A Par 3 Macular Hole? (Video)

The initial evaluation of rapidly progressive visual loss with mildly sluggish pupils raised concern for bilateral occipital disease. The recent 'vasculitis' history with systemic symptoms also put GCA high on the list of differentials. Lab abnormalities included elevated ESR (86), CRP (.90) and platelet count (442). MRI revealed an extensive area of increased signal on FLAIR images bilaterally involving the occipitoparietal regions, cerebellar peduncles and splenium of the corpus callosum. CSF exam revealed 3 WBCs and a protein of 45. 14-3-3 protein test was ambiguous, but tau test was negative. There were no oligoclonal bands but myelin basic protein (28.5) and IgG index (0.71) were elevated. The JC virus PCR was elevated at 343,124 copies in CSF and 3623 copies in serum (nl <500). A diagnosis of progressive multifocal leukoencephalopathy (PML) was made, but the underlying immune predisposition wasn't obvious with negative HIV testing, CD4/CD8 ratio normal and lymphoma/leukemia phenotyping negative. The only risk noted was her steroid use. Over the following 3 weeks of hospitalization vision deteriorated to NLP OU. She was discharged home but readmitted 3 weeks later with progressive lethargy and difficulty breathing. Evaluation for possible pulmonary embolus led to a CT chest revealing extensive bilateral hilar, mediastinal and axillary adenopathy. Prior portable CXR had shown prominence of the superior mediastinum likely related to the portable technique. Axillary lymph node biopsy revealed expansion of the paracortical T zone by pleomorphic lymphocytic population with scattered immunoblasts and increased vascularity throughout. Immunohistochemistry showed positivity for CD3, CD5, CD7 and pan T cell markers consistent with a diagnosis of angioimmunoblastic T cell lymphoma. PCR identified clonal rearrangement of the T-cell receptor genes. Repeat MRI showed progression of her PML lesions. She was discharged to hospice and died a few months later. Post-mortem examination was not obtained.