Osmotic demyelination syndrome in a patient with Noonan syndrome and anterior hypopituitarism. (20th August 2020)

• Record Type: Journal Article
• Authors: Chua, Tzy Harn; Loh, Wann Jia
• Is Part Of: Endocrinology, diabetes & metabolism case reports. (2020)
• Publication Date: 2020-08-20
• Subjects: Adult -- Male -- Asian - other -- Singapore; Pituitary -- Pituitary -- GH -- Thyroxine (T4) -- Triiodothyronine (T3) -- TSH -- Cortisol -- ACTH -- FSH -- LH -- Testosterone -- IGF1 -- Noonan syndrome -- Hypopituitarism -- Hyponatraemia -- Pituitary adenoma -- Microadenoma; Hypopituitarism -- Hyponatraemia -- Dizziness -- Headache -- Insomnia -- Nausea -- Abdominal pain -- Vomiting -- Hypotension -- Kyphoscoliosis -- Gynaecomastia -- Ears - low set -- Neck - short -- Hypogonadism -- Short stature -- Neck - loose skin (nape) -- Hypothyroidism -- Seizures -- Hypotonia -- Aphasia -- Osteoporosis -- Sodium -- MRI -- Blood pressure -- Serum osmolality -- Urine osmolality -- GH -- FT3 -- FT4 -- TSH -- Cortisol -- Cortisol (9am) -- Cortisol (serum) -- ACTH -- FSH -- LH -- Testosterone -- IGF1 -- Bone mineral density -- X-ray -- Chloride -- Haemoglobin -- 25-hydroxyvitamin-D3 -- Sex hormone binding globulin -- Fluid repletion -- Saline -- Hydrocortisone -- Glucocorticoids -- Glucose -- Desmopressin -- Levothyroxine -- Valproic acid; Neurology; Unique/unexpected symptoms or presentations of a disease -- August -- 2020; Endocrinology -- Periodicals; Diabetes -- Periodicals; Diabetes Mellitus; Endocrinology; Diabetes; Endocrinology; Case Reports; Periodicals; Periodicals; 616.4
• DOI: 10.1530/EDM-20-0039
• Languages: English
• ISSNs: 2052-0573

Abstract

Abstract : Summary: Severe hyponatremia and osmotic demyelination syndrome (ODS) are opposite ends of a spectrum of emergency disorders related to sodium concentrations. Management of severe hyponatremia is challenging because of the difficulty in balancing the risk of overcorrection leading to ODS as well as under-correction causing cerebral oedema, particularly in a patient with chronic hypocortisolism and hypothyroidism. We report a case of a patient with Noonan syndrome and untreated anterior hypopituitarism who presented with symptomatic hyponatremia and developed transient ODS. Learning points: Patients with severe anterior hypopituitarism with severe hyponatremia are susceptible to the rapid rise of sodium level with a small amount of fluid and hydrocortisone. These patients with chronic anterior hypopituitarism are at high risk of developing ODS and therefore, care should be taken to avoid a rise of more than 4–6 mmol/L per day. Early recognition and rescue desmopressin and i.v. dextrose 5% fluids to reduce serum sodium concentration may be helpful in treating acute ODS.