![]() ![]() Cerebrospinal fluid (CSF) in autoimmune GFAP astrocytopathy usually shows an inflammatory pattern with a predominance of lymphocytes, elevated protein levels, and elevated immunoglobulins ( 2, 4, 5). Most of the disease has flu-like prodromal infection symptoms, which manifest as nonspecific subacute meningeal symptoms (headache, fever, photophobia, neck stiffness), myelopathy symptoms (weakness or numbness), memory loss, blurred consciousness, or blurred vision (optic disc edema), and other symptoms including dyskinesia, autonomic dysfunction, and cerebellar ataxia ( 1– 3). ![]() This case report aims to increase neurologists’ awareness of this disease and avoid missed or misdiagnosed cases that may lead to delayed treatment.Īutoimmune GFAP astrocytopathy is an autoimmune-mediated disease of the central nervous system (CNS) that can affect the entire neuraxis and was first proposed in 2016 ( 1). Subsequently, the presence of anti-GFAP antibodies was detected in the cerebrospinal fluid (CSF), and the diagnosis of autoimmune GFAP astrocytopathy in conjunction with low-flow PMAVFs was confirmed through spinal digital subtraction angiography (DSA). Multiple patchy abnormal signals were seen adjacent to the anterior and posterior horns of the lateral ventricles bilaterally and at the centers of the semi-ovals on MRI of the cranial brain, with iso signal in T1Flair, the high signal in T2WI, and no high signal seen in Diffusion Weighted Imaging (DWI). Magnetic resonance imaging (MRI) of the spinal cord revealed a significant longitudinal extent of T2 high signal from T11 to L1, with the lesion located proximal to the vascular territory supplied by the anterior spinal artery. We report a middle-aged male patient with autoimmune GFAP astrocytopathy complicated with low flow PMAVFs disease, presenting with lower extremity weakness and dysuria. 5Department of Neurology, General Hospital of Ningxia Medical University, Yinchuan, ChinaĪutoimmune glial fibrillary acidic protein (GFAP) astrocytopathy and low-flow perimedullary arteriovenous fistulas (PMAVFs) may cause longitudinal widespread myelopathy.4Department of Neurology, Cardiovascular and Cerebrovascular Disease Hospital Branch, General Hospital of Ningxia Medical University, Yinchuan, China.3Diagnosis and Treatment Engineering Technology Research Center of Nevous System Disease of Ningxia Hui Autonomous Region, Yinchuan, China.2School of Clinical Medicine, Ningxia Medical University, Yinchuan, China.1Department of Neuroelectrophysiology, Cardiovascular and Cerebrovascular Disease Hospital Branch, General Hospital of Ningxia Medical University, Yinchuan, China.Ting Xu 1† Jingyun Chen 1† Tingting Xuan 2 Jiang Cheng 3,4* Haining Li 3,5* ![]()
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