Congenital extrahepatic portosystemic shunt with progressive myelopathy and encephalopathy.
We report an unusual case of type 2 CEPS in a 29-year-old woman who presented with progressive myelopathy and fluctuating encephalopathy on a background of congenital cardiac disease. Investigations showed hyperammonaemia, and despite no evidence of portal hypertension on ultrasound imaging, CT scan of abdomen showed a shunt between the mesenteric and left internal iliac veins. Patients with unexplained fluctuating or progressive neuropsychiatric symptoms should have their serum ammonia checked. A raised serum ammonia concentration without known portal hypertension should prompt further investigations for extrahepatic shunts.
PMID: 31048365 [PubMed - as supplied by publisher]
Source: Practical Neurology - Category: Neurology Authors: Lux D, Naito A, Harikrishnan S Tags: Pract Neurol Source Type: research
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