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Lymphomatosis cerebri mimicking iatrogenic Creutzfeldt-Jakob disease
Elena Rivero Sanz1, Miguel Ángel Torralba Cabeza2, Francisco Sanjuán Portugal3
1Department of Neurology, Hospital Clinico Universitario Lozano Blesa, Zaragoza, Spain.
Abstract:
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.
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