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2017 Case 11

Case 11

Y. Qi, F. Derakhshan, J. Maguire1

1Division of Neuropathology, Department of Pathology and Laboratory Medicine, Vancouver General Hospital, Vancouver, BC Canada

Clinical Summary:

A 66-year-old woman was found by the police, driving in the dark with no headlights. She was confused and disoriented. She had been missing for about two weeks and was described as being “eccentric” with odd behavior recently. She was admitted to hospital. She was malnourished, and was found to have decreased protein, albumin, calcium, magnesium, and osteoporosis. She had a history of progressive weight loss, diarrhea, confusion, depression, and became withdrawn. She had a past history of weight loss and “non-deficiency anemia” which was not responsive to iron. Sixteen years previously, she had gastric biopsies which showed acute inflammation, and duodenal biopsies that were consistent with “coeliac sprue”. She stated that she had done well on a gluten-free diet and was careful until about 18 months ago, when her symptoms recurred with diarrhea. She was placed on Prednisone (for protein loss) and Azathioprine. Upper endoscopy confirmed monilial esophagitis, treated with Diflucan. A duodenal biopsy was performed, and was described as showing features of “celiac disease”. CT of chest, abdomen, and pelvis showed no evidence of metastatic disease. A lumbar puncture was described as “unhelpful in the diagnosis”. CSF for 14-3-3 was positive. She had stereotactic left frontal biopsies (slides not available). Postoperatively she had hyponatremia and hypokalemia and her delirium settled over time. After consultation with her family, it was decided that her care would be palliative, and she was transferred for hospice care. She died four months after her initial hospitalization, six weeks after her brain biopsies. 

Material submitted: scanned H&E brain slide from autopsy, slide 2, and neuroimaging


Questions: What is the diagnosis? What is the etiology? Is there any treatment?