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

Abstract

Background Eosinophilic meningitis is uncommon and often attributed to infectious causes. Case presentation We describe a case of a 72-year-old man who presented with subacute onset eosinophilic meningitis, vasculitis, and intracranial hypertension with progressive and severe neurologic symptoms. Brain MRI demonstrated multifocal strokes and co-localized right temporo-parieto-occipital vasogenic edema, cortical superficial siderosis, and diffuse leptomeningeal enhancement. He ultimately underwent brain biopsy with immunohistochemical stains for amyloid-β and Congo red that were extensively positive in the blood vessel walls and in numerous diffuse and neuritic parenchymal confirming a diagnosis of amyloid-β related angiitis. He was treated with immunosuppression with clinical stabilization. Conclusions Amyloid-β related angiitis is an underrecognized cause of eosinophilic meningitis that can present fulminantly and is typically responsive to immunosuppression. The presence of eosinophils may provide additional clues to the underlying pathophysiology of amyloid-β related angiitis.

Primary Angiitis of the Central Nervous System with Pathological Findings of Eosinophilic Granulomatosis with Polyangiitis

“…In the present case, findings consistent with EGPA were found only on a brain biopsy, and EGPA was not diagnosed. Amyloid-β-related angiitis (ABRA) can cause eosinophilic vasculitis that is localized in the central nervous system (10). However, a brain biopsy in our case showed no Eosinophilic vasculitis confined to the CNS is extremely rare.…”

Investigation of Age-Related Alterations in Brain and Serum Samples in a Healthy Aging Rat Model

“…The Congo red stained semi-thin brain sections showed the aggregates of amyloid plaques as brick-red cloudy materials in brain tissues (Figure 1C) (Setti,2021;Addi,2022) also accumulation in arterial vessels. This accumulation forms cerebral amyloid angiopathy due to impaired clearence of Ab peptides mostly through perivascular drainage pathways with age (Figure 1D) in older rats (Tanner, 2022).…”

“…It is abundantly evident that Abs trigger cholinergic dysfunction in several ways: affecting a-7 nicotinic acetylcholine receptors, affecting nerve growth factor signaling, and also interacting with the peripheral an- (Addi, 2002;Tanner, 2022). * p< 0.05; GRPI vs GRP II and GRP III ionic site of acetylcholinesterase (Sultzer, 2022).…”

“…Scale bars, 200 mM. Black single arrows indicate aggregated Ab plaques and double arrows indicate the Ab deposition in vessel walls(Addi, 2002;Tanner, 2022).…”