Stroke disclosing primary aldosteronism: Report on three cases and review of the literature.

OBJECTIVES: There is a growing evidence of increased risk of cerebrovascular events in primary aldosteronism (PA). Nevertheless, acute neurologic ailment as presenting feature of PA is uncommon. Our aim is to highlight the diagnosis challenges in stroke unmasking PA and to discuss the underlying physiopathology and management dilemmas. MATERIALS AND METHODS: We hereby describe three consecutive rare cases of stroke revealing PA. All patients had brain imaging and thorough biological and morphological assessment to rule out other etiologies of stroke. The diagnosis of primary aldosteronism was established according to the Endocrine Society Clinical Practice Guideline, with a review of the literature on the spectrum of neurologic manifestations in PA. RESULTS: We report on three cases, two women and a man, presenting with ischemic or hemorrhagic stroke, of early onset in two of them. All of the reported patients had hypertension and hypokaliemia. This association prompted the assessment of renin angiotensin aldosterone system (RAAS) disclosing PA, which was due to bilateral adenomas in the first one or bilateral adrenal hyperplasia in the two others. All patients refused the surgical option and received spironolactone with recurrence of stroke in one of them due to treatment incompliance. CONCLUSION: Although cerebrovascular events are quite common in PA, their occurrence as initial feature can be misleading. The association of hypokaliemia and refractory hypertension in ischemic or hemorrhagic strokes should prompt an assessment of the RAAS to rule out PA and initiate adequate management as soon as possible in order to avoid further complications.

Recurrent status epilepticus in posterior reversible encephalopathy syndrome as initial feature of pediatric lupus: A newly diagnosed case and literature review.

INTRODUCTION: Posterior reversible encephalopathy syndrome (PRES) is a recently described clinico-neuroradiological syndrome with several predisposing conditions. Systemic lupus erythematosus (SLE), beginning in 15-20% in childhood, is considered as a potential underlying etiology of PRES. In children, status epilepticus (SE) rarely complicates PRES, and exceptionally occurs in SLE. METHODS: We report on an illustrative case of PRES complicating pediatric lupus revealed by recurrent SE, and we further review through a Pubmed search the previously reported cases of pediatric SLE, PRES and SE. RESULTS: We describe the case of a 12-year old girl who presented with recurrent status epilepticus associated to high blood pressure and renal involvement. Brain imaging showed classical aspects of PRES. Immunological tests including antinuclear, anti-DNA, and anticardiolipin antibodies were positive. The diagnosis of SLE was established. The Pubmed search identified a total number of 9 children with SE in SLE, and 26 with PRES, including our patient. CONCLUSIONS: We discussed the clinical and paraclinical features of PRES in SLE with epilepsy, their underlying pathophysiological aspects, and their management challenges. PRES should be considered in initial recurrent SE in children, justifying a battery of tests comprising immunological testing. Anticardiolipin antibodies seem to play a crucial role in epilepsy, PRES and renal involvement in pediatric SLE. Further studies are needed to clarify whether PRES should be considered one of the neuropsychiatric manifestations of SLE or a consequence of active disease in other organ systems or its treatment.