BACKGROUND Occurrence of basal ganglia involvement in neuromyelitis optica spectrum disorders (NMOSD) has rarely been reported and none documented pathologically. CASE PRESENTATION A 73-year-old female was clinically diagnosed with a NMOSD based on the clinical and radiological features and positive serum autoantibodies to AQP4. One month before her death, she became acutely ill with disturbed consciousness and right hemiparesis, and was diagnosed and treated as having basal ganglia infarction based on the brain CT. She made a partial recovery but later died from heart failure. At autopsy, the corresponding basal ganglia process revealed a large fresh area of necrosis. Histologically, several pathological signatures of NMOSD could be recognized in the lesion, including inflammatory cell infiltrations by B and T lymphocytes, perivascular complement and fibrinogen deposition, and the appearance of numerous phagocytosed corpora amylacea within the infiltrating macrophages. CONCLUSIONS The present case illustrates that basal ganglia may be directly involved in the pathological processes of NMOSD, although the possibility of modification of the lesions by superimposed regional ischemia could not be excluded.

中文翻译：

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大量基底神经节受累的视神经脊髓炎频谱疾病：一例报告。

背景技术很少有人报道基底节神经节累及视神经脊髓炎频谱疾病（NMOSD），并且没有病理记录。病例介绍根据临床和放射学特征以及针对AQP4的血清自身抗体阳性，临床诊断为一名73岁女性为NMOSD。在她去世前一个月，她因意识障碍和右半偏瘫而患上了严重疾病，并根据脑部CT被诊断为基底节梗塞。她部分康复，但后来因心力衰竭死亡。尸检时，相应的基底神经节突显出大量新鲜的坏死区域。组织学上，病变中可以识别出NMOSD的几种病理学特征，包括B和T淋巴细胞对炎症细胞的浸润，血管周围补体和纤维蛋白原沉积，以及浸润性巨噬细胞内大量吞噬的淀粉体的出现。结论本病例说明基底神经节可能直接参与了NMOSD的病理过程，尽管不能排除由于局部缺血引起的病变改变的可能性。