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[自身免疫性胶质纤维酸性蛋白星形细胞病]

[Autoimmune glial fibrillary acidic protein astrocytopathy].

作者信息

López Bisso Abril, Simison Conrado J, Manín Analisa

机构信息

División de Neurología, Hospital General de Agudos José María Ramos Mejía, Centro Universitario de Neurología José María Ramos Mejía, Facultad de Medicina, Universidad de Buenos Aires, Argentina. E-mail:

División de Neurología, Hospital General de Agudos José María Ramos Mejía, Centro Universitario de Neurología José María Ramos Mejía, Facultad de Medicina, Universidad de Buenos Aires, Argentina.

出版信息

Medicina (B Aires). 2024;84(4):769-773.

Abstract

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy was described for the first time in 2016. The most common clinical manifestation is meningoencephalomyelitis associated with a characteristic imaging pattern that allows diagnostic suspicion and its confirmation through determination of antibodies in serum and cerebrospinal fluid (CSF). We present a case of a 35-year-old patient with involvement of the central and peripheral nervous system and a recent diagnosis of thyroid cancer, which compared to the compatible clinical picture of meningoencephalomyelitis, characteristic findings on MRI and after the exclusion of alternative pathologies, we finally arrived at the diagnosis by the positive determination of anti-GFAP in CSF. The patient underwent surgical treatment and radioactive iodine for the diagnosed thyroid tumor and she subsequently received treatment with corticosteroids with partial improvement of the neurological symptomatology. We emphasize that in this pathology the MRI images usually depict a characteristic pattern, although not pathognomonic, it is necessary to consider other causes. Before a high suspicion of this entity due to the clinical and imaging picture, it is convenient to measure the antibody in CSF, given the greater sensitivity and specificity compared to its serum screening, in order to arrive to the definitive etiological diagnosis as it was done in the clinical case that is presented.

摘要

自身免疫性胶质纤维酸性蛋白(GFAP)星形细胞病于2016年首次被描述。最常见的临床表现是脑膜脑脊髓炎,伴有特征性影像学表现,这有助于诊断怀疑,并通过测定血清和脑脊液(CSF)中的抗体来确诊。我们报告一例35岁患者,其中枢和周围神经系统受累,近期诊断为甲状腺癌,与脑膜脑脊髓炎的相符临床表现、MRI特征性表现以及排除其他病变后,最终通过脑脊液中抗GFAP阳性测定确诊。该患者因诊断出的甲状腺肿瘤接受了手术治疗和放射性碘治疗,随后接受了皮质类固醇治疗,神经症状部分改善。我们强调,在这种疾病中,MRI图像通常呈现出一种特征性模式,尽管不具有特异性,但有必要考虑其他病因。鉴于与血清筛查相比,脑脊液中抗体检测具有更高的敏感性和特异性,在因临床和影像学表现高度怀疑该疾病时,检测脑脊液中的抗体有助于明确病因诊断,正如本文所呈现的临床病例那样。

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