Amyloid β-Related Central Nervous System Angiitis Presenting With an Isolated Seizure Academic Article uri icon

Overview

MeSH Major

  • Alzheimer Disease
  • Amyloid beta-Protein Precursor
  • Arcuate Nucleus of Hypothalamus
  • Hypothalamus
  • Leptin
  • Neuropeptide Y
  • Weight Loss

abstract

  • 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.

publication date

  • January 2014

Research

keywords

  • Academic Article

Identity

Language

  • eng

PubMed Central ID

  • PMC3975790

Digital Object Identifier (DOI)

  • 10.1177/1941874413502796

PubMed ID

  • 24707337

Additional Document Info

start page

  • 86

end page

  • 9

volume

  • 4

number

  • 2