Rare Cause of Severe Neurological Impairment in a Young Patient With Lupus

Abstract

Systemic lupus erythematous (SLE) is a chronic autoimmune disease that frequently leads to kidney involvement. Hypertension in lupus nephritis (LN) is common, but malignant hypertension (MH) is a rare condition. MH in LN is associated with severe clinical manifestations and intrarenal vascular lesions. A 19-year-old woman with a history of SLE, class VI LN and MH presented with sudden drop in general status, confusion, diplopia, bradypsychia, and bradylalia. Physical exam showed cold extremities, cyanosis, high blood pressure (260/100 mm Hg) and tachycardia. Neurological exam showed impairment of the cerebellum, left central facial palsy and left-sided hemiparesis. Laboratory work showed thrombocytopenia, hyperuricemia, hypoalbuminemia and an estimated glomerular filtration rate of 66.1 mL/min/1.73 m². A cerebral computed tomography (CT) showed no hemorrhagic lesions, but a diffuse hypodense edematous area in the pons, midbrain and bilateral thalamic nuclei. She was started on antihypertensive therapy, dexamethasone and mannitol. After 5 days in intensive care unit, a brain magnetic resonance angiogram (MRA) showed petechial hemorrhage and swelling in deep thalamic regions, left internal capsule, pons, and midbrain. Nimodipine was added to the regimen. Workup for JC virus and Listeria monocytogenes was negative. Evolution was favorable with complete remission of neurological symptoms. Repeated brain MRA confirmed regression of swelling and hemorrhagic lesions. The diagnosis of hypertensive encephalopathy was sustained by improved clinical evolution under anti-edema measures, antihypertensives and by CT/magnetic resonance imaging (MRI). Differential diagnosis was made with central nervous system vasculitis and infections due to immunosuppression therapy (tuberculosis, HIV, and JC virus). This case describes the evolution of a young woman with lupus and recurrent MH, complicated with neurological manifestations.




J Med Cases. 2016;7(11):488-490
doi: http://dx.doi.org/10.14740/jmc2676w