Tanaka Nobuyuki, Otake Hiroaki, Ito Suguru, Niiyama Kazuhide, Nanri Kazunori
Department of Neurology, Tokyo Medical University Hachioji Medical Center.
Rinsho Shinkeigaku. 2012;52(5):351-5. doi: 10.5692/clinicalneurol.52.351.
We present a case of slowly progressive gait ataxia with a 16-year history in an 87-year-old woman. In 1994 she became aware of a slight unsteadiness while walking and cortical cerebellar atrophy was diagnosed. She had no familial history of neurological disorders. In 2007, idiopathic thrombocytopenic purpura (ITP) was diagnosed. The symptoms gradually worsened, and she was admitted in 2010 because she could not walk without support. MRI voxel-based morphometry (VBM) imaging showed atrophy of the entire cerebellum, and SPECT using eZIS showed reduced perfusion in the same regions. Her blood was positive for both anti-TPO antibody (42 IU/ml) and anti-gliadin antibody (20.2 EU). We therefore diagnosed autoimmune cerebellar atrophy. The patient showed a positive response to intravenous immunoglobulins (IVIg) and regained the ability to walk unassisted. Her posture and gait disturbance scores on the International Cooperative Ataxia Rating Scale had improved from 20 to 9. Even 16 years after onset, intravenous immunoglobulins were effective. In cases of prolonged disease, immunotherapy can be effective in autoimmune cerebellar atrophy and should not be excluded from the treatment choices.
我们报告一例87岁女性，有16年缓慢进展性步态共济失调病史。1994年，她走路时察觉到轻微不稳，被诊断为皮质小脑萎缩。她没有神经系统疾病家族史。2007年，诊断为特发性血小板减少性紫癜（ITP）。症状逐渐加重，2010年她因无法独立行走而入院。MRI基于体素的形态学测量（VBM）成像显示整个小脑萎缩，使用eZIS的SPECT显示相同区域灌注减少。她的血液抗TPO抗体（42 IU/ml）和抗麦醇溶蛋白抗体（20.2 EU）均呈阳性。因此，我们诊断为自身免疫性小脑萎缩。患者对静脉注射免疫球蛋白（IVIg）有阳性反应，恢复了独立行走能力。她在国际协作共济失调评定量表上的姿势和步态障碍评分从20分提高到了9分。即使发病16年后，静脉注射免疫球蛋白仍有效。对于病程较长的病例，免疫疗法对自身免疫性小脑萎缩可能有效，不应被排除在治疗选择之外。
