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Amyloid-beta-related angiitis: a treatable rapidly progressive dementia
  1. Memoona Nasir1,
  2. Donald Nortey1,
  3. Naveed Malek1,
  4. Fernanda Valerio2,
  5. Abhijit Chaudhuri1
  1. 1 Department of Neurology, Queen's Hospital, Romford, UK
  2. 2 Division of Neuropathology, National Hospital for Neurology and Neurosurgery, London, UK
  1. Correspondence to Dr Memoona Nasir, Department of Neurology, Queen's Hospital, Romford, UK; memoona.nasir{at}nhs.net

Abstract

A 73-year-old woman developed cognitive decline over 1 year. MR scan of the brain showed a focal asymmetrical leukoencephalopathy involving the right frontal, temporal, parietal and occipital lobes. Extensive laboratory investigations found no cause but brain biopsy identified amyloid-beta-related angiitis (ABRA), a potentially treatable cause of rapid-onset dementia. We gave intravenous methylprednisolone and then two courses of intravenous cyclophosphamide, after which her cognitive skills gradually but significantly improved over several months.

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Footnotes

  • Contributors All authors contributed equally to the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned. Externally peer reviewed by Neil Scolding, Bristol, UK.

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  • Editors’ commentary
    Phil E M Smith Geraint N Fuller
  • Editorial
    N J Scolding

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