Skip to main content
Top
Published in: Neuroradiology 4/2014

01-04-2014 | Diagnostic Neuroradiology

Cerebral amyloid angiopathy-related inflammation: imaging findings and clinical outcome

Authors: Matia Martucci, Silvana Sarria, Manuel Toledo, Pilar Coscojuela, Carla Vert, Sahyly Siurana, Cristina Auger, Alex Rovira

Published in: Neuroradiology | Issue 4/2014

Login to get access

Abstract

Introduction

We aim to investigate the clinical onset, computed tomography (CT) and magnetic resonance (MR) imaging findings, and follow-up of patients with cerebral amyloid angiopathy (CAA)-related inflammation, an uncommon but clinically striking presentation of CAA.

Methods

We retrospectively reviewed the clinical manifestations, CT/MR imaging findings, and outcome of ten consecutive patients with CAA-related inflammation. In each patient, a brain CT study was performed at hospital admission, and brain MR imaging was carried out 2 to 4 days later. Clinical and radiologic follow-up findings were evaluated in all patients.

Results

The most common clinical onset was rapidly progressive cognitive decline, followed by focal neurological signs. Brain CT/MR showed unenhanced expansive subcortical lesions, corresponding to areas of vasogenic edema, associated with chronic lobar, cortical, or cortical–subcortical micro/macrohemorrhages. Clinical symptoms recovered in a few weeks under treatment in eight patients and spontaneously in the remaining two. MRI follow-up at 2 to 12 months after treatment showed resolution of the lesions. Three patients experienced symptomatic disease recurrence, with new lesions on CT/MR.

