Primary diffuse leptomeningeal gliomatosis predominantly affecting the spinal cord: case report and review of the literature
Primary diffuse leptomeningeal gliomatosis predominantly affecting the spinal cord: case report and review of the literature
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DOI：
10.1136/jnnp.70.2.256
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发表时间：
2001-02-01
影响因子：
11
通讯作者：
Bamford, JM
中科院分区：
文献类型：
--
作者：
Baborie, A;Dunn, EM;Bamford, JM
原发性软脑膜胶质瘤病是一种罕见的，致命的肿瘤综合征。报告一位71岁男性，在背部僵硬、上腹痛和体重减轻2个月后出现视力模糊。头颅CT和MRI检查显示无软脑膜强化。尸检前10周脑脊液检查显示蛋白质增加，葡萄糖减少，但无恶性细胞。他变得越来越困惑，反复的CSF检查显示炎症和一些可疑细胞，但没有明确的肿瘤证据。他在最初症状出现7个月后死亡。尸检时，在大脑底部和脊髓上方观察到脑膜变白。组织学检查显示脑、视神经和脊髓弥漫性软脑膜胶质瘤病（GFAP阳性，细胞角蛋白阴性），无实质受累。内脏无肿瘤。原发性软脑膜胶质瘤病的诊断在头颅CT、MRI和CSF检查后均不明显。疑似病例需要进行整个脊髓的MRI扫描和脑膜活检。
Primary leptomeningeal gliomatosis is a rare, fatal neoplastic syndrome. A 71 year old man is reported on, who after a 2 month history of back stiffness, epigastric pain, and weight loss developed visual blurring. Cranial CT and MRI studies showed no leptomeningeal enhancement. Examination of CSF 10 weeks premortem showed an increase in protein and decrease in glucose but no malignant cells. He became increasingly confused and repeated CSF examination showed inflammation and a few suspicious cells but no definitive evidence of neoplasia. He died 7 months after onset of his initial symptoms. At postmortem meningeal whitening was seen at the base of the brain and over the spinal cord. Histology disclosed diffuse leptomeningeal gliomatosis (GFAP positive, cytokeratin negative) over the brain, optic nerves, and spinal cord without parenchymal involvement. No tumour was found in internal organs. The diagnosis of primary leptomeningeal gliomatosis was not evident after cranial CT and MRI and CSF examination premortem. Suspected cases need MRI scanning of the entire neuraxis and meningeal biopsy.