Letournel F, Cassereau J, Scherer-Gagou C, Bernard I, Mercat A, Gray F, Tanguy J Y, Richard-Crémieux I, Jeanfaivre Th, Barthelaix A, Dubas F
UF de Neurobiologie et Neuropathologie, Département de Pathologie Cellulaire et Tissulaire, CHU, 4 rue Larrey, 49033 Angers, France.
Clin Neurol Neurosurg. 2008 May;110(5):514-7. doi: 10.1016/j.clineuro.2008.01.011. Epub 2008 Mar 14.
We report a case of a 9-month pregnant woman who presented acute psychiatric and neurological symptoms with extensive involvement of the white matter on MRI and no oligoclonal bands on CSF examination. Despite high doses of intravenous steroids, plasmapheresis and immunosuppressive drugs, a fatal outcome (coma) was noted 8 months later. Neuropathological examination confirmed the diagnosis of Marburg's type of multiple sclerosis showing sharp-edged lesions of demyelination, giant astrocytes, numerous macrophages and little perivascular inflammation. We discuss the definition and limits of the Marburg entity with reference to acute disseminated encephalomyelitis, impact of pregnancy, unusual MRI features, neuropathology and treatment.
我们报告一例9个月身孕的妇女,她出现了急性精神和神经症状,磁共振成像(MRI)显示白质广泛受累,脑脊液检查未发现寡克隆带。尽管使用了大剂量静脉类固醇、血浆置换和免疫抑制药物,但8个月后仍出现致命结局(昏迷)。神经病理学检查确诊为马尔堡型多发性硬化症,表现为边界清晰的脱髓鞘病变、巨大星形胶质细胞、大量巨噬细胞以及轻微的血管周围炎症。我们结合急性播散性脑脊髓炎讨论了马尔堡型病变的定义和局限性、妊娠的影响、不寻常的MRI特征、神经病理学及治疗。