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Neurocritical Care

, Volume 14, Issue 3, pp 453–455 | Cite as

Clinical Diagnosis and Successful Treatment of Inflammatory Cerebral Amyloid Angiopathy

  • Richard A. BernsteinEmail author
  • Michael Gibbs
  • H. Hunt Batjer
Practical Pearl

Abstract

Introduction

Cerebral amyloid angiopathy is a major cause of lobar hemorrhage in older adults, and of microvascular ischemic disease. The rarest form of this disease is an inflammatory form causing seizures. It is important to recognize because the patients usually respond to a brief course of immunosuppression.

Methods

Case report.

Results

A 66-year-old man developed gradual cognitive decline, insidiously increasing headaches, and then had a likely seizure. MRI showed diffuse white matter edema, and innumerable superficial microhemorrhages characteristic of amyloid angiopathy. He was empirically treated with oral prednisone and an anticonvulsant. His symptoms improved and the white matter edema resolved over several months. ApoE genotype was 4/4, which is commonly found in inflammatory amyloid angiopathy.

Conclusions

Inflammatory cerebral amyloid angiopathy can be clinically diagnosed and treated without brain biopsy. Clinical diagnosis is important because of the good response to a brief course of steroids in most cases.

Keywords

Cerebral amyloid angiopathy Amyloid-related inflammation Seizures Microhemorrhages Lobar hemorrhage Leukoencephalopathy 

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Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • Richard A. Bernstein
    • 1
    • 2
    Email author
  • Michael Gibbs
    • 1
    • 2
  • H. Hunt Batjer
    • 1
    • 2
  1. 1.Department of Neurology and Neurological SciencesFeinberg School of Medicine of Northwestern UniversityChicagoUSA
  2. 2.Department of Neurological SurgeryFeinberg School of Medicine of Northwestern UniversityChicagoUSA

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