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University of North Carolina at Chape l Hill June 2013. Neuroradiology- neuropathology conference Felipe Espinoza, MD. Case 1. 58 yo female with decreased vision OD Diagnosed with bilateral granulomatous pan-uveitis 1/2013 presented with dizziness, vision changes & ear discharge.
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University of North Carolina at Chapel Hill June 2013 Neuroradiology-neuropathology conferenceFelipe Espinoza, MD
Case 1 58 yo female with decreased vision OD Diagnosed with bilateral granulomatous pan-uveitis 1/2013 presented with dizziness, vision changes & ear discharge
Case 1 Continuation Had a brain biopsy & started treatment Patient develop left eye blindness 2 days after brain surgery Vitreous fluid cytometry: Large B cell lymphoma
3 of 55 patients during an 11- year period had primary vitreous involvement without systemic symptoms 5% of patients with systemic lymphoma had vitreous involvement
1ry vitreoretinal lymphoma • 1ry vitreoretinal lymphoma (PVRL) is a subset of primary CNS lymphoma (PCNSL). • PVRL presents in the eye with or without simultaneous CNS involvement. • An extranodal non-Hodgkin, diffuse large B-cell lymphoma. • Median age of onset is late 50s and 60s with a female preponderance. • 25% of patients with PCNSL without eye involvement subsequently develop PVRL. • 60–80% with PVRL without CNS involvement subsequently develop PCNSL. • Median survival after CNS involvement is reported to be 12–18 months. Katoch D, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-009354
Case 2 12 yo female, transferred from OSH with 3 week history of HA, numbness of hands & feet & slurred speech CSF-protein of 256, opening pressure of 55 Empirically treated for viral encephalitis & sent home.
Case 2 No improvement & presented to ER again after severe HA with slurred speech & inability to recognize her mother, right arm twitching Outside CT: negative
Case 2 Continuation Follow up MRI: increased FLAIR &enhancement is subarachnoid tissues Spine MRI: leptomeningeal enhancement Negative MRA Negative 4 vessel DSA
Case 2 Continuation Responded to steroids Unclear diagnosis Readmitted 6 weeks after onset of symptoms CSF: negative
Case 2 Lumbosacral hemilaminotomy, dural biopsy, intradural exploration & filumterminale biopsy Diagnosis: Diffuse leptomeningeal oligodendroglioma
Disseminated leptomeningealoligodendroglioma • Diffuse leptomeningealgliomatosis is a rare condition in which gliomagrows primarily within the subarachnoid space rather than within the brain parenchyma. • While the majority of these diffuse leptomeningealgliomasare astrocytic, several cases of diffuse leptomeningealoligodendroglioma have been reported, including primary cases where no intraparenchymal lesions were identified. • Many of the cases of diffuse leptomeningealoligodendroglioma have occurred in children. • Diagnosis can be difficult, since CSF generally shows elevated protein levels without neoplastic cells, and only fibrosis may be evident in the biopsy . • Histology often demonstrates round, oligodendroglioma-like cells with perinuclearhalos. • While these tumors are often low-grade, anaplastic progression can occur and is associated with a shorter survival time. • In a series of 9 patients who died from this disease, survival ranged from 3 months to 21 yrs. • Mathews MS, Par LS, Kuo JV, Kim RC. Primary leptomeningealoligodendrogliomatosis. J Neurooncol. 2009; 94(2):275-8. • OzkulA, Meteoglu I, TatarogluC, Akyol A. Primary diffuse leptomeningealoligodendrogliomatosis causing sudden death. J Neurooncol. 2007; 81(1):75-9. • Armao DM, Stone J, Castillo M, Mitchell KM, Bouldin TW, Suzuki K. Diffuse leptomeningealoligodendrogliomatosis: radiologic/pathologic correlation. AJNR Am J Neuroradiol. 2000; 21(6):1122-6. • Rodriguez FJ, Perry A, Rosenblum MK, Krawitz S, Cohen KJ, Lin D, Mosier S, Lin MT, Eberhart CG, Burger PC. Disseminated oligodendroglial-like leptomeningeal tumor of childhood: a distinctive clinicopathologicentity. ActaNeuropathol. 2012;124(5):627-41.
Case 3 74 yo female with speech difficulty & RUE weakness Relapsing & remitting course Diagnosis: gliomatosiscerebri
Case 4 49 yo male bilateral hemianopsia history of esthesioneuroblastoma s/p 2 cycles of chemo therapy “chemotherapy resistant” disease Transferred for fever & possible meningitis Diagnosis: atypical pituitary adenoma
Case 6 9 yo male had a seizure while playing video games No prior pertinent history
Case 6 Diagnosis: DNET <1% of 1ry brain tumors 1% of neuroepithelial tumors in patients older than 20 years of age Reported in 5-80% of epilepsy specimens