Marianne TİNKLER, Eleanore LYONS, Emily HENDERSON

An unusual cause of confusion hepatic encephalopathy in hereditary haemorrhagic telangectasia

Bu olgu sunumunda konfüzyon nedeni ile başvuran herediter hemorajik ensefalopatili 73 yaşında yaşlı bir kadın sunulmuştur. Daha önceden 3 ay süren kendi kendine sınırlanan atakları vardı. Hastanın bilinen hepatik arterio-venöz malformasyonları vardı. Konfüzyon nedeni olarak idrar yolu enfeksiyonu düşünüldü. Antibiyotik tedavisine rağmen GCS kötüleşti ve hepatik asteriksis gelişti. Hepatik ensefalopati EEG ile tespit edildi ve amonyak düzeyi yüksekti (211umol/L). Laksatif tedavisi geçici düzelme sağladı, fakat hasta AV embolizasyon ve transplantasyon için uygun değildi. Hepatik ensefalopati herediter hemorajik ensefalopa- tinin nadir bir komplikasyonudur.

Konfüzyonun nadir bir nedeni: Herediter hemorajik telenjiektazide hepatik ensefalopati

This case report describes a 73 year old lady with a known Hereditary Haemorrhagic Encephalopathy (HHT) who presented with confusion. She had several previous self-limiting episodes over 3 months. She had known hepatic arterio-venous (AV) malforma- tions. A urinary tract infection was detected and thought to be the cause of her confusion. However despite targeted antibiotic treatment her neurological state worsened (GCS 10/15) and she developed hepatic asterixis. Hepatic encephalopathy was con- firmed with diagnostic EEG and elevated ammonia 211umol/l (

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