Glial fibrillary acidic protein IgG related myelitis: characterisation and comparison with aquaporin-4-IgG myelitis

  title={Glial fibrillary acidic protein IgG related myelitis: characterisation and comparison with aquaporin-4-IgG myelitis},
  author={Elia Sechi and P.P. Morris and Andrew McKeon and Sean J. Pittock and Shannon R. Hinson and Brian G. Weinshenker and Allen Jr. Aksamit and Karl N. Krecke and Timothy J. Kaufmann and Evan A. Jolliffe and Nicholas L. Zalewski and Anastasia Zekeridou and Dean M. Wingerchuk and Jiraporn Jitprapaikulsan and Eoin P. Flanagan},
  journal={Journal of Neurology, Neurosurgery, and Psychiatry},
  pages={488 - 490}
IgG directed against the α-isoform of glial fibrillary acidic protein (GFAP-IgG) predicts a distinct corticosteroid-responsive meningoencephalomyelitis termed autoimmune GFAP astrocytopathy, when detected on cerebrospinal fluid (CSF).1 Optic disc oedema and tremor are common accompaniments. The MRI hallmark is a striking linear perivascular enhancement radially oriented around the ventricles (radial enhancement), while the myelitis component is generally associated with a longitudinally… 

Autoimmune glial fibrillary acidic protein astrocytopathy with lesions distributed predominantly in the entire spinal cord

The case of a 21-year-old girl presenting with dysuria and weariness, who subsequently developed blurry vision, slight dysphagia, slurred speech, and sensory abnormality is reported, and GFAP-IgG was detected in her cerebrospinal fluid.

[A case of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy].

Brain MRI demonstrated multiple linear increased FLAIR signals in bilateral basal ganglia and corona radiata, and Repeated MRI showed T2 hyperintensity areas in the lower brainstem, sparing the area postrema.

Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Review of the Literature

GFAP autoantibody, especially IgG that binds to GFAPα, has been reported in the cerebrospinal fluid (CSF) and serum of patients with GFAP astrocytopathy.

[Recurrent autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy with long cervical cord lesions: a case report].

A 61-year-old man who had suffered two episodes of generalized convulsion in a two-year period was admitted to the authors' hospital because of progressive gait disturbance during the previous five months, and the present case showed a chronic refractory course.

Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Associated With Area Postrema Syndrome: A Case Report

A female patient admitted to the hospital with intractable nausea and vomiting is presented with autoimmune GFAP astrocytopathy, and the symptoms improved rapidly after treatment with corticosteroids, and no recurrence has been observed thus far.

Protein A Immunoadsorption Relieves Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy after Unsuccessful Methylprednisolone Treatment

A case of a patient with GFAP-A with initial symptoms of psychological and cognitive impairment, which did not respond to high-dose methylprednisolone therapy but was successfully treated with protein A immunoadsorption (PAIA) therapy, suggests PAIA might be an effective treatment for patients with GFap-A who respond poorly to conventional IVMP and IVIG therapy.

Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy Presenting with Slowly Progressive Myelitis and Longitudinally Extensive Spinal Cord Lesions

A 65-year-old man with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) who presented with gait disturbance that he had experienced for approximately half a year with treatment with corticosteroids and intravenous immunoglobulin resulted in a clinical improvement.

Astrocytic damage in glial fibrillary acidic protein astrocytopathy during initial attack.

Relapsing MRI-negative myelitis associated with myelin-oligodendrocyte glycoprotein autoantibodies: a case report

MOGAD is one of the conditions which should be considered in MRI-negative myelitis, and the diagnosis may prove difficult, especially if the patient is not exhibiting other neurological symptoms of MOGAD.

A treatable case of autoimmune GFAP astrocytopathy presenting chronic progressive cognitive impairment

A rare case of GFAP-A presenting progressive chronic cognitive impairment with comfortable treatment of a 68-year-old right-handed Japanese woman admitted to the hospital for examination and treatment of progressive cognitive impairment is reported.



Clinical and immunological characteristics of the spectrum of GFAP autoimmunity: a case series of 22 patients

GFAP autoimmunity is not rare and clinical spectrum encompasses meningoencephalitis, myelitis, movement disorders, epilepsy and cerebellar ataxia.

Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: Analysis of 102 patients

A novel autoimmune central nervous system (CNS) disorder with glial fibrillary acidic protein (GFAP)‐IgG as biomarker was recently characterized. Here, 102 patients with GFAP‐IgG positivity are

Autoimmune glial fibrillary acidic protein astrocytopathy in Chinese patients: a retrospective study

The aim was to describe the clinical, radiological and pathological features of an autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy.

Discriminating long myelitis of neuromyelitis optica from sarcoidosis

To compare longitudinally extensive myelitis in neuromyelitis optica spectrum disorders (NMOSD) and spinal cord sarcoidosis (SCS) with real world examples, a large number of patients with NMOSD and SCS are treated with chemotherapy.

Autoimmune encephalitis epidemiology and a comparison to infectious encephalitis

To evaluate the incidence and prevalence of autoimmune encephalitis and compare it to that of infectiousEncephalitis, a large number of patients with known or suspected cases of the disease are referred to a single hospital for evaluation.

re-use. see rights and permissions. Published by BMJ. to cite sechi e

  • J Neurol Neurosurg Psychiatry
  • 2019

GFAP‐IgG as Biomarker of Autoimmune Astrocytopathy: Analysis of 102 Patients

  • 2017