Conclusion

In the absence of histological data, early recognition of the clinical symptoms and typical radiologic features of CAA-related inflammation is essential to enable timely establishment of proper treatment.
Literature
1.
go back to reference Charidimou A, Gang Q, Werring DJ (2012) Sporadic cerebral amyloid angiopathy revisited: recent insights into pathophysiology and clinical spectrum. J Neurol Neurosurg Psychiatry 83:124–137PubMedCrossRef Charidimou A, Gang Q, Werring DJ (2012) Sporadic cerebral amyloid angiopathy revisited: recent insights into pathophysiology and clinical spectrum. J Neurol Neurosurg Psychiatry 83:124–137PubMedCrossRef
2.
go back to reference Kloppenborg RP, Richard E, Sprengers ME et al (2010) Steroid responsive encephalopathy in cerebral amyloid angiopathy: a case report and review of evidence for immunosuppressive treatment. J Neuroinflammation 9:7–18 Kloppenborg RP, Richard E, Sprengers ME et al (2010) Steroid responsive encephalopathy in cerebral amyloid angiopathy: a case report and review of evidence for immunosuppressive treatment. J Neuroinflammation 9:7–18
3.
go back to reference Kinnecom C, Lev MH, Wendell L et al (2007) Course of cerebral amyloid angiopathy-related inflammation. Neurology 68:1411–1416PubMedCrossRef Kinnecom C, Lev MH, Wendell L et al (2007) Course of cerebral amyloid angiopathy-related inflammation. Neurology 68:1411–1416PubMedCrossRef
4.
go back to reference Chao CP, Kotsenas AL, Broderick DF (2006) Cerebral amyloid angiopathy: CT and MR imaging findings. Radiographics 26:1517–1531PubMedCrossRef Chao CP, Kotsenas AL, Broderick DF (2006) Cerebral amyloid angiopathy: CT and MR imaging findings. Radiographics 26:1517–1531PubMedCrossRef
5.
go back to reference Greenberg SM, Rebeck GW, Vonsattel JP et al (1995) Apolipoprotein E epsilon 4 and cerebral hemorrhage associated with amyloid angiopathy. Ann Neurol 38:254–259PubMedCrossRef Greenberg SM, Rebeck GW, Vonsattel JP et al (1995) Apolipoprotein E epsilon 4 and cerebral hemorrhage associated with amyloid angiopathy. Ann Neurol 38:254–259PubMedCrossRef
6.
go back to reference Chung KK, Anderson NE, Hutchinson D et al (2011) Cerebral amyloid angiopathy related inflammation: three case reports and a review. J Neurol Neurosurg Psychiatry 82:20–26PubMedCrossRef Chung KK, Anderson NE, Hutchinson D et al (2011) Cerebral amyloid angiopathy related inflammation: three case reports and a review. J Neurol Neurosurg Psychiatry 82:20–26PubMedCrossRef
7.
go back to reference Clarimón J, Bertranpetit J, Calafell F et al (2003) Association study between Alzheimer's disease and genes involved in Abeta biosynthesis, aggregation and degradation: suggestive results with BACE1. J Neurol 250:956–961PubMedCrossRef Clarimón J, Bertranpetit J, Calafell F et al (2003) Association study between Alzheimer's disease and genes involved in Abeta biosynthesis, aggregation and degradation: suggestive results with BACE1. J Neurol 250:956–961PubMedCrossRef
8.
go back to reference Knudsen KA, Rosand J, Karluk D et al (2001) Clinical diagnosis of cerebral amyloid angiopathy: validation of the Boston criteria. Neurology 56:537–539PubMedCrossRef Knudsen KA, Rosand J, Karluk D et al (2001) Clinical diagnosis of cerebral amyloid angiopathy: validation of the Boston criteria. Neurology 56:537–539PubMedCrossRef
9.
go back to reference Wahlund LO, Barkhof F, Fazekas F et al (2001) European Task Force on Age-related White Matter Changes. A new rating scale for age-related white matter changes applicable to MRI and CT. Stroke 32:1318–1322PubMedCrossRef Wahlund LO, Barkhof F, Fazekas F et al (2001) European Task Force on Age-related White Matter Changes. A new rating scale for age-related white matter changes applicable to MRI and CT. Stroke 32:1318–1322PubMedCrossRef
10.
go back to reference Scolding NJ, Joseph F, Kirby PA et al (2005) Abeta-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy. Brain 128:500–515PubMedCrossRef Scolding NJ, Joseph F, Kirby PA et al (2005) Abeta-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy. Brain 128:500–515PubMedCrossRef
11.
go back to reference Greenberg SM, Vonsattel JP, Stakes JW et al (1993) The clinical spectrum of cerebral amyloid angiopathy: presentations without lobar hemorrhage. Neurology 43:2073–2079PubMedCrossRef Greenberg SM, Vonsattel JP, Stakes JW et al (1993) The clinical spectrum of cerebral amyloid angiopathy: presentations without lobar hemorrhage. Neurology 43:2073–2079PubMedCrossRef
12.
