Case of Cerebral Amyloid Angiopathy-Related Inflammation - Is the Absence of Cerebral Microbleeds A Good Prognostic Sign?
OBJECTIVE
The aim of this study is to describe a case of pathologically proven cerebral amyloid angiopathy-related inflammation (CAA-I) without cerebral microbleeds (CMBs) and its clinical course.


BACKGROUND
CAA-I is an uncommon variant of cerebral amyloid angiopathy. Keys to diagnosis rely on the physician's awareness of this entity, CMBs on magnetic resonance imaging (MRI), an often favorable response to immunosuppression, and ultimately brain biopsy. CAA-I with no CMBs is rarely reported.


RESULTS
A 76-year-old woman presented with 4 weeks of headaches and was found to have visual neglect on the left part of the visual field. MRI of the brain showed sulcal/gyriform hyperintensity with associated leptomeningeal enhancement in the right occipital lobe on fluid-attenuated inversion recovery (FLAIR) imaging. No CMBs or large parenchymal FLAIR lesions were seen on MRI. Biopsy was consistent with CAA-I. The patient's headaches resolved spontaneously and no immunosuppression was initiated. The patient remained asymptomatic for the 18 months of follow-up.


CONCLUSIONS
To the best of our knowledge, there has been only one previous case of pathology-proven CAA-I without CMBs reported and this was associated with a good prognosis. Lack of CMBs and/or large parenchymal FLAIR lesions may be a prognostic factor in this disease.
Department of Neurology, Mount Sinai Beth Israel, Icahn School of Medicine, New York, New York. Electronic address: johnliangmd@gmail.com.
John W
JW
Liang
Department of Pathology, Mount Sinai Beth Israel, Icahn School of Medicine, New York, New York.
Wei
W
Zhang
Department of Pathology, Mount Sinai Beth Israel, Icahn School of Medicine, New York, New York.
Jonathan
J
Sarlin
Department of Neurology, Mount Sinai Beth Israel, Icahn School of Medicine, New York, New York.
Irene
I
Boniece
D000368:Aged; D000802:Angiotensin Amide; D016657:Cerebral Amyloid Angiopathy / Q000150:complications*; D002543:Cerebral Hemorrhage / Q000209:etiology*; D003920:Diabetes Mellitus; D005260:Female; D006801:Humans; D007249:Inflammation / Q000150:complications*; D008279:Magnetic Resonance Imaging
D000802:Angiotensin Amide
Amyloid; CAA; inflammation; microhemorrhage