Plasma exchange for two patients with autoimmune GFAP astrocytopathy with rapid progression to respiratory failure: a case report.
Frontiers in immunology
Background:Patients with autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy can present with early neurological deterioration, but rapidly progressive respiratory failure is rarely reported. We present the cases of two patients with autoimmune GFAP astrocytopathy who experienced rapid progression to respiratory failure and were effectively treated using plasma exchange therapy. Case report:Two patients were diagnosed with autoimmune GFAP astrocytopathy. Their initial symptoms were consistent with those of previously observed cases of autoimmune GFAP astrocytopathy. However, they experienced rapid progression to respiratory failure due to their lesion location. Specifically, case 1 had lesions in the medulla oblongata, and case 2 had lesions in the high cervical spinal cord, which are both common sites of lesions causing respiratory failure. The patients did not respond well to intravenous methylprednisolone and intravenous immunoglobulin initially and could not be withdrawn from ventilator support. Fortunately, subsequent plasma exchange therapy led to significant clinical improvements and successful withdrawal from ventilator support. Discussion:Patients with autoimmune GFAP astrocytopathy can present with rapidly progressive respiratory failure. Early treatment with plasma exchange can be beneficial in withdrawing patients from ventilator support.
10.3389/fimmu.2023.1265609
Autoimmune glial fibrillary acidic protein astrocytopathy: clinical analysis and review of 15 cases.
Acta neurologica Belgica
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.
10.1007/s13760-023-02268-0
Autoimmune glial fibrillary acidic protein astrocytopathy.
Kunchok Amy,Zekeridou Anastasia,McKeon Andrew
Current opinion in neurology
PURPOSE OF REVIEW:To describe a recently characterized autoimmune, inflammatory central nervous system (CNS) disorder known as autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. RECENT FINDINGS:Affected patients present with symptoms of one or more of meningitis (headache and neck ache), encephalitis (delirium, tremor, seizures, or psychiatric symptoms), and myelitis (sensory symptoms and weakness). Optic disc papillitis (blurred vision) is common. CNS inflammation is evident in characteristic T1 postgadolinium enhancement of GFAP-enriched CNS regions, and lymphocytic cerebrospinal fluid (CSF) white cell count elevation. CSF is more reliable than serum for GFAP-immunoglobulin G (IgG) testing. Ovarian teratoma commonly coexists, particularly among patients with accompanying N-methyl-D-aspartate receptor or aquaporin-4 autoimmunity. Parainfectious autoimmunity is suspected in some other patients, though the culprit organism is rarely verified. Pathophysiologic relevance of T cells is underscored by neuropathology and cases of dysregulated T-cell function (HIV or checkpoint inhibitor cancer therapy). Corticosteroid-responsiveness is a hallmark of the disease. Relapses occur in approximately 20% of patients, necessitating transition to a steroid-sparing drug. Reported outcomes vary, though in the authors' experience, early and sustained intervention usually portends recovery. SUMMARY:Autoimmune GFAP astrocytopathy is a treatable autoimmune CNS disease diagnosable by GFAP-IgG testing in CSF. This disease presents opportunities to explore novel mechanisms of CNS autoimmunity and inflammation.
10.1097/WCO.0000000000000676
Autoimmune glial fibrillary acidic protein astrocytosis mimicking tuberculous meningitis: a retrospective study.
Journal of neurology
BACKGROUND:This study aimed to summarize the clinical features of Autoimmune Glial Fibrillary Acidic Protein Astrocytosis mimicking tuberculosis meningitis to improve clinicians' understanding of this disease. METHODS:We retrospectively analyzed the clinical manifestations, cerebrospinal fluid results, and imaging data of five patients with Autoimmune Glial Fibrillary Acidic Protein Astrocytosis mimicking tuberculous meningitis who were admitted to Xiangya Hospital Central South University between October 2021 and July 2022. RESULTS:Five patients were aged 31-59 years, with a male-to-female ratio of 4:1. Among the cases reviewed, four had a history of prodromal infections manifesting as fever and headache. One patient developed limb weakness and numbness with clinical manifestations of meningitis, meningoencephalitis, encephalomyelitis, or meningomyelitis. Cerebrospinal fluid analysis revealed an increased cell count in five cases, with a lymphocyte majority. All five cases had a CSF protein level > 1.0 g/L, CSF/blood glucose ratio < 0.5, and two patients had CSF glucose < 2.2 mmol/L. Decreased CSF chloride was observed in three cases, while increased ADA was observed in one case. Both serum and cerebrospinal fluid were positive for anti-GFAP antibodies in three cases, while in two cases, only CSF was positive for anti-GFAP antibodies. Additionally, hyponatremia and hypochloremia were observed in three cases. No tumors were detected in any of the five patients during tumor screening, and all five cases had a good prognosis following immunotherapy. CONCLUSION:Anti-GFAP antibody testing should be routinely performed in patients with suspected tuberculosis meningitis to avoid misdiagnosis.
