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

• Published in: Irish journal of medical science Vol. 187; no. 2; pp. 319 - 322
• Main Authors: Brett, F. M., Chen, D., Loftus, T., Langan, Y., Looby, S., Hutchinson, S.
• Format: Journal Article
• Language: English
• Published: London Springer London 01-05-2018
• Subjects: Case Based Review; Family Medicine; General Practice; Internal Medicine; Medicine; Medicine & Public Health; sCJD—sporadic CJD; IVLBCL—intravascular large B-cell lymphoma; PCNSL—primary CNS lymphoma
• ISSN: 0021-1265; 1863-4362
• DOI: 10.1007/s11845-017-1653-5

Background
In patients presenting with rapidly progressive dementia, prion disease may enter the differential diagnosis. The commonest malignancies masquerading as prion disease are primary CNS lymphoma and intravascular large B-cell lymphoma, both rare and difficult to diagnose without brain biopsy.
Case presentation
This 82-year-old lady with a past history of hypertension, presented with rapidly progressive cognitive impairment and ataxia. The possibility of sCJD was raised. Brain biopsy was carried out. Western blot for prion protein was negative. Brain biopsy showed intravascular large B-cell lymphoma. She died shortly afterwards.
Conclusion
The clinical presentation of intravascular large B-cell lymphoma is diverse. Patients may present as in this case with dementia, seizures, and myoclonus leading to a clinical diagnosis of sCJD. The diagnosis here was made at biopsy but is made at autopsy in over 50% of cases.