Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is an autoimmune neurological disorder associated with the presence of anti-GFAP IgG. Meningoencephalitis is the predominant clinical presentation of autoimmune GFAP astrocytopathy in published case series. We report a case of autoimmune GFAP astrocytopathy with the unusual feature of radiculoneuritis in addition to encephalomyelitis, resulting in flaccid paralysis unresponsive to immunotherapy. Imaging data confirmed involvement of brain, spinal cord and nerve roots. Electrodiagnostic testing showed changes consistent with a severe sensorimotor neuropathy with active denervation. The results of this case suggest the need for future studies to assess the impact of peripheral nerve involvement on the outcome of autoimmune GFAP astrocytopathy.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1016/j.msard.2019.101924 | DOI Listing |
JAMA Neurol
January 2025
Department of Neurology, Xuanwu Hospital Capital Medical University, National Center for Neurological Disorders, Beijing, China.
Importance: Autoantibodies targeting astrocytes, such as those against glial fibrillary acidic protein (GFAP) or aquaporin protein 4, are crucial diagnostic markers for autoimmune astrocytopathy among central nervous system (CNS) autoimmune disorders. However, diagnosis remains challenging for patients lacking specific autoantibodies.
Objective: To characterize a syndrome of unknown meningoencephalomyelitis associated with an astrocytic autoantibody.
Neurol Sci
January 2025
Department of Infectious Diseases, Shenzhen Nanshan People's Hospital, Shenzhen University Medical School, Shenzhen, 518052, China.
Background: Glial fibrillary acidic protein (GFAP) astrocytosis is a rare autoimmune encephalitis discovered in the last decade. The diagnosis depends on clinical symptoms, imaging, and antibody testing. However, most cases require several months or even longer to make a definite diagnosis.
View Article and Find Full Text PDFJ Neurol Sci
December 2024
Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.
Mol Neurobiol
December 2024
Department of Neurology, Nanfang Hospital, Southern Medical University, Guangzhou, China.
Virus encephalitis (VE), recognized as one of the common kinds of central nervous system (CNS) diseases after virus infection, has a surprising correlation with autoimmune encephalitis (AE) when autoimmune antibodies emerge in cerebrospinal fluid (CSF) or serum. Herpes simplex virus and Epstein-Barr virus are the most critical agents worldwide. By molecular mimicry, herpes viruses can invade the brain directly or indirectly.
View Article and Find Full Text PDFFront Immunol
December 2024
Department of Radiology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, China.
Objective: Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is a novel steroid sensitive autoimmune disease, without a diagnostic consensus. The purpose of this study was to improve early GFAP-A diagnosis by increasing awareness of key clinical characteristics and imaging manifestations.
Methods: Medical records of 13 patients with anti-GFAP antibodies in serum or cerebrospinal fluid (CSF) were reviewed for cross-sectional and longitudinal analysis of clinical and magnetic resonance imaging (MRI) findings.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!