Finally, an autoimmune GFAP astrocytopathy diagnosis was confirmed by the presence of GFAP-IgG in the CSF

Finally, an autoimmune GFAP astrocytopathy diagnosis was confirmed by the presence of GFAP-IgG in the CSF. therapy, then followed with intravenous methylprednisolone (1.0?g/d Biperiden for 3?days) and oral prednisolone. Outcomes: At 1?week after intravenous immunoglobulin therapy, his level of consciousness improved. However, flaccid paralysis persisted without substantial improvement. Conclusion: In conclusion, the provision of an accurate early diagnosis and appropriate treatment are crucial for improving the prognosis of patients with autoimmune GFAP astrocytopathy. Further, this case highlights the importance of realizing the role of peripheral nerve involvement in GFAP autoimmunity. strong class=”kwd-title” Keywords: acute disseminated encephalomyelitis, autoimmune glial fibrillary acidic protein astrocytopathy, glial fibrillary acidic protein-IgG, peripheral nervous system 1.?Introduction Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is increasingly recognized as a form of a nervous system steroid-responsive autoimmune disease. Defined in 2016, it is associated with the presence of anti-GFAP immunoglobulinG (IgG) in a patient’s serum or cerebrospinal fluid (CSF).[1,2] Most frequently GFAP astrocytopathy clinically resembles acute-onset meningoencephalitis with or without spinal cord involvement, and presents as headache, subacute encephalopathy, seizures, psychosis, cerebellar ataxia, optic neuritis, and inflammatory myelitis.[3] In addition, autoimmune GFAP astrocytopathy sometimes is usually associated with neurological and systemic autoimmunity and autoantibodies.[4] Most cases of the disease involve the presence of inflammatory CSF, Nos1 and its characteristic radiological hallmark is brain linear perivascular radial gadolinium enhancement on magnetic resonance imaging (MRI).[2] Herein, we statement a case of autoimmune GFAP astrocytopathy whose clinical manifestation, cerebrospinal fluid (CSF) results and imaging highly mimic acute disseminated encephalomyelitis. Subsequently, the expression of GFAP-IgG in the CSF lead to a final autoimmune GFAP astrocytopathy diagnosis. 2.?Case statement A 56-year-old Chinese man was admitted to the hospital with headache, a fever of 38 to 39?C for 4?days, confusion, and paralysis of the lower extremities. His medical history included hypertension and untreated psoriasis. His family history was unremarkable. A neurological examination revealed that the patient had a reduced alertness level, neck stiffness, flaccid paralysis of limbs with diffuse areflexia, and no spontaneous limb movements. Brain computed tomography revealed no edema, space-occupying lesions, intracranial hemorrhage, or selections. Blood tests showed hyponatremia (Na, 127.9?mmol/L) and hypochloremia (Cl, 91.1?mmol/L), while routine laboratory studies including routine blood, Biperiden C-reactive protein, coagulation assessments, and liver and renal function assessments were all unremarkable. He was admitted to the neurology ward with a provisional diagnosis of central nervous system infection. His condition deteriorated rapidly, and he was intubated due to coma and respiratory failure. Initial cerebrospinal fluid (CSF) analysis revealed lymphocytic pleocytosis, with a white blood cell (WBC) count of 392??106/L (normal: 0C8??106/L) and 95% lymphocytes. The CSF examination also revealed elevated protein, glucose, chloride, and adenosine deaminase (ADA) levels of 1773?mg/L (normal: 450?mg/L), 3.39?mmol/L (normal: 2.50C4.50?mmol/L), 110.0?mmol/L (normal: 120C132?mmol/L), and 4?U/L (normal: 0C25?U/L), respectively. Metagenomic next-generation sequencing of viral and bacterial genomes from your CSF was performed, which was positive exclusively for Epstein-Barr (EB) computer virus DNA. In addition, a serum viral assay revealed the presence of Epstein-Barr computer virus (EBV) Biperiden DNA and EBV-capsid antigen (CA)-IgG, but was unfavorable Biperiden for EBV-CA-IgM, EBV-CA-IgA, and EBV-early antigen (EA)-IgG. Therefore, the patient was initially diagnosed with infectious meningoencephalitis and was treated with 300? mg intravenous ganciclovir twice daily and 4.0 ceftriaxone sodium daily. Further autoimmune encephalitis panel screening for antibodies to anti-aquaporin 4 (AQP4), anti-myelin oligodendrocyte glycoprotein (MOG), anti-N-methyl-D-aspartate receptor (NMDAR), anti-LGI1, and anti-GABABR in the patient’s CSF and serum were negative. Moreover, collection blots for anti-Hu, anti-Yo, anti-Ri, anti-amphiphysin, anti-CV2, anti-Ma1, Biperiden and anti-Ma2 were also unfavorable. No anti-GFAP test was performed at that time. Electroencephalography (EEG) findings were mostly normal, and no epileptiform activity was observed. Electromyography screening (EMG) showed that compound muscle mass action potentials (CMAPs) were absent in the tibial and common peroneal nerves of both lower extremities, and bilateral sural sensory nerve conduction velocities (SNCVs) were decreased, indicating severe, predominantly axonal, sensorimotor neuropathy. Acyclovir was continued, and the patient was empirically treated for infectious meningoencephalitis with ceftriaxone and meropenem. However, his neurological condition progressively worsened. A lumbar puncture was repeated on day 14. At that time, the patient experienced a markedly elevated total protein concentration in the CSF of 2772?mg/L, along with a WBC concentration of 195??106cells/L (95% lymphocytes), a glucose level of 4.11?mmol/L, and ADA concentration of 6?U/L. These findings were inconsistent.