关键词: glial fibrillary acidic protein astrocytopathy (GFAP) peripheral neuropathy polyneuropathy

Mesh : Humans Male Adult Glial Fibrillary Acidic Protein Peripheral Nervous System Diseases / etiology Astrocytes / pathology Autoantibodies / cerebrospinal fluid Methylprednisolone / administration & dosage Magnetic Resonance Imaging Biomarkers / cerebrospinal fluid Polyneuropathies / etiology Myelitis / etiology diagnosis

来  源:   DOI:10.5692/clinicalneurol.cn-001940

Abstract:
A 44-year-old man was admitted due to a fever. He developed unconsciousness and respiratory failure, necessitating mechanical ventilation. After the administration of methylprednisolone and intravenous immunoglobulin for suspected autoimmune encephalitis, his consciousness and respiratory state improved. However, he exhibited pronounced tetraparalysis and impaired sensation below the neck. A spinal MRI revealed swelling of the entire spinal cord, indicating myelitis. Deep tendon reflexes were diminished in all extremities, and a nerve conduction study confirmed motor-dominant axonal polyneuropathy. Subsequently, he developed a fever and headache. Brain MRI demonstrated FLAIR hyperintensities in the basal ganglia and brain stem. CSF analysis for anti-glial fibrillary acidic protein (GFAP) antibody turned out positive, leading to the diagnosis of GFAP astrocytopathy. Although the steroid re-administration improved muscle strength in his upper limbs and reduced the range of diminished sensation, severe hemiparalysis remained. Severe GFAP astrocytopathy can be involved with polyneuropathy. Early detection and therapeutic intervention for this condition may lead to a better prognosis.
摘要:
一名44岁男子因发烧入院。他出现了昏迷和呼吸衰竭,需要机械通风。甲泼尼龙和静脉注射免疫球蛋白治疗疑似自身免疫性脑炎后,他的意识和呼吸状态有所改善。然而,他表现出明显的四透析和颈部以下的感觉受损。脊柱MRI显示整个脊髓肿胀,提示脊髓炎.所有四肢的深肌腱反射减弱,一项神经传导研究证实了运动性轴突多发性神经病。随后,他发烧和头痛。脑MRI显示基底神经节和脑干的FLAIR高信号。CSF分析抗胶质纤维酸性蛋白(GFAP)抗体呈阳性,导致GFAP星形细胞病的诊断。尽管类固醇的再给药改善了他上肢的肌肉力量并减少了感觉减弱的范围,严重的偏瘫仍然存在。严重的GFAP星形细胞病可能与多发性神经病有关。对这种情况的早期发现和治疗干预可能导致更好的预后。
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