Chiba Kentaro, Aihara Yasuo, Eguchi Seiichiro, Tanaka Masahiko, Komori Takashi, Nakazato Yoichi, Okada Yoshikazu
Department of Neurosurgery, Tokyo Women's Medical University, 8-1 Kawada-cho Shinjuku-ku, Tokyo, Japan.
Childs Nerv Syst. 2013 Jun;29(6):1039-45. doi: 10.1007/s00381-013-2100-1. Epub 2013 Apr 19.
Juvenile xanthogranuloma (JXG) preferentially occurs in childhood, and its characteristics have been thought to be benign and with slow growth. JXG is classified as an inflammatory disease, which forms multiple lesions in the patients' head, neck, and other organs and typically skin. JXG is rare, and few case reports have been published in the past, particularly with multiple intracranial lesions, which as in our case, is an extremely rare finding.
Patient is a 4-year-old boy who presented with polydypsia and polyuria for 1 year. He had been followed up only under l-deamino-8-D-arginine vasopressin until he started vomiting and his level of consciousness deteriorated. Then, magnetic resonance imaging (MRI) revealed multiple intracranial lesions. He underwent biopsy via small craniotomy, and pathology was confirmed as juvenile xanthogranuloma. The patient received chemotherapy, in complete compliance with JLSG-02 protocol.
JXG is characteristic with homogenous enhancement with contrast matter and, most importantly, high intensity on diffusion-weighted image (DWI). The DWI was high when the degree of diffusion of water is restricted, as seen in inflammation and, additionally, the change of intensity after administration of steroid would reflect its anti-inflammatory nature. However, though the steroid therapy made high-intensity lesions decrease signal intensity, the size and the number of lesions did not change at all. As we expected, after we stopped steroid administration to the patient, his consciousness deteriorated and we found again the very-high-intensity lesions. We consider the intensity on DWI to reflect progression of the lesions.
幼年性黄色肉芽肿（JXG）多发生于儿童期，其特征被认为是良性且生长缓慢。JXG被归类为一种炎症性疾病，可在患者的头部、颈部及其他器官尤其是皮肤形成多个病灶。JXG较为罕见，过去发表的病例报告很少，特别是伴有多发颅内病灶的情况，如我们的病例，这是极其罕见的发现。
患者为一名4岁男孩，出现烦渴和多尿1年。在开始呕吐且意识水平恶化之前，他仅在去氨加压素治疗下接受随访。随后，磁共振成像（MRI）显示多发颅内病灶。他通过小骨窗开颅进行活检，病理确诊为幼年性黄色肉芽肿。患者接受化疗，完全遵循JLSG - 02方案。
JXG的特征是对比剂均匀强化，最重要的是在扩散加权成像（DWI）上呈高信号。当水的扩散程度受限，如在炎症中所见时，DWI呈高信号，此外，给予类固醇后信号强度的变化将反映其抗炎性质。然而，尽管类固醇治疗使高信号病灶的信号强度降低，但病灶的大小和数量根本没有改变。正如我们所预期的，在我们停止对该患者使用类固醇后，他的意识恶化，我们再次发现了极高信号病灶。我们认为DWI上的信号强度反映了病灶的进展情况。
