Kinoshita Yoshimasa, Yasukouchi Hideoki, Tsuru Eichi, Yamaguchi Rin
Department of Neurosurgery, Munakata Suikokai General Hospital, 341-1 Fukuma, Munakata, Fukuoka 811-3298, Japan.
No Shinkei Geka. 2004 Oct;32(10):1051-6.
Rosai-Dorfman disease (RDD) is a rare idiopathic histioproliferative disease affecting the lymph nodes. Extranodal sites may be involved and occasionally represent the initial or sole manifestation of the disease. Central nervous system manifestations especially are exceedingly rare. We reported a case of isolated, intracranial, dural-based RDD. A 69-year-old male, admitted with a second generalized convulsion, was examined by high-resolution MR images that revealed a thickened, stratified dura in the left frontal region with associated cortical edema. The patient underwent craniotomy with subtotal resection of the lesion. Microscopically, with the cytoplasmic staining against S-100 protein the lesion was shown to consist of proliferative histiocytes exhibiting emperipolesis. The histopathological diagnosis was compatible with RDD. The diagnosis of RDD mimicking pachymeningitis is presented, and the previous reported cases of intracranial RDD are reviewed.
罗萨伊-多夫曼病（RDD）是一种罕见的特发性组织细胞增生性疾病，可累及淋巴结。结外部位也可能受累，偶尔是该疾病的初始或唯一表现。尤其是中枢神经系统表现极为罕见。我们报告了一例孤立的、颅内硬脑膜型RDD病例。一名69岁男性因再次全身性惊厥入院，经高分辨率磁共振成像检查发现左额叶区域硬脑膜增厚、分层，并伴有皮质水肿。患者接受了开颅手术，病变部分切除。显微镜下，通过针对S-100蛋白的细胞质染色，显示病变由具有吞噬现象的增生性组织细胞组成。组织病理学诊断符合RDD。本文介绍了酷似硬脑膜炎的RDD诊断，并回顾了既往报道的颅内RDD病例。
