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Postinfectious vasculopathy with evolution to moyamoya syndrome

¹ Department of Neurology, University of Washington School of Medicine, Harborview Medical Center, Seattle, WA, USA
² Department of Radiology, University of Washington School of Medicine, Harborview Medical Center, Seattle, WA, USA
³ Department of Pathology, University of Washington School of Medicine, Harborview Medical Center, Seattle, WA, USA
⁴ Everett Neurological Center, Everett, WA, USA

Correspondence to:
Correspondence to:
Dr K J Becker
Box 359775 Harborview Medical Center, 325 9th Ave, Seattle, WA 98104–249, USA; kjb{at}u.washington.edu

ABSTRACT
Background: Parainfectious vascular events are a known complication of bacterial meningitis, typically occurring within two weeks of disease onset. Delayed vascular complications are rare. We present a case of progressive vasculopathy following bacterial meningitis.

Case description: A 20 year old woman developed progressive vasculopathy after successful treatment of pneumococcal meningitis. Within eight months of her infection, angiography revealed the appearance of moyamoya syndrome. Despite aggressive immunomodulation and anticoagulation, she had multiple strokes. Autopsy confirmed severe narrowing of proximal cerebral vasculature with absence of inflammation or atherosclerosis.

Conclusions: The inflammation and subsequent postinfectious autoimmune response associated with meningitis can lead to a progressive vasculopathy and may represent a pathophysiologic mechanism for the arterial occlusions seen in moyamoya syndrome.

Abbreviations: ß2-GP1, ß2-glycoprotein 1; CSF, cerebrospinal fluid; LP, lumbar puncture; TCD, transcranial Doppler; WBC, white blood cell

Keywords: meningitis; vasculopathy; autoimmune; postinfectious; antiphospholipid antibodies