A case report of autoimmune GFAP astrocytopathy presenting with abnormal heart rate variability and blood pressure variability

BMC Neurology

Case Report

Guang-qiang Wang¹  Lian-wei Dou²  Li Gong²  Qi Chen²  Xian-wen Chen³  Pu-yuan Wen⁴ 

[1] Department of Cardiology, Affiliated Yantai Yuhuangding Hospital of Qingdao University, Yantai, Shandong, China;Department of Neurology, Affiliated Yantai Yuhuangding Hospital of Qingdao University, Yantai, Shandong, China;Department of Neurology, The First Affiliated Hospital of Anhui Medical University, Hefei, China;Department of Neurology, The First Affiliated Hospital of Anhui Medical University, Hefei, China;Department of Neurology, Affiliated Yantai Yuhuangding Hospital of Qingdao University, Yantai, Shandong, China;

关键词: Astroglial autoimmunity;    GFAP;    Autonomic dysfunction;    Heart rate variability;    Blood pressure variability;    Circadian rhythm;    Case report;   

DOI  :  10.1186/s12883-023-03070-4

 received in 2022-05-03, accepted in 2023-01-10,  发布年份 2023

来源: Springer

【 摘 要 】

BackgroundAutonomic dysfunctions including bladder dysfunction, gastrointestinal dysfunction and orthostasis are common symptoms of autoimmune glial fibrillary acidic protein astrocytopathy (A-GFAP-A); however, cardiac autonomic dysfunction and abnormal circadian rhythm of blood pressure, which can lead to poor prognosis and even sudden cardiac death, has never been reported in A-GFAP-A patient.Case presentationA 68-year-old male Chinese patient presented to our hospital with headache, fever, progressive disturbance of consciousness, dysuria, and limb weakness. Abnormal heart rate variability and non-dipper circadian rhythm of blood pressure gradually developed during hospitalization, which is rare in A-GFAP-A. He had positive GFAP IgG in cerebrospinal fluid (CSF). Enhanced brian MRI showed uneven enhancement and T2 hyperintense lesions of medulla oblongata; Cervical spine MRI showed T2 hyperintense lesions in medulla oblongata and upper margin of the T2 vertebral body. A contrast-enhanced thoracic spine MRI showed uneven enhancement and T2 hyperintense lesions of T1 to T6 vertebral segments. After treatment with intravenous immunoglobulin and corticosteroids, the patient’s symptoms, including autonomic dysfunction, alleviated dramatically. Finally, his heart rate variability and blood pressure variability became normal.ConclusionsOur case broadens the spectrum of expected symptoms in A-GFAP- A syndromes as it presented with heart rate variability and blood pressure variability.

【 授权许可】

CC BY   
© The Author(s) 2023

【 图 表 】

41116_2022_35_Article_IEq140.gif

41116_2022_35_Article_IEq132.gif