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Amyloid-β related angiitis presenting as eosinophilic meningitis: a case report

Jeremy A Tanner1, Megan B Richie1, Cathryn R Cadwell2

  • 1Department of Neurology, University of California, San Francisco (UCSF), 505 Parnassus Avenue, M798 Box 0114, San Francisco, CA, 94143, USA.

BMC Neurology
|March 25, 2022
PubMed
Abstract

Insights

Amyloid-β related angiitis can cause eosinophilic meningitis, presenting with severe neurological symptoms. Prompt immunosuppression can lead to clinical stabilization in affected patients.

Area of Science:

  • Neurology
  • Immunology
  • Pathology

Background:

  • Eosinophilic meningitis is rare and typically linked to infections.
  • Amyloid-β related angiitis (ABRA) is an underrecognized cause of neurological disorders.

Observation:

  • A 72-year-old man presented with subacute eosinophilic meningitis, vasculitis, and intracranial hypertension.
  • Brain MRI revealed multifocal strokes, vasogenic edema, superficial siderosis, and leptomeningeal enhancement.
  • Brain biopsy confirmed ABRA with extensive amyloid-β and Congo red positivity in vessel walls and parenchyma.

Findings:

  • ABRA can manifest as eosinophilic meningitis with severe neurological deficits.
  • Immunohistochemical analysis is crucial for diagnosing ABRA.
  • Eosinophils may indicate specific pathophysiological mechanisms in ABRA.

Implications:

  • ABRA should be considered in cases of eosinophilic meningitis, even with fulminant presentation.
  • Immunosuppressive therapy is a key treatment modality for ABRA.
  • Understanding the role of eosinophils may offer insights into ABRA pathogenesis.
Keywords:
Amyloid-β related angiitisCerebral amyloid angiopathy-related inflammationEosinophilic meningitisPrimary angiitis of the central nervous system

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