Failure of IVIG in steroid-responsive autoimmune glial fibrillary acidic protein astrocytopathy: A case report

Heide, Elena S.; Chaudhari, Amit; Pirverdian, Ateena; Lai, Samuel K.; Courtney, Ardith

doi:10.1016/j.msard.2021.102933

Cited by 6 publications

(3 citation statements)

References 11 publications

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“…Notably, when patients with autoimmune GFAP astrocytopathy have low CSF glucose levels, they are frequently suspected of having infectious encephalitis. A decreased glucose level was reported in the CSF of nine of the 59 patients with autoimmune GFAP astrocytopathy (15.25%) (8,(15)(16)(17)(18)(19). The precise mechanism and clinical importance of hypoglycorrhachia in autoimmune GFAP astrocytopathy remain unknown.…”

Section: Discussionmentioning

confidence: 99%

Case report: Autoimmune glial fibrillary acidic protein astrocytopathy misdiagnosed as tuberculous meningitis

Qin

Wu²,

Wang³

et al. 2023

Front. Neurol.

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IntroductionAutoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a new form of autoimmunity-mediated central nervous system disease. It is especially easy to misdiagnose when clinical symptoms and cerebrospinal fluid (CSF) indicators are similar to those observed in patients with tuberculous meningitis (TBM).MethodsWe retrospectively analyzed five cases of autoimmune GFAP astrocytopathy that were initially misdiagnosed as TBM.ResultsIn the five reported cases, all but one patient had meningoencephalitis in the clinic, and all patients exhibited increased pressure, lymphocytosis, increased protein levels, and decreased glucose levels in their CSF results and did not have typical imaging findings of autoimmune GFAP astrocytopathy. TBM was the initial diagnosis in all five patients. However, we found no direct evidence of tuberculosis infection, and anti-tuberculosis treatment had inconclusive effects. Following a GFAP antibody test, the diagnosis of autoimmune GFAP astrocytopathy was made.ConclusionWhen there is a suspected diagnosis of TBM but TB-related tests are negative, the possibility of autoimmune GFAP astrocytopathy should be considered.

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Section: Discussionmentioning

confidence: 99%

Case report: Autoimmune glial fibrillary acidic protein astrocytopathy misdiagnosed as tuberculous meningitis

Qin

Wu²,

Wang³

et al. 2023

Front. Neurol.

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“…The symptoms of autoimmune GFAP astrocytosis vary depending on the lesion’s location and scope, and there is no apparent specificity. While hormone therapy has been reported to result in better outcomes, the patient in question also demonstrated promising results with immunoglobulin treatment, possibly due to the immune-related pathogenesis of the disease or the presence of different types of the disease that respond differently to hormone therapy ( 16 ).…”

Section: Discussionmentioning

confidence: 99%

Autoimmune GFAP astrocytopathy after viral encephalitis: a case report of bimodal overlapping encephalitis

Cheng,

Huang,

Yang

et al. 2023

Front. Immunol.

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Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a treatable autoimmune disorder affecting the central nervous system. Despite extensive research, the exact etiology and pathogenesis of this condition remain unclear. In recent years, autoimmune encephalitis (AE) after viral encephalitis (VE) has gathered significant attention. Here, we present a case report of autoimmune GFAP astrocytopathy after VE in a 43-year-old Asian male with a history of oral and labial herpes. The patient presented with high-grade fever, headache, urinary retention, unresponsiveness, and apathy. Elevated levels of protein and GFAP-IgG were observed in the cerebrospinal fluid (CSF), and enhanced brain magnetic resonance imaging (MRI) revealed linear enhancement oriented radially to the ventricles. Treatment with intravenous immunoglobulin (IVIG) resulted in symptom relief, reduced lesion enhancement, and decreased protein levels. This case report highlights bimodal encephalitis with no discernible interval between VE and autoimmune GFAP astrocytopathy, which poses diagnostic challenges. Notably, autoimmune GFAP astrocytopathy is a novel form of autoimmune encephalitis, and its treatment lacks sufficient clinical experience. Intriguingly, our patient demonstrated sensitivity to IVIG, a treatment that differed from past reports. Therefore, further exploration of treatment strategies for this condition is warranted.

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“…Intravenous immunoglobulins and plasma exchange may also be required [ 6 ]. Empirical data suggest a superiority of corticosteroids over intravenous immunoglobulins, which is why they are reserved for patients unresponsive to corticotherapy [ 7 ]. Long-term treatment depends on oral low-dose corticosteroids or immunosuppressants.…”

Section: Discussionmentioning

confidence: 99%

Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy Presenting as Mild Encephalopathy with Reversible Splenium Lesion

et al. 2021

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Introduction: Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is scarce and usually presents as meningoencephalomyelitis. Here, we offer the case of an atypical presentation of GFAP-astrocytopathy.Case Presentation: We report the case of a 26-year-old woman admitted to our neurology department for a 3-week progressive and worsening neurologic picture, with secondary worsening. Initial imaging showed a Mild Encephalitis with Reversible Splenium of corpus callosum lesion (MERS). Full infectious and autoimmune workup then revealed positivity of GFAP antibodies, leading us to diagnose GFAP astrocytopathy. Discussion: Our case is the first reported association between MERS and GFAP astrocytopathy in an adult patient. Clinical presentation of GFAP astrocytopathy usually includes various neurologic symptoms and can lead to misdiagnosis.

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Failure of IVIG in steroid-responsive autoimmune glial fibrillary acidic protein astrocytopathy: A case report

Cited by 6 publications

References 11 publications

Case report: Autoimmune glial fibrillary acidic protein astrocytopathy misdiagnosed as tuberculous meningitis

Case report: Autoimmune glial fibrillary acidic protein astrocytopathy misdiagnosed as tuberculous meningitis

Autoimmune GFAP astrocytopathy after viral encephalitis: a case report of bimodal overlapping encephalitis

Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy Presenting as Mild Encephalopathy with Reversible Splenium Lesion

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