Lymphomatosis cerebri mimicking iatrogenic Creutzfeldt-Jakob disease

• Published in: BMJ case reports Vol. 2014; p. bcr2013201246
• Main Authors: Rivero Sanz, Elena, Torralba Cabeza, Miguel Ángel, Portugal, Francisco Sanjuán, García-Bragado, Federico
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group LTD 08-09-2014; BMJ Publishing Group
• Series: Case Report
• Subjects: Brain - pathology; Brain Neoplasms - diagnosis; Creutzfeldt-Jakob disease; Creutzfeldt-Jakob Syndrome - diagnosis; Dementia; Dementia - diagnosis; Dementia - etiology; Diagnosis, Differential; Encephalitis; Europe (West); Fatal Outcome; Female; Humans; Iatrogenesis; Iatrogenic Disease; Lymphoma; Lymphoma, Non-Hodgkin - complications; Lymphoma, Non-Hodgkin - diagnosis; Metabolism; Middle Aged; Personality; Postoperative Complications; Proteins; Toxicity; Tumors; Unusual Association of Diseases/Symptoms; White
• ISSN: 1757-790X; 1757-790X
• DOI: 10.1136/bcr-2013-201246

Lymphomatosis cerebri (LC) is a rare variant of primary central nervous system lymphoma (PCNSL) whereby individual lymphoma cells infiltrate the cerebral white matter without causing a mass effect. The disease characteristically presents as a rapidly progressive dementia, which opens an ample differential diagnosis of toxic, metabolic, neurodegenerative and infective causes. Other presentations also include changes in personality, myoclonus and psychotic symptoms. Here we report a patient who presented with a rapidly progressive dementia with a unique surgical history of a dural mater graft in the 1970s. The diagnosis of iatrogenic Creutzfeldt-Jakob disease (iCJD) was initially considered. However, the patient’s clinical status deteriorated rapidly with no response to symptomatic treatment and she died 2 months after symptom onset. A diagnosis of T-type LC was reached at autopsy.