Chukwuocha Ikechukwu, Shukla Shilpi, Bose Smriti, Ubben Simon, Hayton Tom, Veenith Tonny, Jacob Saiju
Department of Neurology, New Cross Hospital, Royal Wolverhampton NHS Trust, Wolverhampton, UK.
Department of Neurology, University Hospital Birmingham NHS Trust, UK.
Clin Neurol Neurosurg. 2025 Jul 8;257:109047. doi: 10.1016/j.clineuro.2025.109047.
We describe a teenage lady who presented with headache, fever, neck stiffness, torticollis, progressive disturbance of consciousness as well as hemodynamic instability and subsequently developed quadriparesis. CSF revealed lymphocytic pleocytosis, elevated protein and low glucose with negative microbial studies. Her clinical course was complicated by acute encephalopathy requiring ITU admission and intubation. Full neuroaxis MRI revealed focal cerebritis with perivenular radial enhancement, and a non-enhancing longitudinally extensive myelitis. Positive Autoimmune glial fibrillary acidic protein (GFAP) IgG was subsequently identified in the CSF via immunofluorescence (IF) assay. CT thorax, abdomen and pelvis was negative for neoplasms. She was initially treated with pulsed intravenous corticosteroids with minimal improvement; however, following plasmapheresis she made a complete recovery of her neurological function. Long-term treatment consisted of oral prednisolone taper and azathioprine to lower the risk of early relapse. GFAP astrocytopathy is a novel autoimmune disease of the central nervous system. It is especially easy to misdiagnose and as such it is imperative to recognize the clinical spectrum of this disorder as early detection and treatment should improve prognosis.
我们描述了一位青少年女性，她出现头痛、发热、颈部僵硬、斜颈、意识进行性障碍以及血流动力学不稳定，随后发展为四肢瘫痪。脑脊液显示淋巴细胞增多、蛋白升高、葡萄糖降低，微生物学检查为阴性。她的临床病程因急性脑病而复杂化，需要入住重症监护病房并进行插管。全神经轴MRI显示局灶性脑炎伴血管周围放射状强化，以及非强化的纵向广泛脊髓炎。随后通过免疫荧光（IF）检测在脑脊液中发现了阳性的自身免疫性胶质纤维酸性蛋白（GFAP）IgG。胸部、腹部和骨盆CT检查未发现肿瘤。她最初接受了脉冲静脉注射皮质类固醇治疗，改善甚微；然而，在进行血浆置换后，她的神经功能完全恢复。长期治疗包括逐渐减量的口服泼尼松龙和硫唑嘌呤，以降低早期复发的风险。GFAP星形细胞病是一种新型的中枢神经系统自身免疫性疾病。它特别容易误诊，因此必须认识到这种疾病的临床谱，因为早期发现和治疗应能改善预后。
