Introduction: Cerebral oedema has rarely been reported in adrenal insufficiency. We report a case of decompensated cerebral oedema due to autoimmune adrenalitis.
Case report: A 12-year-old boy presented to hospital with a 1 day history of headache, fever up to 39.9 °C, confusion, diarrhoea, and vomiting. He had a left-sided ptosis, reduced conscious level (Glasgow coma scale 8/15) and was hypertensive (blood pressure 134/90 mmHg). He required intubation and 4 days of mechanical ventilation with inotropic support. A CT scan confirmed the presence of raised intracranial pressure with slit like ventricles. Initial investigations confirmed features of sepsis with hyponatraemia (sodium 128 mmol/l, potassium 5 mmol/l, pH 7.3, BE −8, CRP 305.5 mg/l, WCC 15, and neutrophils 10). The presence of pigmentation with persistent hyponatraemia and catecholamine-refractory shock led to a synacthen test which confirmed adrenal failure (30 min peak cortisol 19 nmol/l), with a modestly elevated ACTH of 63 ng/l (N1050). He was commenced on i.v. hydrocortisone treatment with rapid improvement; this was later switched to oral medication.
Further discussion with the family confirmed a 6-month history of salt craving and progressive skin pigmentation that had been attributed to the summer weather.
There was a strong family history of autoimmunity, with systemic lupus erythematosus in the mother, hypothyroidism in the father, and type 1 diabetes in the paternal step daughter.
Convalescence sampling confirmed autoimmune adrenalitis (adrenal antibody positive and thyroid antibody negative), and further elevation of ACTH (>1250 ng/l). Mild hyponatraemia persisted on hydrocortisone, and an elevated plasma renin (33 nmol/l per h; NR 2.84.5) confirmed mineralocorticoid deficiency. Fludrocortisone was therefore commenced with consequent biochemical and clinical improvement.
Conclusions: Cerebral oedema is a rare life-threatening feature of Addisons disease. Earlier detection of chronic glucocorticoid and mineralocorticoid deficiency in our patient may have prevented the acute decompensation. Addisons disease should be considered in any patient with unexplained cerebral oedema.
12 - 14 Nov 2014
British Society for Paediatric Endocrinology and Diabetes