Zhu Min, Zheng JunJun, Zhu Yuanzhao, Wan Hui, Wu Yuchen, Hong Daojun
Department of Neurology, The First Affiliated Hospital of Nanchang University, Yong Wai Zheng Street 17#, Nanchang, 330006, P.R. China.
BMC Neurol. 2015 May 10;15:77. doi: 10.1186/s12883-015-0341-1.
Primary diffuse leptomeningeal gliomatosis (PDLG) is a lethal neoplasm that is characterized by glioma cells exclusively infiltrating into cerebral and spinal meninges. Intraventricular hemorrhage as an initial symptom in PDLG patient has not been reported in the literatures.
A 39-year-old man initially presented with intraventricular hemorrhage. The patient had an improved outcome at the early stage of hemorrhagic course; however, the clinical condition began to a sudden turn for deterioration with intracranial hypertension and cerebral hernia on day 15 after admission. Cerebral CT and MRI showed diffuse patchy signals with enhancement in bilateral cerebellopontine angle cistern, suprasellar cistern, ambient cistern, quadrigeminal cistern, bilateral cerebellum, cerebral hemisphere, and upper cervical cord surface. Pathological examination revealed that numerous spindled cells were scant of cytoplasm with hyperchromatic nuclei and various mitotic figures. Immunohistochemistry showed that the cells were positive to glial fibrillary acidic protein (GFAP) with about 5% Ki-67 positive labeling. The pathological findings were consistent with the diagnostic criteria of anaplastic astrocytoma (WHO grade III).
We reported an interesting case that PDLG initially presented with intraventricular hemorrhage that might be caused by astrocytoma rupturing into pial vessels.
原发性弥漫性软脑膜胶质瘤病（PDLG）是一种致命性肿瘤，其特征是胶质瘤细胞仅浸润至脑和脊髓脑膜。文献中尚未报道过PDLG患者以脑室内出血为首发症状。
一名39岁男性最初表现为脑室内出血。在出血病程早期患者病情有所改善；然而，入院后第15天临床状况突然恶化，出现颅内高压和脑疝。脑部CT和MRI显示双侧小脑脑桥角池、鞍上池、环池、四叠体池、双侧小脑、大脑半球及颈髓上段表面有弥漫性斑片状强化信号。病理检查显示大量梭形细胞，胞质稀少，核染色质增多，可见各种有丝分裂象。免疫组化显示细胞胶质纤维酸性蛋白（GFAP）阳性，约5%的Ki-67阳性标记。病理结果符合间变性星形细胞瘤（世界卫生组织III级）的诊断标准。
我们报告了一例有趣的病例，PDLG最初表现为脑室内出血，可能是星形细胞瘤破裂进入软脑膜血管所致。
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