go back to reference Suo Z, Tan J, Placzek A et al (1998) Alzheimer's beta-amyloid peptides induce inflammatory cascade in human vascular cells: the roles of cytokines and CD40. Brain Res 807:110–117PubMedCrossRef Suo Z, Tan J, Placzek A et al (1998) Alzheimer's beta-amyloid peptides induce inflammatory cascade in human vascular cells: the roles of cytokines and CD40. Brain Res 807:110–117PubMedCrossRef
13.
go back to reference Miao J, Vitek MP, Xu F et al (2005) Reducing cerebral microvascular amyloid-beta protein deposition diminishes regional neuroinflammation in vasculotropic mutant amyloid precursor protein transgenic mice. J Neurosci 25:6271–6277PubMedCrossRef Miao J, Vitek MP, Xu F et al (2005) Reducing cerebral microvascular amyloid-beta protein deposition diminishes regional neuroinflammation in vasculotropic mutant amyloid precursor protein transgenic mice. J Neurosci 25:6271–6277PubMedCrossRef
14.
go back to reference Yamada M, Itoh Y, Shintaku M et al (1996) Immune reactions associated with cerebral amyloid angiopathy. Stroke 27:1155–1162PubMedCrossRef Yamada M, Itoh Y, Shintaku M et al (1996) Immune reactions associated with cerebral amyloid angiopathy. Stroke 27:1155–1162PubMedCrossRef
15.
go back to reference Sakaguchi H, Ueda A, Kosaka T et al (2011) Cerebral amyloid angiopathy-related inflammation presenting with steroid-responsive higher brain dysfunction: case report and review of the literature. J Neuroinflammation 8:116PubMedCentralPubMedCrossRef Sakaguchi H, Ueda A, Kosaka T et al (2011) Cerebral amyloid angiopathy-related inflammation presenting with steroid-responsive higher brain dysfunction: case report and review of the literature. J Neuroinflammation 8:116PubMedCentralPubMedCrossRef
16.
go back to reference Savoiardo M, Erbetta A, Di Francesco JC et al (2011) Cerebral amyloid angiopathy-related inflammation: an emerging disease. Neuroradiol J 24:253–257PubMed Savoiardo M, Erbetta A, Di Francesco JC et al (2011) Cerebral amyloid angiopathy-related inflammation: an emerging disease. Neuroradiol J 24:253–257PubMed
17.
go back to reference Savoiardo M, Erbetta A, Storchi G et al (2010) Case 159: cerebral amyloid angiopathy-related inflammation. Radiology 256:323–327PubMedCrossRef Savoiardo M, Erbetta A, Storchi G et al (2010) Case 159: cerebral amyloid angiopathy-related inflammation. Radiology 256:323–327PubMedCrossRef
18.
go back to reference Eng JA, Frosch MP, Choi K et al (2004) Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Ann Neurol 55:250–256PubMedCrossRef Eng JA, Frosch MP, Choi K et al (2004) Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Ann Neurol 55:250–256PubMedCrossRef
19.
go back to reference Wagle WA, Smith TW, Weiner M (1984) Intracerebral hemorrhage caused by cerebral amyloid angiopathy: radiographic-pathologic correlation. AJNR Am J Neuroradiol 5:171–176PubMed Wagle WA, Smith TW, Weiner M (1984) Intracerebral hemorrhage caused by cerebral amyloid angiopathy: radiographic-pathologic correlation. AJNR Am J Neuroradiol 5:171–176PubMed
20.
go back to reference Brown RT, Coates RK, Gilbert JJ (1985) Radiographic-pathologic correlation in cerebral amyloid angiopathy. A review of 12 patients. J Can Assoc Radiol 36:308–311PubMed Brown RT, Coates RK, Gilbert JJ (1985) Radiographic-pathologic correlation in cerebral amyloid angiopathy. A review of 12 patients. J Can Assoc Radiol 36:308–311PubMed
21.
go back to reference Okazaki H, Reagan TJ, Campbell RJ (1979) Clinicopathologic studies of primary cerebral amyloid angiopathy. Mayo Clin Proc 54:22–31PubMed Okazaki H, Reagan TJ, Campbell RJ (1979) Clinicopathologic studies of primary cerebral amyloid angiopathy. Mayo Clin Proc 54:22–31PubMed
22.
go back to reference Gray F, Dubas F, Roullet E et al (1985) Leukoencephalopathy in diffuse hemorrhagic cerebral amyloid angiopathy. Ann Neurol 18:54–59PubMedCrossRef Gray F, Dubas F, Roullet E et al (1985) Leukoencephalopathy in diffuse hemorrhagic cerebral amyloid angiopathy. Ann Neurol 18:54–59PubMedCrossRef
Metadata
Title
Cerebral amyloid angiopathy-related inflammation: imaging findings and clinical outcome
Authors
Matia Martucci
Silvana Sarria
Manuel Toledo
Pilar Coscojuela
Carla Vert
Sahyly Siurana
Cristina Auger
Alex Rovira
Publication date
01-04-2014
Publisher
Springer Berlin Heidelberg
Published in
Neuroradiology / Issue 4/2014
Print ISSN: 0028-3940
Electronic ISSN: 1432-1920
DOI
https://doi.org/10.1007/s00234-014-1330-6

Other articles of this Issue 4/2014

Neuroradiology 4/2014 Go to the issue