Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
July 2020
Next Case of the Month Coming August 4...
Cerebral Amyloid Angiopathy–Related Inflammation (CAARI)
- Background:
- CAARI, also called amyloid-β-related angiitis, is a rare form of cerebral amyloid angiopathy with a predominantly vascular inflammation or angiitis.
- Clinical Presentation:
- Patients typically present with seizures, headache, and strokelike episodes, along with an acute or subacute decline in cognitive status.
- Key Diagnostic Features:
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The striking radiologic features include vasogenic edema involving the subcortical white matter, accompanied by mass effect, typically in frontal and temporal lobes, but presenting as a multifocal involvement of both hemispheres in 30% of patients. Cortical involvement can occur and accounts for seizures in these patients. These changes in association with multiple microhemorrhages diffusely distributed throughout the brain (best seen on SWI) make the diagnosis.
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Slight leptomeningeal enhancement can also be present.
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A reliable diagnosis of CAARI can be reached from clinical and MRI information alone, with good sensitivity and excellent specificity, exempting brain biopsy.
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According to new criteria, probable CAARI will be diagnosed in the setting of the following:
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Age ≥ 40 years
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Acute or subacute onset of symptoms
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Presence of ≥ 1 of the following clinical features: headache, decrease in consciousness, behavioral change, or focal neurologic signs and seizures; the presentation is not directly attributable to an acute ICH
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MRI shows unifocal or multifocal WMH lesions (corticosubcortical or deep) that are asymmetric and extend to the immediately subcortical white matter; the asymmetry is not due to past ICH
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Presence of ≥ 1 of the following corticosubcortical hemorrhagic lesions: cerebral macrobleed, cerebral microbleed, or cortical superficial siderosis
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Absence of neoplastic, infectious, or other cause
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- Differential Diagnoses
- The main differential diagnosis includes primary CNS vasculitis, PRES, and PML. When there is one single lesion, brain tumor or tumefactive inflammatory lesion may also be considered in the differential.
- Treatment:
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These patients usually respond well to a pulse of corticosteroids.
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Some authors have stated the role of immune or cytostatic agents.
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