Autoimmune GFAP astrocytopathy: Prospective evaluation of 90 patients in 1 year

Dubey, Divyanshu; Hinson, Shannon R.; Jolliffe, Evan; Ζεκερίδου, Αναστασία; Flanagan, Eoin P.; Pittock, Sean J.; Basal, Eati; Drubach, Daniel A.; Lachance, Daniel H.; Lennon, Vanda A.; McKeon, Andrew

doi:10.1016/j.jneuroim.2018.04.016

Cited by 154 publications

(208 citation statements)

References 23 publications

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“…) . Other reports also described linear perivascular radial gadolinium enhancement, which is sometimes punctate in appearance; therefore, this finding is characteristic of GFAP‐A and might be an imaging hallmark of this disease . A similar radial enhancement pattern was occasionally observed in the cerebellum .…”

Section: Clinical Characteristicsmentioning

confidence: 61%

“…In our previous study, all seven patients who underwent single‐photon emission computed tomography had decreased cerebral blood flow in the frontal lobes . Positron emission tomography imaging of the brain and spinal cord at the active stage might show hypermetabolism corresponding to areas of abnormality on MRI . In general, electroencephalography shows diffuse slow waves.…”

Section: Clinical Characteristicsmentioning

confidence: 86%

“…This occurrence was similar to that of NMDA‐R encephalitis ( n = 13, 5.8%). GFAP‐A tends to present in patients aged >40 years . In our previous study, among 14 GFAP‐A patients, the median age of onset was 44 years (range 18–66 years), and men and women were affected equally .…”

Section: Clinical Characteristicsmentioning

confidence: 88%

“…In our previous study, among 14 GFAP‐A patients, the median age of onset was 44 years (range 18–66 years), and men and women were affected equally . However, a previously reported 10 child cases whose clinical phenotypes were similar to adult patients …”

Section: Clinical Characteristicsmentioning

confidence: 90%

“…A fatal necrotizing meningoencephalitis, of which GFAP‐IgG is a biomarker, was described in canines before the discovery of human GFAP‐A . This disorder manifests as a distinctive corticosteroid‐responsive meningoencephalitis/meningoencephalomyelitis with lymphocytic pleocytosis, which is often accompanied by hallmark linear perivascular radial gadolinium enhancement in the brain detected by magnetic resonance imaging (MRI) . After the initial reports of 16 patients with GFAP‐A, this disorder has been reported in several countries, including the USA, China, Italy and the UK .…”

Section: Introductionmentioning

confidence: 99%

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Autoimmune glial fibrillary acidic protein astrocytopathy

Kimura

Takekoshi

Yagi

et al. 2019

Clinical & Exp Neuroim

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Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP‐A) was recently reported as a spectrum of autoimmune inflammatory central nervous system disorders characterized by the detection of immunoglobulin G (IgG) against GFAP in cerebrospinal fluid using tissue‐ and cell‐based testing. The most common phenotypes of GFAP‐A are meningoencephalitis with or without myelitis. Patients present with headache and fever followed by consciousness disturbance and meningeal signs. Movement disorders, ataxia, hyperreflexia and blurred vision related to optic disc edema are sometimes observed. Some patients with GFAP‐A have a neoplasm. The most commonly detected neoplasm is ovarian teratoma. Coexisting neural antibodies are also sometimes detected. N‐methyl‐D‐aspartate receptor (NMDA‐R)‐IgG is the most common coexisting antibody, and patients with coexisting N‐methyl‐D‐aspartate receptor‐IgG have a clinical course resembling N‐methyl‐D‐aspartate receptor encephalitis. Lymphocytic pleocytosis lasts for several months and brain linear perivascular radial gadolinium‐enhancement patterns, an imaging hallmark of GFAP‐A, are sometimes observed. A previous neuropathological study reported inflammatory infiltrates including T‐, B‐ and plasma cells surrounding blood vessels. Although the pathophysiological mechanisms of GFAP‐A remain to be elucidated, GFAP‐specific CD8+ T cells are likely effectors of this disorder. GFAP‐A is usually corticosteroid responsive in the acute stage. However, some patients are prone to relapse or even die. In relapsed or refractory patients, the administration of oral prednisolone or a steroid‐sparing agent might be necessary. Because the timing of corticosteroid therapy might affect patient prognosis, cerebrospinal fluid GFAP‐IgG should be examined in patients with meningoencephalitis with or without myelitis who present with characteristic clinical features.

