Article Text

Download PDFPDF

Letter
Minimally symptomatic cerebral amyloid angiopathy-related inflammation: three descriptive case reports
  1. Gargi Banerjee1,
  2. Debie Alvares2,
  3. John Bowen3,
  4. Matthew E Adams4,
  5. David J Werring1
  1. 1 Stroke Research Centre, Department of Brain Repair and Rehabilitation, UCL Institute of Neurology and the National Hospital for Neurology and Neurosurgery, London, UK
  2. 2 Department of Neurology, St. Richard’s Hospital, Western Sussex Hospitals NHS Foundation Trust, Chichester, UK
  3. 3 Department of Neurology, Royal Shrewsbury Hospital, The Shrewsbury and Telford Hospital NHS Trust, Shrewsbury, UK
  4. 4 Lysholm Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, London, UK
  1. Correspondence to Dr David J Werring, UCL Stroke Research Centre, Department of Brain Repair and Rehabilitation, UCL Institute of Neurology, London WC1B 5EH, UK; d.werring{at}ucl.ac.uk

Statistics from Altmetric.com

Introduction

Cerebral amyloid angiopathy-related inflammation (CAA-ri) is an unusual cause of encephalopathy, seizures and focal neurological deficits.1 2 We report three cases of CAA-ri with minimal symptoms but striking and dynamically evolving brain MRI findings.

Case 1

A 62-year-old man presented with a moderately severe non-radiating frontal headache. Brain MRI 9 months later showed multiple discrete regions of abnormal signal and mild swelling involving white matter and overlying cortex. Susceptibility-weighted imaging (SWI) demonstrated numerous cortical lobar microbleeds throughout both cerebral hemispheres. Repeat MRI another 9 months later showed resolution of many of the parenchymal abnormalities, but with several new regions containing more peripheral microbleeds. Amyloid-PET (using 18F-florbetapir) demonstrated moderate widespread amyloid deposition; CSF analysis showed reduced amyloid-beta 1–42 and high-normal total tau. Formal neuropsychological testing suggested mild compromise in frontal functioning only. The patient was treated with 5 days of intravenous methylprednisolone (1 g daily), followed by an oral taper from prednisolone 60 mg over 8 weeks. Follow-up MRI after 8 months showed almost complete resolution of the parenchymal abnormalities, but with persisting lobar microbleeds. At 24 months following symptom onset, he remains asymptomatic, with stable brain imaging.

Case 2

A 74-year-old man presented with mild subjective memory difficulties only, with no objective neuropsychological deficits. MRI demonstrated a substantial region of abnormal signal in the right temporal and occipital white matter, with no enhancement. Repeat imaging after a few weeks showed partial regression. Over the following 4 years, three further MRIs showed multiple areas of abnormal white matter (sometimes involving cortex as well) within the temporal, parietal and occipital lobes, which …

View Full Text

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.