Kastenbauer S, Danek A, Klein W, Yousry T A, Bise K, Reifenberger G, Pfister H W
Department of Neurology, Ludwig-Maximilians University, Marchioninistrasse 15, 81377 Munich, Germany.
J Neurol Neurosurg Psychiatry. 2000 Sep;69(3):385-8. doi: 10.1136/jnnp.69.3.385.
A 28 year old man presented with a 1 month history of symptoms of intracranial hypertension. Examination showed bilateral papilloedema and meningeal signs. Magnetic resonance imaging showed nodular lesions on the cerebellar and pontine surface and thickening of the thoracic spinal leptomeninges. Throughout the course of the disease, contrast enhancement was detected in the spinal leptomeninges but not intracranially. Primary diffuse leptomeningeal gliomatosis (PDLG) was diagnosed by biopsy and later confirmed on necropsy. The present case is remarkable for the nodular superficial cerebellar lesions and the absence of intracranial contrast enhancement of the leptomeninges.
一名28岁男性，有1个月的颅内高压症状病史。检查发现双侧视乳头水肿和脑膜刺激征。磁共振成像显示小脑和脑桥表面有结节状病变，胸段脊髓软脑膜增厚。在疾病过程中，脊髓软脑膜有强化表现，但颅内未出现强化。经活检诊断为原发性弥漫性软脑膜胶质瘤病（PDLG），尸检后得以证实。本病例的显著特点是小脑表面有结节状病变，且软脑膜在颅内未出现强化。
