Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA.
Neurology. 2011 May 17;76(20):1705-10. doi: 10.1212/WNL.0b013e31821a44f1.
To describe a patient presenting with a clinically silent, incidentally found, and pathologically confirmed active demyelinating solitary cortical lesion showing MRI gadolinium contrast enhancement, in whom biopsy was performed before the radiographic appearance of disseminated white matter lesions.
Neurologic examination, MRI, CSF and serologic analyses, and brain biopsy were performed. Sections of formalin-fixed paraffin-embedded biopsied brain tissue were stained with histologic and immunohistochemical stains.
Biopsy revealed an inflammatory subpial lesion containing lymphocytes and myelin-laden macrophages. Recurrent relapses with dissemination of MRI-typical white matter lesions characterized the subsequent course.
Our findings highlight that cortical demyelination occurs on a background of inflammation and suggest that the noninflammatory character of chronic cortical demyelination may relate to long intervals between lesion formation and autopsy. This case provides pathologic evidence of relapsing-remitting MS presenting with inflammatory cortical demyelination and emphasizes the importance of considering demyelinating disease in the differential diagnosis of patients presenting with a solitary cortical enhancing lesion.
描述一位患者出现临床无症状、偶然发现、经病理证实的活性脱髓鞘单发皮质病变，表现为 MRI 钆对比增强，在弥散性脑白质病变的影像学表现之前进行了活检。
进行了神经学检查、MRI、CSF 和血清学分析以及脑活检。对福尔马林固定石蜡包埋的活检脑组织进行了组织学和免疫组织化学染色。
活检显示炎症性软脑膜下病变，包含淋巴细胞和含髓鞘的巨噬细胞。随后的病程中出现了 MRI 典型白质病变的复发和扩散。
我们的发现强调了皮质脱髓鞘发生在炎症背景下，并提示慢性皮质脱髓鞘的非炎症特征可能与病变形成和尸检之间的长间隔有关。该病例提供了复发缓解型多发性硬化症以炎症性皮质脱髓鞘为表现的病理证据，并强调了在鉴别诊断表现为单发皮质增强病变的患者时，考虑脱髓鞘疾病的重要性。
