Repeated Brain Magnetic Resonance Imaging Provides Clues for the Diagnosis of Autoimmune Glial Fibrillary Acid Protein Astrocytopathy

Natori, Takahiro; Fukao, Toko; Kurita, Takafumi; Hata, Takanori; Kimura, Akio; Shindo, Kazumasa; Shimohata, Takayoshi; Takiyama, Yoshihisa

doi:10.2169/internalmedicine.8964-21

Cited by 4 publications

(2 citation statements)

References 11 publications

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“…This study included five patients who initially presented with neurological symptoms without abnormalities on MRI but later developed radiographic lesions. Previous research have found that initial brain MRI reveals non-specific findings, but the brain MRI in reexamination and follow-up reveals characteristic autoimmune GFAP-A findings (17,18). A case report also suggested that there was some light meningeal enhancement at first, followed by the gradual development of multiple intracranial lesions (19).…”

Section: Discussionmentioning

confidence: 96%

Clinical, imaging features and outcomes of patients with anti-GFAP antibodies: a retrospective study

et al. 2023

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ObjectiveTo evaluate and compare the clinical features, imaging, overlapping antibodies, and prognosis of pediatric and adult patients with anti-GFAP antibodies.MethodsThis study included 59 patients with anti-GFAP antibodies (28 females and 31 males) who were admitted between December 2019 and September 2022.ResultsOut of 59 patients, 18 were children (under 18 years old), and 31 were adults. The overall cohort’s median age at onset was 32 years old, 7 for children, and 42 for adults. There were 23 (41.1%) patients with prodromic infection, 1 (1.7%) patient with a tumor, 29 (53.7%) patients with other non-neurological autoimmune diseases, and 17 (22.8%) patients with hyponatremia. Fourteen (23.7%) patients had multiple neural autoantibodies, with the AQP4 antibody being the most common. Encephalitis (30.5%) was the most common phenotypic syndrome. Common clinical symptoms included fever (59.3%), headache (47.5%), nausea and vomiting (35.6%), limb weakness (35.6%), and disturbance of consciousness (33.9%). Brain MRI lesions were primarily located in the cortex/subcortex (37.3%), brainstem (27.1%), thalamus (23.7%), and basal ganglia (22.0%). Spinal cord MRI lesions often involved the cervical and thoracic spinal cord. There was no statistically significant difference in the MRI lesion site between children and adults. Out of 58 patients, 47 (81.0%) had a monophasic course, and 4 died. The last follow-up showed that 41/58 (80.7%) patients had an improved functional outcome (mRS <3), and children were more likely than adults to have no residual disability symptoms (p = 0.001).ConclusionThere was no statistically significant difference in clinical symptoms and imaging findings between children and adult patients with anti-GFAP antibodies; Patients with anti-GFAP antibodies may present with normal MRI findings or delayed MRI abnormalities, and patients with overlapping antibodies were common. Most patients had monophasic courses, and those with overlapping antibodies were more likely to relapse. Children were more likely than adults to have no disability. Finally, we hypothesize that the presence of anti-GFAP antibodies is a non-specific witness of inflammation.

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Section: Discussionmentioning

confidence: 96%

Clinical, imaging features and outcomes of patients with anti-GFAP antibodies: a retrospective study

et al. 2023

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“…[ 8 ] Initial brain MRI reveals nonspecific findings, but careful re-examination and follow-up reveal characteristic autoimmune GFAP astrocytopathy findings, as described in a few studies. [ 12 13 ] Other MRI findings include symmetric, small T2/FLAIR lesions (<2 cm) in the basal ganglia (70.6%), thalamus (64.7%), cerebral white matter (29.4%), and leptomeningeal enhancement[ 2 ] (46.2% in the brain and 62.5% in the spinal cord). [ 8 ] No significant differences in symptoms and imaging were observed in pediatric and adult patients[ 2 11 ]; however, few reports describe diffuse abnormalities in the periventricular white matter commonly in adults, while basal ganglia and thalamus enhancement is more common in pediatric populations.…”

Section: Discussionmentioning

confidence: 99%

Unveiling Distinctive MRI Characteristics in the Diagnosis of GFAP Astrocytopathy: A Rare Autoimmune Neuroinflammatory Disorder

Charan,

Priya,

Goel

et al. 2024

Annals of Indian Academy of Neurology

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Glial fibrillary acidic protein (GFAP) astrocytopathy is a rare autoimmune inflammatory disorder affecting the central nervous system, involving the meninges, brain parenchyma, and spinal cord. The distinctive radiologic feature observed on magnetic resonance imaging (MRI) is characterized by periventricular radial and linear contrast enhancement. This case report details a 45-year-old male who initially exhibited constitutional symptoms, followed by encephalitis, lower limb weakness, and urinary retention. The MRI findings revealed meningoencephalitis with longitudinal extensive myelitis. Notably, the cerebrospinal fluid analysis confirmed the presence of anti-GFAP antibodies.

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Autoimmune Glial Fibrillary Acid Protein Astrocytopathy with Hyper-Long Segmental Spinal Cord and Meningeal Enhancement: A Case Report

金¹

2022

ACM

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Repeated Brain Magnetic Resonance Imaging Provides Clues for the Diagnosis of Autoimmune Glial Fibrillary Acid Protein Astrocytopathy

Cited by 4 publications

References 11 publications

Clinical, imaging features and outcomes of patients with anti-GFAP antibodies: a retrospective study

Clinical, imaging features and outcomes of patients with anti-GFAP antibodies: a retrospective study

Unveiling Distinctive MRI Characteristics in the Diagnosis of GFAP Astrocytopathy: A Rare Autoimmune Neuroinflammatory Disorder

Autoimmune Glial Fibrillary Acid Protein Astrocytopathy with Hyper-Long Segmental Spinal Cord and Meningeal Enhancement: A Case Report

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