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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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case 1
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
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

slide5

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

slide7

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
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 21
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
Case 2 Continuation

Follow up MRI: increased FLAIR &enhancement is subarachnoid tissues

Spine MRI: leptomeningeal enhancement

Negative MRA

Negative 4 vessel DSA

case 2 continuation1
Case 2 Continuation

Responded to steroids

Unclear diagnosis

Readmitted 6 weeks after onset of symptoms

CSF: negative

case 22
Case 2

Lumbosacral hemilaminotomy, dural biopsy, intradural exploration & filumterminale biopsy

Diagnosis: Diffuse leptomeningeal oligodendroglioma

slide19

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
Case 3

74 yo female with speech difficulty & RUE weakness

Relapsing & remitting course

Diagnosis: gliomatosiscerebri

case 4
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
Case 6

9 yo male had a seizure while playing video games

No prior pertinent history

case 61
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