Intravascular large B-cell lymphoma presenting clinically as rapidly progressive dementia

Area of Science:

• Neurology
• Oncology
• Pathology

Background:

• Prion diseases are a key differential diagnosis for rapidly progressive dementia.
• Primary CNS lymphoma and intravascular large B-cell lymphoma are rare malignancies that can mimic prion disease.
• Diagnosis often requires invasive procedures like brain biopsy due to overlapping clinical presentations.

Observation:

• An 82-year-old female with hypertension presented with rapidly progressive cognitive impairment and ataxia.
• Creutzfeldt-Jakob disease (sCJD) was initially suspected.
• Brain biopsy revealed intravascular large B-cell lymphoma, with negative prion protein Western blot.

Findings:

• Intravascular large B-cell lymphoma can present with diverse neurological symptoms, including dementia, seizures, and myoclonus.
• The clinical presentation can lead to a misdiagnosis of sCJD.
• Diagnosis was confirmed by biopsy in this case, but is often made post-mortem.

Implications:

• Highlights the importance of considering intravascular large B-cell lymphoma in the differential diagnosis of rapidly progressive dementia.
• Emphasizes the diagnostic challenges and the potential for misdiagnosis without definitive tissue analysis.
• Underscores the need for prompt diagnostic evaluation, as delays can impact patient outcomes.