Aohara Kenta, Kurooka Keitaro, Nishikawa Masafumi, Hasegawa Itsuki, Takeda Akitoshi, Tsuji Hiroshi, Itoh Yoshiaki
Department of Neurology, Osaka Metropolitan University, Osaka, JPN.
School of Medicine, Osaka Metropolitan University, Osaka, JPN.
Cureus. 2025 May 11;17(5):e83926. doi: 10.7759/cureus.83926. eCollection 2025 May.
A 25-year-old woman presented with mild fever and fatigue that began four months prior to admission. At 30 days after onset, she developed bilateral visual field impairment with hand tremors. Gait difficulty occurred at 60 days after onset. On admission, the patient was alert and well-oriented. Examination revealed central scotomas, papilledema in both eyes, postural tremors in both hands, hyperreflexia in the limbs, positive pathological reflexes in the lower extremities, and spastic gait. Cerebrospinal fluid analysis showed an elevated cell count and protein levels as well as positive glial fibrillary acidic protein (GFAP) α antibodies. Brain magnetic resonance imaging (MRI) revealed hyperintensities in the bilateral cerebral hemispheres and dorsal brainstem on T2-weighted images. These lesions did not show enhancement with gadolinium. Methylprednisolone pulse therapy followed by oral prednisolone led to almost complete resolution of neurological symptoms and MRI abnormalities. Although GFAP astrocytopathy typically presents acutely or subacutely with severe symptoms, it should be considered when patients present with a more chronic course and relatively mild symptoms, as seen in this case. GFAP astrocytopathy should also be considered when patients present with gradually expanding white matter lesions.
一名25岁女性在入院前四个月开始出现低热和乏力。发病30天后,她出现双侧视野缺损并伴有手部震颤。发病60天后出现步态困难。入院时,患者神志清醒,定向力正常。检查发现双侧中心暗点、双眼视乳头水肿、双手姿势性震颤、四肢腱反射亢进、下肢病理反射阳性以及痉挛性步态。脑脊液分析显示细胞计数和蛋白水平升高,胶质纤维酸性蛋白(GFAP)α抗体呈阳性。脑磁共振成像(MRI)在T2加权图像上显示双侧大脑半球和脑桥背侧高信号。这些病变在注射钆剂后未显示强化。甲泼尼龙冲击治疗后口服泼尼松龙使神经症状和MRI异常几乎完全缓解。尽管GFAP星形细胞病通常急性或亚急性起病且症状严重,但当患者病程较为慢性且症状相对较轻时,如本例所见,也应考虑该病。当患者出现逐渐扩大的白质病变时,也应考虑GFAP星形细胞病。