Idiopathic Intracranial Hypertension Associated With Either Primary or Secondary Aldosteronism

Abstract

BackgroundIdiopathic intracranial hypertension (IIH) is a syndrome consisting of headache, visual field defects and papilledema of uncertain etiology. The prospect was raised previously as to an association between aldosteronism and increased intracranial pressure in 2 middle-aged women with IIH and primary aldosteronism (PAL). Since then, 2 additional adults were identified and 2 other cases were reported from the United Kingdom, whereas 6 cases of IIH and secondary aldosteronism (SAL) in children have been reported in the English literature worldwide. MethodsA retrospective analysis of cases from author institutions and published literature comparing clinical features, laboratory findings and therapeutic interventions in these 12 cases. ResultsThe female-to-male ratio was 10:2. The mean age of the PAL patients was 49±3years—all hypertensive, with adrenal pathology in most. The mean age of the SAL patients was 11±2years—mostly normotensive, with 3 having Bartter’s and 2 Gitelman syndromes, and 1 renal congenital hypoplasia. Plasma aldosterone levels were elevated (31±5ng/dL) in PAL and SAL, whereas plasma renin activity was suppressed in PAL. Hypokalemia (3.2±0.2mmol/L), hypomagnesemia (1.6±0.3mg/dL) and a putative metabolic alkalosis (serum HCO3 30±1mmol/L) were observed. IIH symptoms were controlled by spironolactone in 5, amiloride in 1, correction of hypokalemia and hypomagnesemia in 2, discontinuation of nonsteroidal antiinflammatory drugs in 2, and reduction of body weight in 1. One patient required serial lumbar punctures, 2 a ventriculoperitoneal shunt, whereas all 3 patients with adrenal adenoma underwent surgical resection. ConclusionsAn association between IIH and PAL occurs in hypertensive middle-aged women, whereas normotensive girls having an inherited renal tubular defect may have IIH with SAL. Patients with IIH should be evaluated for aldosteronism and considered for spironolactone therapy.