Area Postrema Syndrome in Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy

Deng, Bo; Wang, Jingguo; Yu, Hai; Jin, Lirong; Qiu, Yue; Liu, Xiaoni; Wang, Pengyu; Zhang, Xiang; Chen, Xiangjun

doi:10.1212/nxi.0000000000200029

Cited by 18 publications

(16 citation statements)

References 30 publications

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“…Four patients had symptoms of speech dysfunction, which had been rarely reported in earlier studies. Two patients developed APS, as previously reported ( 12 ), implying that APS should not only be considered as a diagnosis of neuromyelitis optica spectrum disorders (NMOSD) but should also be tested for anti-GFAP antibodies.…”

Section: Discussionsupporting

confidence: 77%

Clinical, imaging features and outcomes of patients with anti-GFAP antibodies: a retrospective study

et al. 2023

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ObjectiveTo evaluate and compare the clinical features, imaging, overlapping antibodies, and prognosis of pediatric and adult patients with anti-GFAP antibodies.MethodsThis study included 59 patients with anti-GFAP antibodies (28 females and 31 males) who were admitted between December 2019 and September 2022.ResultsOut of 59 patients, 18 were children (under 18 years old), and 31 were adults. The overall cohort’s median age at onset was 32 years old, 7 for children, and 42 for adults. There were 23 (41.1%) patients with prodromic infection, 1 (1.7%) patient with a tumor, 29 (53.7%) patients with other non-neurological autoimmune diseases, and 17 (22.8%) patients with hyponatremia. Fourteen (23.7%) patients had multiple neural autoantibodies, with the AQP4 antibody being the most common. Encephalitis (30.5%) was the most common phenotypic syndrome. Common clinical symptoms included fever (59.3%), headache (47.5%), nausea and vomiting (35.6%), limb weakness (35.6%), and disturbance of consciousness (33.9%). Brain MRI lesions were primarily located in the cortex/subcortex (37.3%), brainstem (27.1%), thalamus (23.7%), and basal ganglia (22.0%). Spinal cord MRI lesions often involved the cervical and thoracic spinal cord. There was no statistically significant difference in the MRI lesion site between children and adults. Out of 58 patients, 47 (81.0%) had a monophasic course, and 4 died. The last follow-up showed that 41/58 (80.7%) patients had an improved functional outcome (mRS <3), and children were more likely than adults to have no residual disability symptoms (p = 0.001).ConclusionThere was no statistically significant difference in clinical symptoms and imaging findings between children and adult patients with anti-GFAP antibodies; Patients with anti-GFAP antibodies may present with normal MRI findings or delayed MRI abnormalities, and patients with overlapping antibodies were common. Most patients had monophasic courses, and those with overlapping antibodies were more likely to relapse. Children were more likely than adults to have no disability. Finally, we hypothesize that the presence of anti-GFAP antibodies is a non-specific witness of inflammation.

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Section: Discussionsupporting

confidence: 77%

Clinical, imaging features and outcomes of patients with anti-GFAP antibodies: a retrospective study

et al. 2023

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“…Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) is an inflammatory autoimmune disorder of the central nervous system that was first described in humans in 2016. Currently, GFAP immunoglobulin G (GFAP-IgG) is regarded as a specific biomarker for the diagnosis of autoimmune GFAP-A [ 1 , 2 ]. However, it is debated whether anti-GFAP antibodies possess pathogenicity.…”

Section: Introductionmentioning

confidence: 99%

Autoimmune glial fibrillary acidic protein astrocytopathy: clinical analysis and review of 15 cases

Wei

et al. 2023

Acta Neurol Belg

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Background To review clinical characteristics, auxiliary examination results, treatment effects, and outcomes of patients with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A). Methods We collated and retrospectively analyzed clinical data of 15 patients admitted with clinical characteristics of an autoimmune GFAP-A acute encephalitis or meningitis phenotype. Results All patients were diagnosed with acute-onset meningoencephalitis and meningoencephalomyelitis. Initial presentations included pyrexia and headache at onset; dual symptoms of prominent tremor with urinary and bowel dysfunction; ataxia, psychiatric and behavioral abnormalities, and impaired consciousness; neck resistance; reduced extremity muscle strength; blurred vision; epileptic seizures; and reduced basic blood pressure. Cerebrospinal fluid (CSF) examination showed that the degree of protein elevation was significantly higher than the degree of increase in white blood cells. Moreover, in the absence of obvious low chloride and glucose levels, CSF chloride levels decreased in 13 patients, accompanied by a CSF glucose level decrease in four. Brain abnormalities were found in magnetic resonance imaging of ten patients, with a linear radial perivascular enhancement present in the lateral ventricles of two patients and symmetric abnormalities in the splenium of the corpus callosum in three patients. Conclusions Autoimmune GFAP-A may be a spectrum disorder, with acute- or subacute-onset meningitis, encephalitis, and myelitis being the main phenotypes. When used for acute stage treatment, combined hormone and immunoglobulin therapy was superior to hormone pulse therapy or immunoglobulin pulse therapy alone. However, hormone pulse therapy alone without immunoglobulin pulse therapy was associated with a greater number of remaining neurological deficits.

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“…Whereas, the incidence of APS has not been well understood. To date, a total of 16 GFAP-IgG-positive patients presented with APS have been described in the literature [ 18 ]. Our case emphasizes that isolated APS can be the initial manifestation of autoimmune GFAP astrocytopathy.…”

Section: Discussionmentioning

confidence: 99%

Area postrema syndrome with linear enhancement along the surface of the brainstem and fourth ventricle in autoimmune GFAP astrocytopathy

Liang

Shen

2023

BMC Neurol

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Background Glial fibrillary acidic protein (GFAP) astrocytopathy, a novel autoimmune disease of the nervous system, was first defined in 2016. To our knowledge, area postrema syndrome (APS) with linear enhancement along the surface of the brainstem and fourth ventricle is extremely rare in this disorder. Case presentation A Chinese woman presented with intractable nausea and vomiting after onset of flu-like symptoms. Brain magnetic resonance imaging (MRI) disclosed abnormal signal intensities in the dorsal medulla oblongata including area postrema. Besides, linear enhancement surrounding the surface of the brainstem and fourth ventricle was visualized after gadolinium injection. Cerebrospinal fluid (CSF) analysis showed increased cell count and protein. A cell-based assay was positive for anti-GFAP IgG in CSF. She was diagnosed with autoimmune GFAP astrocytopathy and treated with high-dose glucocorticoid. The patient received a quick recovery with entire resolution of the initial abnormalities. Conclusions Isolated APS can be the initial manifestation of autoimmune GFAP astrocytopathy. Linear enhancement surrounding the surface of the brainstem and fourth ventricle is another neuroradiological hallmark.

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Area Postrema Syndrome in Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy

Cited by 18 publications

References 30 publications

Clinical, imaging features and outcomes of patients with anti-GFAP antibodies: a retrospective study

Clinical, imaging features and outcomes of patients with anti-GFAP antibodies: a retrospective study

Autoimmune glial fibrillary acidic protein astrocytopathy: clinical analysis and review of 15 cases

Area postrema syndrome with linear enhancement along the surface of the brainstem and fourth ventricle in autoimmune GFAP astrocytopathy

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