Cerebral amyloid angiopathy (CAA) is characterized by deposition of beta amyloid protein in cortical and leptomeningeal arteries of the brain resulting in lobar microhemorrhages on MRI imaging.  Patients can present with cognitive decline, lobar hemorrhages, seizures and small vessel ischemic infarcts. Primary progressive aphasia (PPA) is a neurodegenerative disease variant of fronto-temporal dementia causing atrophy in the language dominant hemisphere and progressive language deficits. While there are some anecdotal reports of amyloid deposition in the Alzheimer’s variant of PPA, the association between the above two disease entities has not been established. 

Case report

A 73-year-old Caucasian woman presented with 2 intraparenchymal hemorrhages in the left cerebellum (2006) and left temporal lobe (2009) over 3 years with no residual physical deficits.  Over the next few years she experienced significant language impairment characterized by progressive semantic aphasia, difficulty following 3 step commands and ideomotor apraxia, difficulty with repetition and reading which met criteria for clinical logopenic PPA.  Serial MRI scans from 2010- present have shown development of microhemorrhages in cortical areas, worsening superficial siderosis in bilateral hemispheres and progression of white matter hyperintensities consistent with CAA.  In 2017 she had a distinct period of worsening of aphasia associated with CAA related inflammation that was treated with corticosteroids.

This is a novel case reporting a distinct association between CAA and PPA as the patient started to develop isolated progressive language impairment with no physical deficits after her intracerebral hemorrhages. The clinical progression of her aphasia has correlated anatomically with the radiological progression of CAA. Further research is necessary to anatomically and pathologically identify the link between the two disease entities.