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Diagnosis and Treatment of Cerebral Amyloid Angiopathy

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Headache

Language disturbances Vision changes

Seizures

Palinopsia: Persistence of a visual image after removing the stimulus (occasionally related to

focal seizures)

Optic ataxia: Impairment of visually-guided

reaching

A 55-year-old woman with recurrent episodes of:

Differential diagnosis:

Cerebral amyloid angiopathy-related inflammation

Patient treatment and outcome Posterior reversible

encephalopathy syndrome CNS vasculitis

Asymmetric white matter

hyperintensities involving U-fibers

Enlarged

perivascular spaces Microhemorrhages

Reversible cerebral

vasoconstriction syndrome Autoimmune or

inflammatory disorder

Diagnosis and Treatment of

Cerebral Amyloid Angiopathy- Related Inflammation

Multifocal process involving occipital or

parieto-occipital and frontal regions

Thick and hyalinized leptomeningeal and cortical vessel walls Strongly positive for ß-amyloid

Immunosuppressive therapy: Intravenous solumedrol 1 g daily for 5 days followed by prednisone 60 mg daily, tapered to discontinuation over 12 weeks Levetiracetam 2,000 mg and lacosamide 200 mg, both twice daily

The patient remained seizure-free Marked improvement in brain MRI Mycophenolate 1,000 mg twice daily

As most patients respond to IV steroid therapy, correct diagnosis of cerebral amyloid angiopathy-related

inflammation is critical to achieve favorable clinical outcomes

Radiologic findings

Immuno-histochemical staining

doi:10.1212/WNL.0000000000013219

Copyright © 2022 American Academy of Neurology

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