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Washington University Experience | VASCULAR | Infarct - (Pseudo) Laminar necrosis | 9A0 Case 9 History
Case 9 History ---- The patient was a 45 year old woman with a history of progressive neurological problems which began with a seizure and later status epilepticus in September of 2005. At that time an outside hospital MRI brain revealed T2 hyperintensities in the bilateral hippocampi. She continued to suffer from seizures as well as problems with memory and psychosis. She was tested for Whipple disease with an esophagogastroduodenoscopy and small bowel biopsy that were negative both by staining and PCR for the Whipple's organism. A repeat lumbar puncture on November 16, 2006 found markedly elevated protein of 216, mild pleocytosis of 8 nucleated cells, low normal glucose of 49 (serum glucose 89), and xanthochromia attributable to high protein. Infectious etiologies were excluded. Magnetic resonance imaging of the brain in December 2006 showed extensive vasogenic edema throughout the right frontal lobe with areas of petechial hemorrhage, restricted effusion, and enhancement. A cerebral angiogram at the same time showed overall diminished caliber of cerebral vessels but no vessels showed evidence of inflammation, fibrinoid necrosis, embolic material, or involvement by organisms. However, a subset of small and medium vessels showed mural thickening with concomitant luminal narrowing and occlusion. Overall, this pattern was felt to be consistent with a vasculopathy. Given that it was impossible to exclude a partially treated cerebral vasculitis, the patient was started on cyclophosphamide. She was readmitted for altered mental status. A repeat MRI brain revealed interval development of hemorrhage in the right frontal lobe with mildly increased edema, but decreased mass-effect in the right frontal lobe. There were no vegetations in her heart valves, and she was treated for her infection. However, she was soon admitted for left sided focal motor seizures. Brain MRI revealed multiple subacute infarcts involving bilateral thalami and cerebellar hemispheres. She was evaluated by rheumatology who did not think her symptoms were consistent with Hashimoto's encephalopathy. She was initially started on high-dose Solu-Medrol on admission, but she did not improve. Further work-up revealed a copper level of 0.54 and she subsequently underwent repletion with five days of intravenous copper. She also had a hypercoagulable work-up that was positive for lupus anticoagulant. A lumbar puncture was performed for comparison to prior CSF studies and it revealed increased IgG synthesis rate in the cerebrospinal fluid of 27.5, increased IgG index of 0.71, and 5 oligoclonal bands in the cerebrospinal fluid consistent with an autoimmune non-inflammatory encephalopathy. Her ANCA was also positive at 1:160. Since she did not improve on high dose intravenous steroids, she underwent plasma pheresis and her mental status and right lower extremity weakness improved. She was discharged on prednisone and CellCept. Over the next year, she was admitted for multiple rounds of plasmapheresis as well as placement of an IVC filter on 11/2/07 for venous thrombosis of the right superficial femoral vein. She was started on anticoagulation given her DVT and her positive lupus anticoagulant. She was readmitted for status epilepticus. Infectious work-up was negative. Three days later after discharge, she was readmitted for partial status epilepticus, and her urine culture grew vancomycin-resistant Enterococcus. She had two more hospitalizations for status epilepticus in the setting of indwelling line infections and subtherapeutic Dilantin level. Given her multiple infections leading to seizures, she was taken off CellCept. During her last hospitalization, an EEG revealed seizure activity over her left occipital region as opposed to a generalized or right hemispheric focus. She was last discharged from BJH to a skilled nursing facility. She was noted to have progressive hypotension without acute septic symptoms and died soon thereafter.
