Department of Neurology, Fondation Ophtalmogique Adolphe de Rothschild, 25 rue Manin, 75019, Paris, France.
Department of Neuroradiology, Fondation ophtalmogique Adolphe de Rothschild, Paris, France.
Metab Brain Dis. 2018 Feb;33(1):353-355. doi: 10.1007/s11011-017-0141-y. Epub 2017 Oct 31.
The radiological spectrum of neuromyelitis optica has become broader since the detection of aquaporin4 antibodies. We report a case of neuromyelitis optica patient with pseudotumoral encephalic lesion. A 66 year-old woman presented with sudden left lateral homonymous hemianopsia. A brain MRI showed an isolated and extensive right temporo-parieto-occipital lesion, involving periventricular white matter and the corpus callosum, with strong enhancement on post-gadolinium T1 weighted images, highly suggestive of lymphoma. Spinal cord MRI and body CT scan were unremarkable. Lumbar puncture showed pleocytosis, raised total protein level without abnormal cells or oligoclonal bands. A brain biopsy demonstrated non-specific demyelination. Serum aquaporin4 antibodies were positive, which was consistent with the diagnosis of neuromyelitis optica. Cases of central nervous system aquaporin4 autoimmunity presenting with an isolated brain lesion without optic neuritis or myelitis are extremely rare: this is the second case so far and the first one with advanced magnetic resonance characterization. Pseudotumoral encephalic lesions should include a large differential diagnosis, and testing aquaporin4 antibodies must be considered in order to avoid brain biopsy.
自从检测到水通道蛋白 4 抗体后，视神经脊髓炎的放射学谱变得更加广泛。我们报告了一例伴有假性脑瘤性脑内病变的视神经脊髓炎患者。一名 66 岁女性突发左侧同向性偏盲。脑部 MRI 显示孤立且广泛的右侧颞顶枕叶病变，累及脑室周围白质和胼胝体，钆后 T1 加权图像上有强烈强化，高度提示为淋巴瘤。脊髓 MRI 和全身 CT 扫描无明显异常。腰椎穿刺显示细胞增多，总蛋白水平升高，无异常细胞或寡克隆带。脑活检显示非特异性脱髓鞘。血清水通道蛋白 4 抗体阳性，符合视神经脊髓炎的诊断。中枢神经系统水通道蛋白 4 自身免疫性疾病表现为孤立性脑病变而无视神经炎或脊髓炎的病例极为罕见：这是目前为止的第二例，也是首例具有先进磁共振特征的病例。假性脑瘤性脑病变的鉴别诊断范围很广，因此必须考虑检测水通道蛋白 4 抗体，以避免进行脑活检。
