Endocrine Abstracts

Background: Idiopathic intracranial hypertension (IIH) can rarely be associated with underlying endocrine disorders such as Cushing’s syndrome, hyperthyroidism or with administration of thyroxin or growth hormone. We describe a case of acute adrenal deficiency presented along with IIH.

Case history: A 24-year-old Caucasian female (body mass index 21 kg/m²) presented with nausea, vomiting and collapse. She had several months of generalised fatigue and intermittent headaches but denied taking any regular medication. She appeared drowsy and unwell. Her blood pressure was 103/56 mm of Hg. Neurological and systemic examination was unremarkable other than generalised hyperpigmentation, facial melasma and marked pigmentation of a recent scar over her left shoulder. Biochemical analysis revealed sodium 127 mmol/l, potassium 3.2 mmol/l, glucose 4.1 mmol/l and CRP <5, with normal renal and liver functions. Computerised tomography (CT) and magnetic resonance (MR) of the brain were normal. A lumbar puncture done to rule out meningo-encephalitis showed normal cerebrospinal fluid (CSF) but increased opening pressure (40 mm of water) suggested IIH. Visual acuity and visual field (goldman perimetry) testing were unremarkable. A flat short synacthen (250 mcg) test (basal cortisol, 231 nmol/l,30 min cortisol, 265 nmol/l) and a raised plasma adrenocorticotrophic hormone (ACTH), >278 pmol/l were consistent with primary adrenal failure. Adrenal antibodies were negative. Adrenal insufficiency was treated with hydrocortisone 10 mg bd and fludrocortisone 50 mcg while acetazolamide 250 mg daily was also commenced to treat IHH and prevent visual loss. A rapid clinical improvement and reduction in CSF pressure were noted.

Conclusion: Addison’s disease can remain unrecognised until precipitated by an acute stress. This case suggests that IIH can rarely be associated with Addison’s disease and present as acute illness. IIH is possibly secondary to increased serum and CSF arginine vasopressin peptide (AVP) in glucocorticoid deficiency.