MEN1 syndrome presents as Cushing’s disease with proximal lower limb weakness: A rare phenomenon

Abstract

Abstract The typical presentation of MEN 1 syndrome in most cases is primary hyperparathyroidism. The manifestation of hypercortisolism due to a functional Pituitary microadenoma in an adult as the first presenting feature in MEN 1 is rare. This report of Cushing’s disease presenting as proximal muscle weakness due to an adrenocorticotrophic hormone (ACTH) producing pituitary microadenoma as the initial feature of multiple endocrine neoplasia type 1 is an unusual occurrence. The patient had presented with proximal muscle weakness of lower limbs along with abdominal striae and uncontrolled diabetes. On hematological and radiological evaluations, she was detected to have a pituitary microadenoma along with a parathyroid adenoma causing increased levels of ACTH, serum (S) cortisol, parathyroid hormone, and S calcium. The patient underwent a transsphenoidal decompression of the pituitary microadenoma using frameless neuronavigation. Within 2 weeks of surgery, the patient achieved normal levels of S cortisol. She is off medication for blood sugar control. At 6 months, follow-up, she is symptom-free.