Nelson syndrome
Overview
Nelson syndrome refers to a spectrum of symptoms and signs arising from an adrenocorticotropin (ACTH)–secreting pituitary macroadenoma after a therapeutic bilateral adrenalectomy. The spectrum of clinical features observed relates to the local effects of the tumor on surrounding structures, the secondary loss of other pituitary hormones, and the effects of the high alpha-melanocyte–stimulating hormone (a-MSH), another derivative of proopiomelanocortin (POMC, the precursor peptide from which ACTH is derived) on the skin. The first case was reported by Nelson et al in 1958.
Symptoms
Increased adrenocorticotropic hormone production Increased melanocyte stimulating hormone Muscle weakness Increased skin pigmentation Enlargement of pituitary adenoma Vision loss Pituitary failure Headache Cessation of menstrual periods
Causes
Headache Increased skin pigmentation Muscle weakness Vision loss
Diagnosis
The clinical history and skin pigmentation findings are usually obvious. Hormonal tests generally show elevated ACTH levels in the blood but low or normal levels of cortisol. Magnetic Resonance Imaging (MRI) or computed tomography (CT) scan is important for the identification of a pituitary adenoma.
Treatment
Transsphenoidal surgery is the primary option but not always effective. Radiotherapy, preferably with stereotactic radiation, controls tumor growth in the majority of patients with residual tumor growth after surgery. The Neuro-ICU cares for patients with all types of neurosurgical and neurological injuries, including stroke, brain hemorrhage, trauma and tumors. We work in close cooperation with your surgeon or medical doctor with whom you have had initial contact. Together with the surgeon or medical doctor, the NeuroICU attending physician and team members direct your family member's care while in the ICU. The NeuroICU team consists of the bedside nurses, nurse practioners, physicians in specialty training (Fellows) and attending physicians.