RESUMEN
Systemic amyloidosis manifests in several organs and causes different clinical scenarios, To date, an isolated affection of intracranial blood vessels is only known to occur in cerebral amyloid angiopathy [CAA] but not is occult systemic amyloidosis or in monoclonal gammopathy of undetermined significance [MGUS]. This report describes a very rare disease because the amyloid protein was deposited for a period of nine years only in corticoleptomeningeal blood vessels as it occurs in CAA. In contrast to CAA, the reported case is coincident with a monoclonal gammopathy of undetermined significance that was not clinically and chemically detectable during about nine years. Systemic amyloid deposition was positive for the first time nine years after initial manifestation caused by a plasma cell-secreted monoclonal lambda-light chain protein representing a progression of MGUS. A pathological plasma cell clone or any signs of a proliferative plasma cell disease were not detectable until today except for the monoclonal protein. A case like has never been published before