febrile response related to massive cytokine release that can occur in response to treatment of a number of bacterial infections. The similarities in pathophysiology of the JHR and the Septic Shock Syndrome are discussed, with particular reference to use of the JHR as a potential model for therapeutic agents in the treatment of septic shock.
一名37岁男性，有2年进行性认知衰退、单侧耳鸣和耳聋病史，出现复杂部分性发作和发热。检查发现右侧瞳孔反应迟钝，但无其他异常发现。血清和脑脊液梅毒血清学检查均呈强阳性。大剂量静脉注射青霉素治疗出现严重的雅-赫反应（JHR），表现为发热、意识模糊、上运动神经元体征波动以及复杂的视幻觉和听幻觉。这些症状在三天内消退，青霉素疗程完成。出院时，患者的认知功能与治疗前状态相同。在接下来的几个月里他逐渐好转，但仍无法独自生活或工作。本文回顾了神经梅毒的临床、病理和影像学表现，以及雅-赫反应，这是一种与大量细胞因子释放相关的自限性全身发热反应，可发生于多种细菌感染的治疗过程中。讨论了雅-赫反应与脓毒症休克综合征在病理生理学上的相似性，特别提及将雅-赫反应作为脓毒症休克治疗中治疗药物潜在模型的应用。
