An elderly hereditary hemorrhagic telangiectasia female with portosystemic encephalopathy

• Published in: Rinsho Shinkeigaku Vol. 49; no. 5; pp. 271 - 274
• Main Authors: Fukunaga, Mami, Fujiki, Ryosuke, Santa, Yo, Kato, Akira, Yoshimura, Takeo
• Format: Journal Article
• Language: Japanese
• Published: Japan Societas Neurologica Japonica 01.05.2009
• Subjects: Aged; Amino Acids, Branched-Chain - therapeutic use; Diet, Protein-Restricted; Electroencephalography; Female; Hepatic Encephalopathy - diagnosis; Hepatic Encephalopathy - etiology; Hepatic Encephalopathy - therapy; hepatic vascular malformations; hereditary hemorrhagic telangiectasia (HHT); Humans; Magnetic Resonance Imaging; portosystemic encephalopathy; Sugar Alcohols - therapeutic use; Telangiectasia, Hereditary Hemorrhagic - complications
• ISSN: 0009-918X; 1882-0654
• DOI: 10.5692/clinicalneurol.49.271

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.