Isolated intracerebral light chain deposition disease: novel imaging and pathologic findings

Light chain deposition disease (LCDD) is a rare clinicopathologic entity first described in 1976 and is characterized by a monoclonal gammopathy resulting in nonamyloid immunoglobulin light chain tissue deposition. Only four cases of intracerebral LCDD have been previously reported, all in the setting of a known plasma cell dyscrasia or in the presence of local mature plasma cells. We present the first case of intracranial LCDD in the absence of a known plasma cell dyscrasia or local mature plasma cells.