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Section: Clinical Characteristicsmentioning

confidence: 61%

Section: Clinical Characteristicsmentioning

confidence: 86%

Section: Clinical Characteristicsmentioning

confidence: 88%

Section: Clinical Characteristicsmentioning

confidence: 90%

Section: Introductionmentioning

confidence: 99%

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Autoimmune glial fibrillary acidic protein astrocytopathy

Kimura

Takekoshi

Yagi

et al. 2019

Clinical & Exp Neuroim

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Neuronal intermediate filament IgGs in CSF: Autoimmune Axonopathy Biomarkers

McKeon

Shelly

Zivelonghi

et al. 2020

Ann Clin Transl Neurol

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Objectives To describe CSF‐defined neuronal intermediate filament (NIF) autoimmunity. Methods NIF‐IgG CSF‐positive patients (41, 0.03% of 118599 tested, 1996–2019) were included (serum was neither sensitive nor specific). Criteria‐based patient NIF‐IgG staining of brain and myenteric NIFs was detected by indirect immunofluorescence assay (IFA); NIF‐specificity was confirmed by cell‐based assays (CBAs, alpha internexin, neurofilament light [NF‐L]), heavy‐[NF‐H] chain). Results Sixty‐one percent of 41 patients were men, median age, 61 years (range, 21–88). Syndromes were encephalopathy predominant (23), cerebellar ataxia predominant (11), or myeloradiculoneuropathies (7). MRI abnormalities (T2 hyperintensities of brain, spinal cord white matter tracts. and peripheral nerve axons) and neurophysiologic testing (EEG, EMG, evoked potentials) co‐localized with clinical neurological phenotypes (multifocal in 29%). Thirty patients (73%) had ≥ 1 immunological perturbation: cancer (paraneoplastic), 22; systemic infection (parainfectious [including ehrlichosis, 3] or HIV), 7; checkpoint‐inhibitor cancer immunotherapy, 4; other, 5. Cancers were as follows: neuroendocrine‐lineage carcinomas, 12 (small cell, 6; Merkel cell, 5; pancreatic, 1 [11/12 had NF‐L‐IgG detected, versus 8/29 others, P = 0.0005]) and other, 11. Onset was predominantly subacute (92%) and accompanied by inflammatory CSF (75%), and immunotherapy response (77%). In contrast, CSF controls (15684 total) demonstrated NIF‐IgG negativity (100% of test validation controls), and low frequencies of autoimmune diagnoses (20% of consecutively referred clinical specimens) and neuroendocrine‐lineage carcinoma diagnosis (3.1% vs. 30% of NIF cases), P < 0.0001. Median NF‐L protein concentration was higher in 8 NF‐L‐IgG‐positive patients (median, 6718 ng/L) than 16 controls. Interpretation Neurological autoimmunity, defined by CSF‐detected NIF‐IgGs, represents a continuum of treatable axonopathies, sometimes paraneoplastic or parainfectious.

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Rapidly progressive dementia: Extending the spectrum of GFAP‐astrocytopathies?

Friedrich

Hartig

Prüß

et al. 2022

Ann Clin Transl Neurol

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Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is a steroid-responsive meningoencephalomyelitis, sometimes presenting with atypical clinical signs such as movement disorders or psychiatric and autonomic features. Beyond clinical presentation and imaging, diagnosis relies on detection of GFAP-antibodies (AB) in CSF. Using quantitative behavioral, serologic, and immunohistochemical analyses, we characterize two patients longitudinally over 18-24 months who presented with rapidly progressive neurocognitive deterioration in the context of GFAP-AB in CSF and unremarkable cranial MRI studies. Intensified immunotherapy was associated with clinical stabilization. The value of GFAP-AB screening in selected cases of rapidly progressive dementias is discussed. Case Reports: Methods and ResultsBoth patients presented around their seventh decade with abrupt onset and rapidly progressive neurocognitive deficits over 4-6 months accompanied by autonomic and psychiatric abnormalities. Focal neurologic signs (especially visual, motor, or sensory disturbances, movement 410

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Autoimmune GFAP astrocytopathy: Prospective evaluation of 90 patients in 1 year

Cited by 154 publications

References 23 publications

Autoimmune glial fibrillary acidic protein astrocytopathy

Autoimmune glial fibrillary acidic protein astrocytopathy

Neuronal intermediate filament IgGs in CSF: Autoimmune Axonopathy Biomarkers

Rapidly progressive dementia: Extending the spectrum of GFAP‐astrocytopathies?

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Autoimmune GFAP astrocytopathy: Prospective evaluation of 90 patients in 1 year

Cited by 154 publications

References 23 publications

Autoimmune glial fibrillary acidic protein astrocytopathy

Autoimmune glial fibrillary acidic protein astrocytopathy

Neuronal intermediate filament IgGs in CSF: Autoimmune Axonopathy Biomarkers

Rapidly progressive dementia: Extending the spectrum of GFAP‐astrocytopathies?

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Product

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Autoimmune GFAP astrocytopathy: Prospective evaluation of 90 patients in 1 year