Infundibuloneurohypophysitis associated with autoimmune thrombocytopenia and chiasmal syndrome: a case report

Acta Neurol Belg. 2019 Sep;119(3):337-342. doi: 10.1007/s13760-018-1007-8. Epub 2018 Aug 20.

Abstract

Lymphocytic hypophysitis is an uncommon autoimmune disease. It may involve the adenohypophysis, neurohypophysis and pituitary stalk. It is most often encountered in pregnant women. Its diagnosis and management pose a significant challenge, as its clinical manifestation and appearance in imaging studies are difficult to distinguish from more common lesion of the sellar region (e.g., pituitary adenomas). We present the case of a 42-year-old man who presented with a chiasmatic syndrome, diabetes insipidus and hypopituitarism. Brain MRI documented a sellar lesion with suprasellar extension and optic chiasm dislocation. He underwent an endoscopic endonasal transsphenoidal resection of the mass. Histopathological examination revealed a lymphocytic hypophysitis. Visual symptoms improved dramatically postoperatively with permanent diabetes insipidus and panhypopituitarism. We discuss the indication for surgical resection in patients with inflammatory lesions extending to the suprasellar region associated with visual impairment.

Keywords: Chiasmal syndrome; Hypophysitis; Pituitary; Transsphenoidal surgery.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Autoimmune Hypophysitis*
  • Diabetes Insipidus*
  • Humans
  • Hypopituitarism*
  • Male
  • Optic Chiasm / pathology*
  • Purpura, Thrombocytopenic, Idiopathic*
  • Vision Disorders*