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

Kudo, Takuya; Kimura, Akio; Higashida, Kazuhiro; Yamada, Megumi; Hayashi, Yuichi; Shimohata, Takayoshi

doi:10.2169/internalmedicine.5074-20

Cited by 9 publications

(5 citation statements)

References 14 publications

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“…The reason for this is also uncertain. Myelitis in pediatric patients is relative rare compared adult patients [ 10 , 14 , 24 – 27 ]. In our case, the two patients with myelitis showed longitudinally extensive myelitic abnormalities, and a slight enhancement.…”

Section: Discussionmentioning

confidence: 99%

Autoimmune glial fibrillary acidic protein astrocytopathy in children: a retrospective study

Zhuang

Jin

et al. 2022

Eur J Med Res

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Objective To describe the clinical features of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy in children. Method Data from 11 pediatric patients with autoimmune GFAP astrocytopathy were retrospectively analyzed. Results All of the patients showed encephalitis and meningoencephalitis or meningoencephalomyelitis with or without myelitis. 45.4% of the patients had fever, 27.3% headaches, 18.2% dizziness, 18.2% drowsiness, and 18.2% mental disorders. Cerebrospinal fluid (CSF) was detected in all patients. The white blood cell counts (WBC) (90.9%), lactic dehydrogenase levels (72.7%), protein level (36.4%), and adenosine deaminase activity (ADA) level (27.3%) were elevated, and the CSF glucose levels (72.7%) were slightly reduced. Nine patients (90%) were found to have brain abnormalities, of which five (50.0%) patients had abnormal symmetrical laminar patterns or line patterns hyperintensity lesions on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images in the basal ganglia, hypothalamus, subcortical white matter and periventricular white matter. The linear radial enhancement pattern of the cerebral white matter was only seen in two patients, with the most common being abnormal enhancement of leptomeninges (50%). Five patients had longitudinally extensive spinal cord lesions. Conclusion The findings of pediatric patients with autoimmune GFAP astrocytopathy are different from previous reports.

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Section: Discussionmentioning

confidence: 99%

Autoimmune glial fibrillary acidic protein astrocytopathy in children: a retrospective study

Zhuang

Jin

et al. 2022

Eur J Med Res

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“…Like the previous studies, most patients have an acute or subacute onset. Clinical manifestations were encephalitis and meningoencephalitis, including fever ( 45.4%), headaches (27.3%), dizziness (18.2%), drowsiness (18.2%), and mental disorders (18.2%) [3,9,10,13,14,15,16,17] . In our case, all of the patients who suffered from myelitis were showed longitudinally extensive transverse myelitis, like the previous study.…”

Section: Discussionmentioning

confidence: 99%

Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy in Children: A Retrospective Study

Zhuang¹,

Jin²,

Li³

et al. 2021

Preprint

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Objective: To describe the clinical features of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy in children.Method: Data from 11 pediatric patients with autoimmune GFAP astrocytopathywas retrospectively analyzed.Results: All of the patients showed encephalitis and meningoencephalitis or meningoencephalomyelitis with or without myelitis, include fever (45.4%), headaches (27.3%), dizziness (18.2%), drowsiness (18.2%) and mental disorders (18.2%). Cerebrospinal fluid (CSF) were detected in all patients. The white cell counts (WBC) (90.9%), lactic dehydrogenase levels (72.7%), protein level (36.4%), and adenosine deaminase activity (ADA) level (27.3%) were elevated, and the CSF glucose levels (72.7%) were slightly reduced. Nine patients (90%) were found to have brain abnormalities, of which five (50.0%) patients had abnormal symmetrical laminar patterns or line patterns hyperintensity lesions on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images in the basal ganglia, hypothalamus, subcortical white matter and periventricular white matter. The linear radial enhancement pattern of the cerebral white matter was only seen in two patients, the most common being abnormal enhancement of leptomeninges (50%). Five patients had longitudinally extensive spinal cord lesions.Conclusion: The findings of pediatric patients with autoimmune GFAP astrocytopathy is different from the previous reports.

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“…The lesions were predominantly located centrally within the cord, affecting the grey matter, and characterized as hazy, subtle, or diffuse on T2weighted imaging [1,2,7,26,27]. Notably, three case reports described the lesions as bilateral longitudinal with an eccentric location [28][29][30].…”

Section: Spinal Cord Mri Findingsmentioning

confidence: 99%

“…Whereas some authors reported normal findings [25], even in areas with pathology on MRI [39,40], others reported increased or decreased uptake in different regions [27]. A few studies reported high intramedullary metabolism associated with extensive myelitis [29,37,41].…”

Section: Advanced and Nuclear Neuroimaging Findingsmentioning

confidence: 99%

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 Astrocytopathy Presenting with Slowly Progressive Myelitis and Longitudinally Extensive Spinal Cord Lesions

Cited by 9 publications

References 14 publications

Autoimmune glial fibrillary acidic protein astrocytopathy in children: a retrospective study

Autoimmune glial fibrillary acidic protein astrocytopathy in children: a retrospective study

Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy in Children: A Retrospective Study

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

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