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Neurohospitalist. 2014 Apr;4(2):86-9. doi: 10.1177/1941874413502796.

Amyloid β-Related Central Nervous System Angiitis Presenting With an Isolated Seizure.

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Department of Neurology and the Brain and Mind Research Institute, Weill Cornell Medical College, New York, NY, USA.
Department of Pathology, Weill Cornell Medical College, New York, NY, USA.
Department of Radiology, Weill Cornell Medical College, New York, NY, USA.


Amyloid beta-related angiitis (ABRA) of the central nervous system (CNS) is a very rare inflammatory disorder that causes destruction of CNS arteries and subsequent neuronal injury. Most patients with ABRA are old and present with cognitive dysfunction and stroke; however, some patients may present atypically. In this article, we report a 44-year-old man who presented with a first-time seizure but was otherwise neurologically intact and denied any headache. Brain MRI showed right hemispheric and bilateral medial frontal lobe hyperintensities and microhemorrhages that were most suspicious for a mass lesion. An extensive diagnostic evaluation including CSF analysis and catheter angiography was unremarkable. A brain biopsy with specific stains for amyloid surprisingly demonstrated ABRA and led to immunosuppressive treatment. The patient has remained neurologically intact and seizure-free 1 year after presentation. This case demonstrates that ABRA can occur in young patients without headache or neurologic deficits, and should be considered in patients with new-onset seizures and mass lesions. It also reinforces the need to consider a brain biopsy in patients with idiopathic brain lesions and negative non-invasive testing, as it is virtually impossible to confirm the diagnosis of ABRA otherwise.


central nervous system vasculitis; imaging; neuropathology; seizures

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