Neurologic complications of multiple endocrine syndromes

Abstract

Multiple endocrine syndromes are unusual familial disorders affecting multiple endocrine glands; they result from an autoimmune process, causing progressive inflammatory destruction of glandular tissue, leading to hormonal insufficiency. Neurologic manifestations result from endocrine failure and/or independent autoimmune neurologic disorders. Multiple endocrine neoplasia (MEN) results from oncogene mutations in cells derived from the neural crest with proliferation of neuroectodermal cells within endocrine glands and skin. Although most MEN-associated tumors are histologically benign, medullary carcinoma of the thyroid is invasive and potentially lethal. Neurologic symptoms may be caused by increased hormonal production and by the presence of other peripheral nervous system (PNS) and central nervous system (CNS) tumors.

Keywords: Polyendocrine autoimmune syndrome; autoimmune Addison’s disease; autoimmune hypothyroidism; bilateral pheochromocytoma; hypoparathyroidism; medullary carcinoma of the thyroid; multiple endocrine neoplasms.