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Clinical Diagnosis and Successful Treatment of Inflammatory Cerebral Amyloid Angiopathy

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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.

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Authors and Affiliations

  1. Department of Neurology and Neurological Sciences, Feinberg School of Medicine of Northwestern University, 710 North Lake Shore Drive, Abbott Hall 11th Floor, Chicago, IL, 60611, USA

    Richard A. Bernstein, Michael Gibbs & H. Hunt Batjer

  2. Department of Neurological Surgery, Feinberg School of Medicine of Northwestern University, Chicago, USA

    Richard A. Bernstein, Michael Gibbs & H. Hunt Batjer

Authors
  1. Richard A. Bernstein
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  2. Michael Gibbs
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  3. H. Hunt Batjer
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Corresponding author

Correspondence to Richard A. Bernstein.

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Bernstein, R.A., Gibbs, M. & Hunt Batjer, H. Clinical Diagnosis and Successful Treatment of Inflammatory Cerebral Amyloid Angiopathy. Neurocrit Care 14, 453–455 (2011). https://doi.org/10.1007/s12028-010-9497-0

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  • DOI: https://doi.org/10.1007/s12028-010-9497-0

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