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Neurological rarities
Relapsing cerebral amyloid angiopathy-related inflammation: the wax and the wane
  1. Sharfaraz Salam1,
  2. Mayooreshan Anandarajah2,
  3. Sarah Al-Bachari1,
  4. Piyali Pal3,
  5. Jonathan Sussman1,
  6. Hisham Hamdalla1
  1. 1Department of Neurology, Greater Manchester Neurosciences Centre, Salford Royal NHS Trust, Salford, UK
  2. 2Royal Preston Hospital, Preston, UK
  3. 3Department of Neuropathology, Greater Manchester Neurosciences Centre, Salford Royal NHS Trust, Salford, UK
  1. Correspondence to Dr Sharfaraz Salam, Department of Neurology, Greater Manchester Neurosciences Centre, Salford Royal NHS Trust, Salford M6 8HD, UK; sharfaraz{at}doctors.org.uk

Abstract

Cerebral amyloid angiopathy-related inflammation (CAA-I) is a rare variant of cerebral amyloid angiopathy (CAA). Its precise pathophysiology remains uncertain and we currently have limited evidence on which immunosuppressive agents are the most effective in its treatment. The disease course of CAA-I disorders can vary from an isolated clinical event to recurrent episodes. We present a case of biopsy-confirmed CAA-I that gives insight into its potential relapsing nature and the challenges of its long-term management.

  • cerebral amyloid angiopathy related inflammation cerebrovascular disease

https://doi.org/10.1136/practneurol-2017-001599

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    Footnotes

    • Contributors SS was responsible for conception, writing of the article and revision of drafts. MA aided with case presentation for the article. SAB contributed to conception and supervised the editing of the article. PP provided histology slides for the article and description of changes seen. JS is the attending neurologist responsible for the patient’s care. HH contributed to conception of the article and was responsible for the patient’s initial care.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed. This paper was reviewed by Neil Scolding, Bristol, UK.