F M Brett1,2, D Chen3, T Loftus3, Y Langan4, S Looby3, S Hutchinson4. 1. Department of Clinical Neurological Sciences, RCSI, Dublin 9, Ireland. francescabrett@rcsi.ie. 2. Department of Clinical Neurological Sciences, Beaumont Hospital, Dublin, Ireland. francescabrett@rcsi.ie. 3. Department of Clinical Neurological Sciences, RCSI, Dublin 9, Ireland. 4. Department of Neurology, St James Hospital, Dublin, Ireland.
Abstract
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.
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.
Entities:
Keywords:
IVLBCL—intravascular large B-cell lymphoma; PCNSL—primary CNS lymphoma; sCJD—sporadic CJD
Authors: M Ponzoni; G Arrigoni; V E Gould; B Del Curto; M Maggioni; A Scapinello; S Paolino; A Cassisa; C Patriarca Journal: Hum Pathol Date: 2000-02 Impact factor: 3.466
Authors: Jonathan M Schott; Lilla Reiniger; Maria Thom; Janice L Holton; Joan Grieve; Sebastian Brandner; Jason D Warren; Tamas Revesz Journal: Acta Neuropathol Date: 2010-07-18 Impact factor: 17.088
Authors: J M Baehring; C Henchcliffe; C J Ledezma; R Fulbright; F H Hochberg Journal: J Neurol Neurosurg Psychiatry Date: 2005-04 Impact factor: 10.154
Authors: William W L Choi; Dennis D Weisenburger; Timothy C Greiner; Miguel A Piris; Alison H Banham; Jan Delabie; Rita M Braziel; Huimin Geng; Javeed Iqbal; Georg Lenz; Julie M Vose; Christine P Hans; Kai Fu; Lynette M Smith; Min Li; Zhongfeng Liu; Randy D Gascoyne; Andreas Rosenwald; German Ott; Lisa M Rimsza; Elias Campo; Elaine S Jaffe; David L Jaye; Louis M Staudt; Wing C Chan Journal: Clin Cancer Res Date: 2009-08-25 Impact factor: 12.531
Authors: Alexander H Peden; Lynne I McGuire; Nigel E J Appleford; Gary Mallinson; Jason M Wilham; Christina D Orrú; Byron Caughey; James W Ironside; Richard S Knight; Robert G Will; Alison J E Green; Mark W Head Journal: J Gen Virol Date: 2011-10-26 Impact factor: 3.891
Authors: F M Brett; S Looby; A Chalissery; D Chen; C Heaney; J Heffernan; F Cunningham; R Howley; T Loftus; H Kearney; M A Farrell Journal: Ir J Med Sci Date: 2017-08-12 Impact factor: 1.568