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ArticleNeurology(R) neuroimmunology & neuroinflammation2026

Bilateral Orbital Fat Hyperintensity in Early Glial Fibrillary Acidic Protein Astrocytopathy: A Case Report.

Yusuke Ito, Ken Ohyama, Akio Kimura, Kei Yamashita, Mikiya Nakayabu, Yuki Fukami, Takayoshi Shimohata, Masahisa Katsuno

Abstract readCase Reports
In one paragraph

Article in Neurology(R) neuroimmunology & neuroinflammation, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

8 authors.

Yusuke ItoDepartment of Neurology, Okazaki City Hospital, Japan.ORCID 0009-0004-8624-175X
Ken OhyamaDepartment of Neurology, Okazaki City Hospital, Japan.
Akio KimuraDepartment of Neurology, Gifu University Graduate School of Medicine, Japan.ORCID 0000-0003-0383-2008
Kei YamashitaDepartment of Neurology, Okazaki City Hospital, Japan.
Mikiya NakayabuDepartment of Neurology, Okazaki City Hospital, Japan.ORCID 0009-0006-1026-9267
Yuki FukamiDepartment of Neurology, Nagoya University Graduate School of Medicine, Japan; and.ORCID 0000-0003-1117-5925
Takayoshi ShimohataDepartment of Neurology, Gifu University Graduate School of Medicine, Japan.ORCID 0000-0002-8788-9089
Masahisa KatsunoDepartment of Neurology, Nagoya University Graduate School of Medicine, Japan; and.ORCID 0000-0001-9453-9311

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectivesGlial fibrillary acidic protein (GFAP) astrocytopathy is an inflammatory autoimmune disease of the CNS that targets astrocytes. Although optic disc edema is a frequently reported ocular manifestation in GFAP astrocytopathy, it has not been well-characterized as orbital fat hyperintensity. Here, we report a rare case in which GFAP astrocytopathy initially presented with orbital fat hyperintensity.

methodsA 26-year-old woman presenting with headache, fever, and bilateral ocular pain was evaluated using neurologic examination, CSF examination, autoantibody testing, and multimodal MRI including orbital conditions.

resultsOrbital MRI revealed hyperintensity in the bilateral orbital fat tissue on short tau inversion recovery sequences without optic nerve involvement, whereas intracranial MRI was initially unremarkable. CSF examination showed increase in lymphocytes and was positive for anti-GFAP antibodies. Subsequent MRI revealed characteristic lesions in the thalamic and perivascular area. High-dose corticosteroid therapy led to rapid clinical improvement and resolution of orbital and intracranial MRI lesions. DISCUSSION: This case demonstrates that orbital fat hyperintensity may be an early manifestation of GFAP astrocytopathy, preceding intracranial lesions. Recognition of this orbital MRI finding facilitates earlier diagnosis and treatment, potentially leading to improved prognosis.

Indexed as

Adipose TissueAstrocytesAutoimmune DiseasesAutoimmune Diseases of the Nervous SystemGlial Fibrillary Acidic ProteinOrbitAdultFemaleHumansMagnetic Resonance ImagingGFAP protein, humanGlial Fibrillary Acidic Protein

Identifiers

PMID42636414
PMCPMC13504191

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