Ischemic stroke as a presenting feature of VIPoma due to MEN 1 syndrome

Presentation of the ischemic stroke due to vasoactive intestinal peptide producing tumor (VIPoma) or Verner Morrison syndrome is rare. This is first of its kind case which we are reporting here which was later turned out to be multiple endocrine neoplasia type 1 (MEN 1) syndrome with diagnosis of primary hyperparathyroidism in the same patient in follow-up.

A 13-year-old girl presented to our emergency department with features of disorientation, weakness of left sided extremities. She had watery high volume diarrhea and related dehydration with renal failure. Blood chemistry was suggestive of hypokalemia with metabolic acidosis. Patient had flushing on her face during this episode of illness. Magnetic resonance imaging (MRI) of brain suggested venous infarct. Computed tomography (CT) scan of abdomen done with high index of suspicion was suggestive of mass in tail of pancreas mostly a VIPoma. Patient was operated for the tumor after which there was no recurrence of diarrhea. Biopsy of tumor was consistent with VIPoma with chomogranin A positivity. Patient improved of her stroke episode with time. On follow-up she is diagnosed to have primary hyperparathyroidism with hypercalcemia due to left inferior parathyroid adenoma which improved with intravenous (IV) zolindronic acid therapy and now she is planned to undergo parathyroidectomy.

VIPoma is a rare tumor but is well-described with MEN 1. Stroke as a presenting feature of VIPoma is first reported with this case.