Abstract

Leukoencephalopathy with severe hypertension is a recently described entity in nephrology, with only a few case reports to date in children. We prospectively studied 18 children with severe hypertension to evaluate the clinical features, severity, reversibility, and prognosis. All were subjected to clinical and biochemical tests, magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA). Headache was reported in 16 children, 13 had confusion and drowsiness, 12 had nausea and vomiting, and 9 had visual disturbances, seizure, and dyspnea. Only 2 had focal neurological deficit (1 with right facial palsy and another with right lateral rectus palsy). Of these 18 children, 14 patients had hypertensive retinopathy and 4 had normal fundus. MRI revealed leukoencephalopathic changes in 16 of 18 patients. These changes were bilateral occipito-parietal in 9 patients, diffuse white/gray matter lesion in 2, brain stem hyperintensity in 2, and hemorrhagic lesion in 3. On MRA, 11 of 18 patients had attenuation of cerebral arteries of different degree. On follow-up, MRI findings resolved in all except 3 patients and all patients had normal MRA, except for 1 with persistent minimal attenuation and another with spasm in all vessels. We conclude that leukoencephalopathy with severe hypertension is reversible both clinically and radiologically in the majority of children after the control of hypertension. However, a few patients may have residual damage and may need psychometric analysis and follow-up for neurodevelopmental sequelae.

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