Prealbumin type cerebral amyloid angiopathy in familial amyloid polyneuropathy

Immunocytochemical and electron-microscopic studies were performed on the central nervous system(CNS) in 10 cases with type I familial amyloid polyneuropathy. All cases showed CNS amyloid deposits, mainly on the leptomeningeal vessels and pia-arachnoid membranes, with arteries and arterioles in subarachnoidal space being the predominant site of cerebral amyloid accumulation. All of these amyloid deposits were specifically immunolabeled by an anti-human prealbumin antibody. However, there were no prealbumin deposits in the brain parenchyma. It is concluded that CNS prealbumin-type amyloid deposition with cerebral amyloid angiopathy is a common pathological finding in this disease.