A 94–year–old African American female patient admitted to the hospital with altered mental status and slurring of speech. Patient daughter had home blood sugar measured as 22 mg/dl. She had been suffering from recurrent hypoglycemic episodes for the past 2 years with one hospital admission for hypoglycemia 2 years ago. She used to avoid low blood sugar by eating around 8–9 times a day. Patient experienced palpitations, sweating and dizziness during most of the episodes of hypoglycemia. Her sister and daughter had parathyroidectomy for hypercalcemia. She was hypercalcemic, but was not treated. She complained of weight gain 25 lbs within 6 months, constipation and polyuria. Her vitals were normal. Initial labs were serum calcium 8.8 mg/dl, HbA1C 4.9, TSH 0.9 uIU/ml (0.34–5.6), high intact PTH 84 pg/ml (15–65) and low vitamin D level of 26.1 ng/ml (32–100). She was subjected to a monitored fasting and stopped after 3 hours where the blood sugar dropped to 24 mg/dl. Further tests revealed a C–peptide level 6.9 ng/ml (1.1–4.4), proinsulin 12.9 pmol/L (0–10), insulin 75.1 uIU/ml (2.6–24.9), undetectable sulfonylurea level biochemically consistent with diagnosis of insulinoma. CT abdomen with contrast revealed no mass in the pancreas. Prolactin level and MRI brain were normal. Patient refused any surgical intervention and was treated with prednisone, octreotide and D10 drip. Blood sugar later improved, so subsequently D10 drip was stopped, prednisone was tapered and octreotide was continued.
Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant disease with predisposition to tumors of the parathyroid glands, anterior pituitary and pancreatic islet cells. It affects all age groups with a range of 8–81 years and more than 95% of patients develop clinical manifestations by the fifth decade. MEN 1 occurs in approximately 1 in 30,000 individuals with an equal sex distribution. Hyperparathyroidism is the most common manifestation of MEN 1, almost 100% penetrance rate and recurrence rate. Clinically apparent pituitary adenomas have been found in approximately 15–20% of patients with MEN 1 and nearly 10% have insulinomas. Endoscopic ultrasound in association with octreoscan scintigraphy increases the pancreatic tumoral detection rate to 90%. Direct DNA testing for MEN 1 gene mutations is available, but should be done only in patients when diagnosis is unclear. Surgical treatment ranging from enucleation of a single tumor to a distal or partial pancreatectomy is the treatment of choice. Medical treatment consists of frequent carbohydrate feeds, diazoxide, glucocorticoid and octreotide.
Early recognition of affected and at risk individuals using the International Guidelines for Diagnosis and Therapy of the MENs syndromes surveillance program reduces the morbidity and mortality of MEN 1 and provides an opportunity to initiate treatment at early stages. This case to our knowledge represents the oldest patients with MEN 1 reported in the literature.