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

@article{Sechi2018GlialFA,
  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},
  year={2018},
  volume={90},
  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

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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].

TLDR
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

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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].

TLDR
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

TLDR
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

TLDR
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

TLDR
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

TLDR
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

TLDR
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.

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  • J Neurol Neurosurg Psychiatry
  • 2019

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

  • 2017