Whipple's disease is a granulomatous infectious disease caused by Tropheryma whipplei. The bacteria accumulate within macrophages preferentially in the intestinal mucosa. Disease manifestation seems to be linked to immunologic abnormalities of macrophages. We describe a patient with cerebral Whipple's disease who presented with mental status changes, confusion, inversed sleep-wake-cycle, bilateral ptosis, and vertical gaze palsy. Endoscopic biopsy sampling for the first time revealed Whipple's disease in the stomach but not in the duodenum. Whole blood stimulation displayed reactivity to T. whipplei that was at the lower end of healthy controls while reactivity of duodenal lymphocytes was not diminished. We propose that in cases of neurological symptoms suspicious for WD with normal duodenal and jenunal findings, biopsy sampling should be extended to gastric mucosa. The robust reactivity of duodenal lymphocytes might have prevented our patient from small bowel disease, whereas the impaired reactivity in peripheral blood lymphocytes might yet explain the bacterial spreading to the CNS leading to the rare case of predominant neurological symptoms without relevant systemic involvement.
- T. whipplei
- central nervous system manifestation
- duodenal lymphocytes
- gastric involvement