Rinsho Shinkeigaku (Clinical Neurology)

Brief Clinical Note

An elderly hereditary hemorrhagic telangiectasia female with portosystemic encephalopathy

Mami Fukunaga, M.D.1)3), Ryosuke Fujiki, M.D.1), Yo Santa, M.D.1), Akira Kato, M.D.2) and Takeo Yoshimura, M.D.1)

1)Department of Neurology, Shimonoseki Kousei Hospital
2)Department of Gastroenterology, Shimonoseki Kousei Hospital
3)Department of Neurology, Neurological Institute, Graduate School of Medical Science, Kyushu University

We present a 71-year-old woman with hereditary hemorrhagic telangiectasia (HHT) who at age 69, had undergone total gastrectomy because of repeated upper gastrointestinal bleeding. A day prior to admission she began to demonstrate abnormal behavior. Examination showed she was restless and had higher brain dysfunction. Triphasic waves were seen on EEG, and a high signal in the globus pallidus on T1-weighted MRI. Plasma NH3 level was increased after a meal. Abdominal CT scan showed vascular anomalies including a portohepatic vein shunt. She was diagnosed with portosystemic encephalopathy. After treatment with a low-protein diet, lactitol, and branched chain-amino acids, her clinical condition, plasma NH3 level after a meal, and EEG returned to normal.
Because portosystemic shunt is rare in HHT, there have been few reports of portosystemic encephalopathy with this condition. However, with aging, the possibility of portosystemic encephalopathy increases because of age-related increases in portosystemic shunt volume.
Full Text of this Article in Japanese PDF (365K)

(CLINICA NEUROL, 49: 271|274, 2009)
key words: hereditary hemorrhagic telangiectasia (HHT), portosystemic encephalopathy, hepatic vascular malformations

(Received: 9-Oct-08)