Evidence map›Paper›PMID 41791017›Full record

ArticleNeurology(R) neuroimmunology & neuroinflammation2026

Fulminant Amyloid β-Related Angiitis With Herniation and Rapid Response to Tocilizumab: A Case Report.

James V Nguyen, Emma M Byrne, Salina Yuan, Shamik Bhattacharyya, Mariel G Kozberg, Marshall Lukacs, Philippe A Bilodeau

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. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

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Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

7 authors.

James V NguyenDepartment of Neurology, Mass General Brigham, Boston, MA; and.
Emma M ByrneDepartment of Neurology, Mass General Brigham, Boston, MA; and.
Salina YuanDepartment of Neurology, Mass General Brigham, Boston, MA; and.ORCID 0000-0002-7503-7081
Shamik BhattacharyyaDepartment of Neurology, Mass General Brigham, Boston, MA; and.ORCID 0000-0001-8047-3622
Mariel G KozbergDepartment of Neurology, Mass General Brigham, Boston, MA; and.ORCID 0000-0002-9358-3262
Marshall LukacsDepartment of Pathology, Mass General Brigham, Boston, MA.
Philippe A BilodeauDepartment of Neurology, Mass General Brigham, Boston, MA; and.ORCID 0000-0002-2672-554X

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectivesAmyloid β-related angiitis (ABRA) is a rare, inflammatory vasculopathy resulting from intravascular amyloid deposition. We describe a refractory presentation of ABRA and its response to tocilizumab.

methodsSingle-patient, biopsy-confirmed ABRA with serial MRI and clinical outcomes. Treatments included high-dose IV methylprednisolone, hyperosmolar therapy, cyclophosphamide, and escalation to tocilizumab.

resultsA 70-year-old woman presented with seizure and focal deficits. Initial MRI showed multifocal subcortical T2/FLAIR hyperintensities with cortical microhemorrhages without enhancement. Despite steroids, interval imaging showed progressive vasogenic edema, new leptomeningeal enhancement, and rising microhemorrhage burden. She developed severe headache and worsening hemiparesis; CT/MRI demonstrated marked edema with subfalcine/uncal herniation. Biopsy confirmed ABRA. Edema did not improve with steroids, maximal hyperosmolar therapy, or cyclophosphamide. After tocilizumab, MRI within 48 hours showed reduced edema and midline shift with near-resolution of sulcal enhancement; hemiparesis markedly improved without hemicraniectomy. DISCUSSION: We expand the phenotypic spectrum of ABRA by presenting a herniation syndrome associated with fulminant disease and a clear clinicoradiographic natural history. The precipitous progression of microhemorrhage accrual suggests a pathophysiologically distinct mechanism of disease from cerebral amyloid angiopathy-related inflammation. IL-6 receptor blockade was associated with rapid clinical and radiographic improvement and stabilization, supporting prospective evaluation of tocilizumab in amyloid-related neuroinflammatory disorders.

Indexed as

Amyloid beta-PeptidesAntibodies, Monoclonal, HumanizedEncephaloceleVasculitis, Central Nervous SystemAgedFemaleHumansMagnetic Resonance ImagingAmyloid beta-PeptidesAntibodies, Monoclonal, Humanizedtocilizumab

Identifiers

PMID41791017
PMCPMC12971120

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