Open AccessDementia and leukoencephalopathy due to lymphomatosis cerebri
Author(s) -
Jan Lewerenz,
XiaoQi Ding,
Jakob Matschke,
Claudia Schnabel,
Pedram Emami,
Daniel von Borczyskowski,
Ralph Buchert,
Thorsten Krieger,
Maike de Wit,
Alexander Münchau
Publication year - 2009
Publication title -
bmj case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.231
H-Index - 26
ISSN - 1757-790X
DOI - 10.1136/bcr.08.2008.0752
Subject(s) - medicine , leukoencephalopathy , dementia , progressive multifocal leukoencephalopathy , neurology , pediatrics , pathology , psychiatry , disease , virology , virus
Lymphomatosis cerebri (LC) is a rare variant of primary central nervous system lymphoma (PCNSL). Clinically, the disease typically presents with a rapidly progressive dementia and unsteadiness of gait. Its presentation on cerebral MRI, which is characterised by diffuse leukoencephalopathy without contrast enhancement, often causes diagnostic confusion1 with suspected diagnoses ranging from Binswanger's disease to leukoencephalopathy or encephalomyelitis. Here we report a patient with subacute dementia and diffuse bilateral white matter changes in the cerebral hemispheres and additional involvement of the brainstem, basal ganglia and thalamus on MRI. Initially, she was considered to suffer from an autoimmune encephalitis, transiently responded to immunosuppression but then developed multiple solid appearing cerebral lymphomas.