A Case of Amyloid-β-Related Angiitis: Histology, CSF-Findings and Treatment

Amyloid- β -related angiitis (ABRA) is characterized by vasculitis and accumulation of amyloid- β in vessel walls. In this case report we describe findings of cranial MRI, brain biopsy and cerebrospinal fluid in a patient with Amyloid- β -related angiitis. The cranial MRI displayed extensive multifocal white matter lesion and brain biopsy revealed amyloid angiopathy as well as granulomatous angiitis. In the cerebrospinal fluid our patient showed normal cell count, slightly elevated protein and intrathecal immunoglobulin synthesis which recovered during steroid therapy. We illustrate that detailed CSF analysis including tests for intrathecal immunoglobulin synthesis is very useful in monitoring the efficacy of therapy and disease activity in ABRA since cranial MRI and histology are not suitable for this purpose. In addition we propose that CSF findings may be very helpful in diagnosing ABRA and starting early immunosuppressive therapy. doi: http://dx.doi.org/10.4021/jnr123w