Abstract
Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a relatively rare syndrome of reversible encephalopathy and could be divided into two subtypes of inflammatory CAA (ICAA) and amyloid-β-related angiitis (ABRA) according to histopathology. We present a case of pathologically proved ABRA with partial seizures and headache, and a focal lesion in the right temporal lobes on magnetic resonance imaging. Summarized from previous 139 ABRA and ICAA cases, ABRA is preferred when the lesion is enhanced on MRI and requires combination drug therapy, while ICAA is highly suspected with ApoE genotype of ɛ4/ɛ4. More clinical markers for diagnosis of CAA-ri warrant further researches.
Keywords:
Amyloid-β-related angiitis; cerebral amyloid angiopathy; cerebral amyloid angiopathy-related inflammation; inflammatory cerebral amyloid angiopathy.
Publication types
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Case Reports
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Comparative Study
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Research Support, Non-U.S. Gov't
MeSH terms
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Apolipoproteins E / genetics
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Cerebral Amyloid Angiopathy / diagnostic imaging
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Cerebral Amyloid Angiopathy / pathology
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Cerebral Amyloid Angiopathy / physiopathology*
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Cerebral Amyloid Angiopathy / therapy*
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Headache / diagnostic imaging
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Headache / pathology
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Headache / physiopathology
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Headache / therapy
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Humans
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Inflammation / diagnostic imaging
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Inflammation / pathology
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Inflammation / physiopathology*
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Inflammation / therapy*
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Magnetic Resonance Imaging
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Male
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Middle Aged
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Seizures / diagnostic imaging
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Seizures / pathology
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Seizures / physiopathology
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Seizures / therapy
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Vasculitis, Central Nervous System / diagnostic imaging
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Vasculitis, Central Nervous System / pathology
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Vasculitis, Central Nervous System / physiopathology*
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Vasculitis, Central Nervous System / therapy*