ABSTRACT
An unusual systemic amyloidosis with visceral and cerebral amyloid deposits was reported by W. Krücke. However, the amyloid protein of this patient has never been classified according to the chemical nosology. Subsequent citations referred to this disease as “primary amyloidosis”, indicating a monoclonal gammopathy as the underlying disease. Here, this disorder was re-diagnosed using immunohistochemistry and gene analysis. The results show the identification of the hereditary amyloidosis of transthyretin-origin (ATTR) due to the M30V mutant.
