Fayet G, Bertout-Lemercier C, Gugenheim M, Auffray E, Jardel-Bouissière J, Lacroix C, Cuillière P, Lhuillier de Cordoze M, Fève J R
Clinique Neurologique, Hôpital Laënnec, Nantes.
Rev Neurol (Paris). 1994 Oct;150(10):700-3.
Leptomeningeal gliomatosis is a diffuse glial infiltration of the subarachnoid space. It is primary and very rare when primary astrocytoma arises in the leptomeninges from heterotopic neuroglial tissue; it is secondary and more frequently reported when associated with a medullar or cerebral intraparenchymal astrocytoma and secondary involvement of the leptomeninges. Primary and secondary forms are difficult to differentiate before neuropathological examination. The authors report 2 anatomo-clinical cases of leptomeningeal gliomatosis in adults, with clinical courses of 6 months and 40 days respectively. The initial clinical picture was aseptic chronic or subacute meningitis. Cytologic examinations of the cerebrospinal fluid (CSF) showed moderate lymphocytosis, with elevated protein and low glucose levels, without abnormal cells. On case 2 CT scan and in case 1 spinal MRI isolated diffuse meningeal contrast enhancement was present, without intraparenchymal lesion. The neuropathological study revealed a diffuse astrocytoma glial leptomeningeal tumour with a focal involvement of the central nervous system (spinal cord in one case, temporal lobe in the other). In conclusion, an isolated aseptic lymphocytosis meningitis with meningeal abnormal signal may reveal leptomeningeal gliomatosis. Neuropathological examination can distinguish primary from secondary forms.
软脑膜胶质瘤病是蛛网膜下腔的弥漫性胶质浸润。它是原发性的，当原发性星形细胞瘤起源于异位神经胶质组织的软脑膜时非常罕见；它是继发性的，当与髓内或脑实质内星形细胞瘤相关且软脑膜继发受累时更常被报道。在神经病理学检查之前，原发性和继发性形式很难区分。作者报告了2例成人软脑膜胶质瘤病的解剖临床病例，临床病程分别为6个月和40天。最初的临床表现为无菌性慢性或亚急性脑膜炎。脑脊液（CSF）的细胞学检查显示中度淋巴细胞增多，蛋白水平升高，葡萄糖水平降低，无异常细胞。病例2的CT扫描和病例1的脊髓MRI显示孤立的弥漫性脑膜对比增强，无实质内病变。神经病理学研究显示为弥漫性星形细胞瘤性软脑膜胶质肿瘤，中枢神经系统有局灶性受累（1例为脊髓，另1例为颞叶）。总之，孤立的无菌性淋巴细胞性脑膜炎伴脑膜异常信号可能提示软脑膜胶质瘤病。神经病理学检查可以区分原发性和继发性形式。
