Division of Neurology, Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada.
Can J Neurol Sci. 2011 Jan;38(1):82-7. doi: 10.1017/s0317167100011112.
Caseating granuloma is a classic histopathological feature of mycobacterial infections. Occasionally, no infectious organism is demonstrated despite extensive examination of intracranial caseating granulomas. The pathogenesis and optimal management strategy for patients with such intracranial caseating granulomas with no detectable infectious organism (ICGNs) remain unclear.
The study was a retrospective case-series design in a referral hospital setting. Patients with intracranial caseating granulomas in whom no infectious etiology was identified after appropriate investigations were reviewed.
Eight patients with ICGN (four females and four males) were identified in an eight-year-period. Average age on presentation was 46 years (range 21-69 years). Cerebrospinal fluid showed lymphocytic pleocytosis, elevated protein and decreased glucose. Neuroimaging showed multiple or single intraparenchymal and meningeal enhancements. Intracranial ICGN were demonstrated on biopsy. Immunomodulation was tried and resulted in improvement in five out of eight patients. In four patients, anti-mycobacterial treatment resulted in no improvement or worsening of clinical or radiological features.
The response to therapy of intracranial caseating granulomas where no organism is identified after thorough investigations hints to non-infectious causes, and suggests current dogma regarding the significance of necrosis in granulomatous diseases should be re-evaluated. Our retrospective series suggests that patients may benefit from an early trial of immunomodulation therapy, a hypothesis to be tested in a randomized trial.
干酪样肉芽肿是分枝杆菌感染的经典组织病理学特征。尽管对颅内干酪样肉芽肿进行了广泛检查，但偶尔仍无法发现任何感染病原体。对于没有可检测到的感染病原体的颅内干酪样肉芽肿（ICGN）患者，其发病机制和最佳管理策略仍不清楚。
本研究是在一家转诊医院进行的回顾性病例系列设计。对经过适当检查后仍未确定颅内干酪样肉芽肿感染病原体的患者进行了回顾性分析。
在 8 年期间共发现 8 例 ICGN（4 名女性和 4 名男性）患者。就诊时的平均年龄为 46 岁（范围 21-69 岁）。脑脊液显示淋巴细胞增多、蛋白升高和葡萄糖降低。神经影像学显示多发或单发脑实质和脑膜增强。活检显示颅内 ICGN。尝试免疫调节治疗后，8 例患者中有 5 例得到改善。在 4 例患者中，抗分枝杆菌治疗后临床或影像学特征无改善或恶化。
对经过彻底检查后仍未发现病原体的颅内干酪样肉芽肿进行治疗的反应提示可能为非感染性原因，这表明目前关于坏死在肉芽肿性疾病中的意义的教条应该重新评估。我们的回顾性研究表明，患者可能受益于早期免疫调节治疗试验，这一假说有待随机试验进行检验。
