An unusual presentation of hypoglycemic distal polyneuropathy caused by an insulinoma in the context of multiple endocrine neoplasia 1

• Autores: Castillo Sayán, Oscar, Medina Sánchez, Cecilia, Verona Rubio, Roger, Machicado Zuñiga, Enrique, Mendoza Pérez, German, Tello Cebreros, Lida
• Formato: artículo
• Fecha de Publicación: 2018
• Institución: Universidad Nacional Mayor de San Marcos
• Repositorio: Revistas - Universidad Nacional Mayor de San Marcos
• Lenguaje: español
• OAI Identifier: oai:ojs.csi.unmsm:article/15637
• Enlace del recurso: https://revistasinvestigacion.unmsm.edu.pe/index.php/anales/article/view/15637
• Nivel de acceso: acceso abierto
• Materia: Neoplasia Endocrina Múltiple tipo 1; Hipoglicemia; Polineuropatía; Insulinoma; Cordoma; Multiple Endocrine Neoplasia Type 1; Hypoglycemia; Polyneuropathies; Chordoma

Multiple endocrine neoplasia type 1 is a rare disease. It is characterized by the neuroendocrine, parathyroid, pituitary, and enteropancreatic tumor involvement. We present the case of a 19 year old patient with symptoms of headache, convulsions and weakness of the four extremities. The presence of hypoglycemia 33mg/dL was confirmed. The electromyographic study showed motor sensory polyneuropathy in all four extremities. The abdominal magnetic resonance showed a tumor in the tail of the pancreas that after the pancreatectomy was confirmed as insulinoma. The glycemia was normalized. In addition, he presented a pituitary macroadenoma, primary hyperparathyroidism and non-functioning adrenal tumor. At 25 years of age, he presented severe headache and amaurosis of the right eye. Axial tomography showed a giant pituitary tumor and in the study of pathology chondroid malignancy (chordoma) was diagnosed.