Matà Sabrina, Muscas Gian Carlo, Cincotta Massimo, Bartolozzi Maria Letizia, Ambrosini Stefano, Sorbi Sandro
Department of Neurological and Psychiatric Sciences, Azienda Ospedaliero-Universitaria di Careggi, Florence, Italy.
J Neuroimmunol. 2010 Oct 8;227(1-2):175-7. doi: 10.1016/j.jneuroim.2010.07.011. Epub 2010 Aug 8.
We investigated the prevalence and the clinical association of high titer of antibodies against glutamic acid decarboxylase (hGADAb) among unselected patients with inflammatory/autoimmune disorders of the nervous system. By indirect immunofluorescence examination of samples from 1435 patients, we identified 7 cases (0.48%) with hGADAb. Although stiff-person plus syndrome was the commonest clinical accompaniment, most of the patients presented with a combination of different symptoms, including psychiatric disturbances and intestinal motility disorders. Diagnosis delay and chronic evolution were common findings. In two cases persistently high values of hGADAb over the years were observed. The rarity and the phenotype heterogeneity of hGADAb clinical association should not discourage clinicians from antibody screening, at least in selected cases, as an early immunotherapy can change the otherwise chronic progression of this complex disorder spectrum.
我们在未经挑选的神经系统炎性/自身免疫性疾病患者中，调查了高滴度谷氨酸脱羧酶抗体（hGADAb）的患病率及其临床相关性。通过对1435例患者的样本进行间接免疫荧光检查，我们识别出7例（0.48%）hGADAb阳性患者。尽管僵人加综合征是最常见的临床伴随症状，但大多数患者表现为不同症状的组合，包括精神障碍和肠道运动障碍。诊断延迟和慢性病程演变是常见的发现。在两例患者中，观察到多年来hGADAb值持续升高。hGADAb临床相关性的罕见性和表型异质性不应阻碍临床医生进行抗体筛查，至少在某些特定病例中如此，因为早期免疫治疗可以改变这种复杂疾病谱原本的慢性进展。