10.1007/s00415-023-11818-8
Clinical and electrophysiological characteristics of peripheral neuropathy in autoimmune glial fibrillary acidic protein astrocytopathy: an observational study and literature review.
Therapeutic advances in neurological disorders
Background:The phenotype of peripheral neuropathy (PN) associated with glial fibrillary acidic protein-immunoglobulin G (GFAP-IgG) has not been well described. Objectives:The aim of this study was to report the frequency, clinical, and electrophysiological characteristics of PN in GFAP-IgG-positive patients. Design:This study is a single-center retrospective observational study. Data Sources and methods:GFAP-IgG-positive patients with PN were retrospectively identified from the Huashan Hospital Autoimmune Encephalitis Cohort between 2017 and 2021. Eight patients who presented with PN from other published studies were also included in the analysis. The clinical and electrophysiological characteristics of GFAP-IgG-related PN were described. Results:A total of 21 (31%) patients (7 females, 14 males; : 42 ± 16 years) from a cohort of 68 GFAP-IgG-positive patients presented with PN. Twenty of 21 patients had symmetrical weakness. Sensory and autonomic symptoms were present in 16 and 15 patients, respectively. Lower extremities were the most frequently involved regions for both motor (20/21) and sensory (15/21) symptoms. Moreover, 13 patients (4 females, 9 males; : 43 ± 13 years) had electrodiagnostic study data, and 12 of 13 patients had abnormal findings. Regarding clinical features, motor nerve fibers were predominantly involved (12/13), and symmetric lower extremities (12/13) were the most commonly affected regions. Axonal neuropathy is the typical underlying pathophysiologic process of PN. All 21 patients responded to immunotherapy. However, four patients with tetraplegia had poor outcomes, and PN was the major determinant of their long-term disability. Most cases (6/8) from the literature presented with similar clinical and electrophysiological features to those from our cohort. Conclusion:Peripheral nerves could be involved in autoimmune GFAP astrocytopathy. Predominant motor axonal neuropathy mainly involving the lower extremities is the most common PN phenotype in this disorder. GFAP-IgG-related PN is responsive to immunotherapy. Registration:Chinese Clinical Trial Registry: ChiCTR2000029115 (http://www.chictr.org).
10.1177/17562864231164806
Clinical characteristics and MRI features of autoimmune glial fibrillary acidic protein astrocytopathy: a case series of 34 patients.
Frontiers in neurology
Objectives:To analyze the clinical and imaging characteristics of autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A). Methods:Forty-three patients diagnosed with GFAP-A between March 2017 and July 2023 were retrospectively recruited. The clinical characteristics and magnetic resonance imaging (MRI) features were collected. Results:Twenty-one patients (61.8%) had a fever and 16 (47.1%) had a headache. Five patients (14.7%) had coexisting neural autoantibodies and one patient (2.9%) had a coexisting neoplasm. The most common presentation was meningoencephalomyelitis (13/34, 38.3%), followed by meningoencephalitis (12/34, 35.3%). The other clinical manifestations included blurred visions (5/34, 14.7%) and peripheral nervous system involvement (4/34, 11.8%). Twenty-six patients (76.5%) had elevated nucleated cell count, predominantly lymphocytes (15/15, 100%), and 27 (79.4%) had elevated protein levels of cerebrospinal fluid. One-half (50%) of the patients presented with hyponatremia. A majority of the patients (30/33, 90.9%) exhibited abnormal hyperintense lesions on T2WI, which were often located in juxtacortical white matter (18/33, 54.5%), followed by periventricular white matter (16/33, 48.5%), basal ganglia (15/ 33, 45.5%), brainstem (11/33, 33.3%), and thalamic lesions (9/33, 27.3%). Twenty-four patients (72.7%) had abnormal brain enhancement, with supratentorial leptomeningeal enhancement being the most frequent enhancement pattern (15/33, 45.5%), followed by linear perivascular radial enhancement (14/33, 42.4%). Nineteen patients (70.4%) had hyperintense intramedullary spinal cord lesions, with long segments (15/27, 55.6%) and transverse lesions (14/27, 51.9%) being the most frequent lesions. Most cases were sensitive to immunotherapy, such as glucocorticoids, intravenous immunoglobulin, and tacrolimus, with three patients (8.8%) experiencing relapses. Patients with brainstem lesions had higher onset modified Rankin scale scores and were more prone to intensive care unit admissions. Linear perivascular radial enhancement was positively associated with poor prognosis ( < 0.05). Conclusion:GFAP-A presented with meningoencephalomyelitis and meningoencephalitis. The brain lesions were often located in juxtacortical white matter, periventricular white matter, basal ganglia, brainstem, and thalamus. Long segments and transverse were the most frequent spine lesions. Leptomeningeal enhancement was the most frequent enhancement pattern, followed by linear perivascular radial enhancement, which may provide new insight into the differential diagnosis of GFAP-A.
10.3389/fneur.2024.1375971