Mesh : Humans Male Middle Aged Glial Fibrillary Acidic Protein / immunology blood Sulfoglycosphingolipids / immunology Immunoglobulin G / blood cerebrospinal fluid Autoantibodies / blood Methylprednisolone / therapeutic use Encephalitis / diagnosis immunology Anti-N-Methyl-D-Aspartate Receptor Encephalitis / diagnosis immunology complications Astrocytes / immunology pathology Autoimmune Diseases of the Nervous System / diagnosis immunology

来  源:   DOI:10.1097/MD.0000000000038983   PDF(Pubmed)

Abstract:
BACKGROUND: Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a rare autoimmune disease of the central nervous system that affects the meninges, brain, spinal cord, and optic nerves. GFAP astrocytopathy can coexist with a variety of antibodies, which is known as overlap syndrome. Anti-NMDAR-positive encephalitis overlap syndrome has been reported; however, encephalitis overlap syndrome with both anti-NMDAR and sulfatide-IgG positivity has not been reported.
METHODS: The patient was a 50-year-old male who was drowsy and had chills and weak limbs for 6 months. His symptoms worsened after admission to our hospital with persistent high fever, dysphoria, gibberish, and disturbance of consciousness. Positive cerebrospinal fluid NMDA, GFAP antibodies, and serum sulfatide antibody IgG were positive.
METHODS: Autoimmune GFAP astrocytopathy with anti-NMDAR and sulfatide-IgG-positive encephalitis overlap syndrome.
METHODS: In addition to ventilator support and symptomatic supportive treatment, step-down therapy with methylprednisolone (1000 mg/d, halved every 3 days) and pulse therapy with human immunoglobulin (0.4 g/(kg d) for 5 days) were used.
RESULTS: After 6 days of treatment, the patient condition did not improve, and the family signed up to give up the treatment and left the hospital.
CONCLUSIONS: Patients with autoimmune GFAP astrocytopathy may be positive for anti-NMDAR and sulfatide-IgG, and immunotherapy may be effective in patients with severe conditions.
CONCLUSIONS: Autoimmune GFAP astrocytopathy with nonspecific symptoms is rarely reported and is easy to be missed and misdiagnosed. GFAP astrocytopathy should be considered in patients with fever, headache, disturbance of consciousness, convulsions, and central infections that do not respond to antibacterial and viral agents. Autoimmune encephalopathy-related antibody testing should be performed as soon as possible, early diagnosis should be confirmed, and immunomodulatory therapy should be administered promptly.
摘要:
背景:自身免疫胶质纤维酸性蛋白(GFAP)星形细胞病是一种罕见的中枢神经系统自身免疫性疾病,会影响脑膜,大脑,脊髓,和视神经.GFAP星形细胞病可以与多种抗体共存,这就是所谓的重叠综合症。抗NMDAR阳性脑炎重叠综合征已有报道;然而,尚未报道具有抗NMDAR和硫酸盐IgG阳性的脑炎重叠综合征。
方法:患者是一名50岁的男性,昏昏欲睡,寒战和四肢虚弱6个月。他因持续高烧入院后症状加重,烦躁不安,胡言乱语,和意识障碍。脑脊液NMDA阳性,GFAP抗体,血清硫酸脂抗体IgG阳性。
方法:自身免疫性GFAP星形细胞病合并抗NMDAR和硫酸脂IgG阳性脑炎重叠综合征。
方法:除了呼吸机支持和对症支持治疗,甲基强的松龙降压治疗(1000mg/d,每3天减半),并使用人免疫球蛋白进行脉冲治疗(0.4g/(kgd),持续5天)。
结果:治疗6天后,病人的情况没有好转,一家人报名放弃了治疗,离开了医院。
结论:患有自身免疫性GFAP星形细胞病的患者抗NMDAR和硫酸脂IgG可能呈阳性,和免疫疗法可能是有效的患者与严重的条件。
结论:具有非特异性症状的自身免疫性GFAP星形细胞病很少报道,容易漏诊和误诊。发热患者应考虑GFAP星形细胞病,头痛,意识障碍,抽搐,和对抗菌和病毒药物没有反应的中枢感染。应尽快进行自身免疫性脑病相关抗体检测,应确认早期诊断,免疫调节治疗应及时进行。
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