Autoimmune Glial Fibrillary Acidic Protein (Gfap) Astrocytopa-Thy Accompanied with Reversible Splenial Lesion Syndrome (RESLES): A Case Report and Literature Review

Wang, Siting; Yuan, Jianlan; Liu, Jingli

doi:10.3390/brainsci13040659

Cited by 5 publications

(3 citation statements)

References 20 publications

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“…In our case, early MRI findings revealed inflammatory changes in the meninges and splenium of corpus callosum. There have been a few reports of cases of GFAP brain disease with Reversible Splenium of corpus callosum lesion (RESLES) [ [8] , [9] ]. RESLES typically presents as mild encephalitis/encephalopathy, associated with squamous cell carcinoma [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

“…There have been a few reports of cases of GFAP brain disease with Reversible Splenium of corpus callosum lesion (RESLES) [ [8] , [9] ]. RESLES typically presents as mild encephalitis/encephalopathy, associated with squamous cell carcinoma [ 8 ]. The mechanism of GFAP astrocytopathy associated with RESLES remains unclear but may be related to secondary changes following viral infection.…”

Section: Discussionmentioning

confidence: 99%

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Organ donation after brain death from autoimmune glial fibrillary acidic protein astrocytopathy: A case report

Li,

Shen,

Chen

et al. 2024

Heliyon

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Organ donation after brain death from autoimmune glial fibrillary acidic protein astrocytopathy: A case report

Li,

Shen,

Chen

et al. 2024

Heliyon

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“…The characteristic MRI features of GFAP-A are linear radially enhancing lesions perpendicular to the lateral ventricles, meningeal enhancement, and early brainstem and spinal cord involvement ( 2 ). Additionally, a small proportion of patients develop reversible lesions in the splenium of the corpus callosum ( 3 , 4 ). The detection of glial fibrillary acidic protein (GFAP)- IgG in the cerebrospinal fluid (CSF) by both tissue- and cell-based testing serves as a biomarker for GFAP-A ( 5 ).…”

Section: Introductionmentioning

confidence: 99%

Case report: Excessive daytime sleepiness as a presenting manifestation of autoimmune glial fibrillary acidic protein astrocytopathy

Tang,

Huang,

Guo

et al. 2023

Front. Immunol.

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Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is a recently discovered autoimmune inflammatory disease of the central nervous system. It presents with a variety of clinical symptoms, including fever, seizures, psychiatric symptoms, limber weakness, and sensory symptoms. However, the symptoms of sleep disorders have not been sufficiently addressed. Here, we report a case of GFAP-A in which the patient complained of excessive daytime sleepiness and an excessive need for sleep. Our patient was a 58-year-old male who experienced excessive daytime sleepiness for 50 days following SARS-CoV-2 infection. He was diagnosed with coronavirus disease 2019 on June 1st. On the 7th of June, he experienced excessive daytime sleepiness, nausea, reduced food intake, lower limb weakness, and dysuria. Subsequently, his sleepiness significantly deteriorated on July 21st. Five months prior, the patient underwent laparoscopic partial right nephrectomy for clear-cell renal cell carcinoma. Brain MRI revealed abnormal hyperintense lesions in the pontine brain and around the mesencephalic aqueduct on T2 and T2-fluid attenuated inversion recovery (T2-FLAIR) sequences However, these lesions did not exhibit any pathological enhancement. Spinal cord MRI revealed lesions in the C6–C7 and T2–T3 segments on the T2 sequence. His Epworth Sleepiness Scale (ESS) score was 16 (reference range, <10), and 24-hour polysomnography supported the diagnosis of rapid-eye-movement sleep disorder and severe sleep apnea-hypopnea syndrome. Glial fibrillary acidic protein IgG antibodies were detected in the cerebrospinal fluid (1:32, cell-based assay) but not in the serum. The level of hypocretin in the cerebrospinal fluid was 29.92 pg/mL (reference range ≥110 pg/mL), suggesting narcolepsy type 1. After treatment with corticosteroids for approximately 1 month, the patient showed considerable clinical and radiological improvement, as well as an increase in hypocretin levels. Although repeated polysomnography and multiple sleep latency tests suggested narcolepsy, his ESS score decreased to 8. Our findings broaden the range of clinical manifestations associated with GFAP-A, thereby enhancing diagnostic and therapeutic strategies for this disease. Additionally, our results indicate a potential common autoimmune mechanism involving GFAP-A and orexin system dysregulation, warranting further investigation.

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Clinical and neuroimaging phenotypes of autoimmune glial fibrillary acidic protein astrocytopathy: A systematic review and meta‐analysis

Hagbohm,

Ouellette,

Flanagan

et al. 2024

Euro J of Neurology

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ObjectiveThis study was undertaken to provide a comprehensive review of neuroimaging characteristics and corresponding clinical phenotypes of autoimmune glial fibrillary acidic protein astrocytopathy (GFAP‐A), a rare but severe neuroinflammatory disorder, to facilitate early diagnosis and appropriate treatment.MethodsA PRISMA (Preferred Reporting Items for Systematic Reviews and Meta‐Analysis)‐conforming systematic review and meta‐analysis was performed on all available data from January 2016 to June 2023. Clinical and neuroimaging phenotypes were extracted for both adult and paediatric forms.ResultsA total of 93 studies with 681 cases (55% males; median age = 46, range = 1–103 years) were included. Of these, 13 studies with a total of 535 cases were eligible for the meta‐analysis. Clinically, GFAP‐A was often preceded by a viral prodromal state (45% of cases) and manifested as meningitis, encephalitis, and/or myelitis. The most common symptoms were headache, fever, and movement disturbances. Coexisting autoantibodies (45%) and neoplasms (18%) were relatively frequent. Corticosteroid treatment resulted in partial/complete remission in a majority of cases (83%). Neuroimaging often revealed T2/fluid‐attenuated inversion recovery (FLAIR) hyperintensities (74%) as well as perivascular (45%) and/or leptomeningeal (30%) enhancement. Spinal cord abnormalities were also frequent (49%), most commonly manifesting as longitudinally extensive myelitis. There were 88 paediatric cases; they had less prominent neuroimaging findings with lower frequencies of both T2/FLAIR hyperintensities (38%) and contrast enhancement (19%).ConclusionsThis systematic review and meta‐analysis provide high‐level evidence for clinical and imaging phenotypes of GFAP‐A, which will benefit the identification and clinical workup of suspected cases. Differential diagnostic cues to distinguish GFAP‐A from common clinical and imaging mimics are provided as well as suitable magnetic resonance imaging protocol recommendations.

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Autoimmune Glial Fibrillary Acidic Protein (Gfap) Astrocytopa-Thy Accompanied with Reversible Splenial Lesion Syndrome (RESLES): A Case Report and Literature Review

Cited by 5 publications

References 20 publications

Organ donation after brain death from autoimmune glial fibrillary acidic protein astrocytopathy: A case report

Organ donation after brain death from autoimmune glial fibrillary acidic protein astrocytopathy: A case report

Case report: Excessive daytime sleepiness as a presenting manifestation of autoimmune glial fibrillary acidic protein astrocytopathy

Clinical and neuroimaging phenotypes of autoimmune glial fibrillary acidic protein astrocytopathy: A systematic review and meta‐analysis

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