Lymphomatosis cerebri mimicking iatrogenic Creutzfeldt-Jakob disease

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.

Figure 1

A brain MRI showing bilateral hyperintense signal in the white matter of the frontal lobes and the caudate nuclei, putamen and thalamus in fluid-attenuated inversion recovery and T2-weighted images.

Figure 2

(A) An EEG study on admission showing paroxysmal sharp-wave periodic complexes suggesting the diagnosis of Creutzfeldt-Jacob disease. (B) A repeat EEG showing 2 months after admission with similar findings to the first EEG.

Figure 3

(A) Atypical lymphoid cells in close relation with blood vessels. (B) Absence of spongiform changes in the cerebral cortex. (C) Positive CD3 staining of the tumour cells.