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• Vol.56 No.9
• CLINICA NEUROL, 56: 622−626, 2016
• Vol.56 No.9 contents

Case Report

A case of posterior reversible leukoencephalopathy syndrome caused by fibromuscular dysplasia

Fumi Kobayashi, M.D.¹⁾, Hidetaka Kato, M.D.¹⁾, Miki Suzuki, M.D., Ph.D.¹⁾, Ryosuke Usui, M.D., Ph.D.²⁾, Minako Koike, M.D., Ph.D.²⁾ and Takashi Ohashi, M.D., Ph.D.¹⁾

¹⁾Division of Neurology, Department of Internal Medicine, Tokyo Women's University Yachiyo Medical Center
²⁾Division of Nephrology, Department of Internal Medicine, Tokyo Women's University Yachiyo Medical Center

A 23-year-old woman presented with disturbance of consciousness and seizure. Her blood pressure was remarkably high, and brain magnetic resonance imaging (MRI) showed high-intensity T₂ signals in the bilateral basal ganglia, corpus callosum, cerebral white matter, and cortex. With the administration of angiotensin II receptor blocker, the symptoms and MRI findings improved, along with normalization of blood pressure, and a diagnosis of posterior reversible leukoencephalopathy syndrome (PRES) was made. Plasma renin activity was high, and the right kidney was severely atrophic. Results from renal and adrenal vein sampling revealed renal vascular hypertension derived from the right renal artery stenosis. The right kidney was then removed by laparoscopic nephrectomy. Pathological examination of the kidney confirmed the diagnosis of fibromuscular dysplasia (FMD). In juvenile-onset encephalitis/encephalopathy, PRES due to FMD should be included in the differential diagnosis.
Full Text of this Article in Japanese PDF (1088K)

(CLINICA NEUROL, 56: 622−626, 2016)
key words: posterior reversible leukoencephalopathy syndrome, fibromuscular dysplasia, renal vascular hypertension

(Received: 10-May-16)