Intravenous Immunoglobulin Therapy for Autoantibody-Positive Cerebellar Ataxia

Intravenous Immunoglobulin Therapy for Autoantibody-Positive Cerebellar Ataxia
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DOI:
10.2169/internalmedicine.48.1802
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发表时间:
2009-01-01
期刊:
影响因子:
1.2
通讯作者:
Koizumi, Kiyoshi
Koizumi, Kiyoshi
中科院分区:
医学4区
文献类型:
--
作者:
Nanri, Kazunori;Okita, Mitsunori;Koizumi, Kiyoshi

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目的自身免疫性小脑性共济失调,如抗谷氨酸脱羧酶(GAD)抗体阳性的小脑性共济失调和谷蛋白共济失调是可以治疗的。在这里,我们观察了静脉注射免疫球蛋白(IVIg)对自身抗体阳性的小脑性共济失调的治疗效果。结果4例小脑皮质萎缩患者(2例抗GAD抗体阳性,2例抗醇溶蛋白抗体阳性)和1例抗甲状腺抗体阳性的脊髓小脑型共济失调(SCA3)患者治疗有效,2例多系统萎缩(MSA)患者无效。接受SPECT检查的4例IVIg有效的患者均有明显的小脑血流灌注增加。结论如果怀疑有自身免疫机制的小脑性共济失调,而放射学结果未显示MSA,则值得考虑包括IVIg在内的免疫治疗。
Objective It has been reported that autoimmune cerebellar ataxias, such as anti-glutamic acid decarboxylase (GAD)-antibody-positive cerebellar ataxia and gluten ataxia, are treatable. Here, we examined the therapeutic efficacy of intravenous immunoglobulin (IVIg) on autoantibody-positive cerebellar ataxia.Patients and Methods IVIg therapy was administered in seven autoantibody-positive cerebellar ataxia patients. Therapeutic efficacy was examined in terms of its effects on clinical symptoms and changes in brain perfusion using single photon emission computed tomography (SPECT).Results Treatment was effective in four cerebellar cortical atrophy patients (two anti-GAD antibody-positive and two anti-gliadin antibody-positive) and in one anti-thyroid antibody-positive spinocerebellar ataxia type 3 (SCA3) patient, but not in two multiple system atrophy (MSA) patients. All four IVIg effective patients who underwent SPECT showed apparent increases in cerebellar perfusion.Conclusion If cerebellar ataxia with an autoimmune mechanism is suspected and radiological findings do not reveal MSA, it is worth considering immunotherapy including IVIg.