Takewaki Daiki, Tsuji Yukiko, Kasai Takashi, Yoshida Tomokatsu, Nakagawa Masanori, Mizuno Toshiki
Department of Neurology, Kyoto Prefectural University of Medicine.
Rinsho Shinkeigaku. 2015;55(11):844-7. doi: 10.5692/clinicalneurol.cn-000719. Epub 2015 Oct 10.
We report a 76-year-old male with ANCA-associated hypertrophic pachymeningitis, who presented with crescentic glomerulonephritis. At the initial visit, he had episodic frontal headache and multiple cranial nerve palsy, including double vision, right deafness, hoarseness, and dysphagia. Because proteinuria and hematuria were detected on urinalysis, we performed a kidney biopsy, leading to the diagnosis of crescentic glomerulonephritis. The presence of vascular inflammation in the kidney biopsy led us to consider that this patient may show progression to the systemic type of MPO-ANCA-positive hypertrophic pachymeningitis. This proved useful for prognostic and treatment determination. Based on the results of laboratory tests, imaging studies, and biopsies of the dura mater and kidney, the patient was diagnosed with ANCA-associated hypertrophic pachymeningitis.
我们报告了一名76岁的男性，患有抗中性粒细胞胞浆抗体（ANCA）相关的肥厚性硬脑膜炎，并伴有新月体性肾小球肾炎。初诊时，他有发作性额部头痛和多组颅神经麻痹，包括复视、右耳聋、声音嘶哑和吞咽困难。由于尿液分析检测到蛋白尿和血尿，我们进行了肾活检，诊断为新月体性肾小球肾炎。肾活检中存在血管炎症，使我们认为该患者可能会进展为系统性MPO-ANCA阳性肥厚性硬脑膜炎。这对预后和治疗决策很有帮助。根据实验室检查、影像学研究以及硬脑膜和肾脏活检结果，该患者被诊断为ANCA相关的肥厚性硬脑膜炎。
