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Washington University Experience | VASCULAR | Herniation, tonsillar | 8A0 Case 8 History
Case 8 History ---- The patient was a 60-year-old woman with a history of hypertension, hyperlipidemia, diabetes mellitus and sickle cell trait, who presented with a steroid-responsive encephalopathy and stroke syndrome. The patient initially presented in January 2013 with altered mental status, and was found to have multiple left middle cerebral artery distribution subcortical strokes secondary to left internal carotid artery stenosis. The patient was readmitted in early October 2014 and early December 2014 with similar symptoms. An extensive laboratory work-up in October 2014 included multiple CSF analyses, skin and muscle biopsy, all of which were negative for malignancy, given a concern for intravascular lymphoma. Similarly, an extensive work-up for infectious etiologies and a hepatitis panel were negative. Among multiple serum markers of rheumatologic disease, a serum ANA was positive at 1:5120, with a nucleolar staining pattern, meriting concern for lupus cerebritis given a reported history of lupus in her mother. Following treatment with high-dose intravenous steroids, the patient showed marked improvement in her mental status, and was discharged on a prednisone taper to 20 mg/day. She was seen as an outpatient in late November 2014, and was started on CellCept in addition to her prednisone, given the presumption of lupus cerebritis. She re-presented in early December with word-finding difficulty and bilateral lower extremity weakness. MR imaging performed December 2014 showed multiple punctate MCA infarcts throughout the left hemisphere a well as several, scattered punctate infarcts involving the right frontal and parietal lobes. There was absent flow void of the left internal carotid artery compatible with patient's known stenosis. Following treatment with high-dose intravenous steroids and a dose of Cytoxan, the patient again showed marked improvement in her mental status, and was discharged to inpatient rehabilitation in mid-December 2014. At the time of discharge in December 2014, she was alert and oriented to person but with signs of a fluent aphasia. She was re-admitted at the end of December for similar but worse symptoms. She was initially admitted to the Stroke Service, where she was started on empiric vancomycin, cefepime, acyclovir and ampicillin for possible infections. She developed respiratory distress, was intubated and transferred to the Intensive Care Unit (ICU). MR imaging performed December 31 showed extensive microangiopathic disease. Steroids were held until a brain biopsy could be obtained. The day of the planned biopsy, the patient had worsening mental status with blown pupils. A head CT scan showed uncal and tonsillar herniation. The patient was given 1000 mg of methylprednisolone, with no improvement. Osmotics were started but were withdrawn due to elevated sodium levels. The patient underwent several tests and was declared brain dead on January 7. ---- At autopsy the unfixed brain weighed 1460 grams, widespread intravascular lymphoma (immuno-phenotypically consistent with an NK/T-cell subtype) was found with prominent occlusive vasculopathy of leptomeningeal, and intraparenchymal vessels with associated micro-infarcts and acute hypoxic-ischemic insults.
