Abstract
Acquired hepatocerebral degeneration (AHD) is an extrapyramidal syndrome that develops secondary to hepatic dysfunction. It may accompany advanced liver disease and is related to the presence of portosystemic shunts. We describe a 44-year-old woman who presented with bleeding from gastric fundus varices secondary to splenic vein thrombosis and concomitant development of rapidly progressive extrapyramidal symptoms. No overt liver dysfunction was identified. Brain imaging demonstrated damage to the basal ganglia. The splenic artery was embolized with disappearance of the varices. Medical treatment consisted of amantadine and low-dose clonazepam for 6 months. The patient's neurologic symptoms gradually improved with few sequelae, although imaging demonstrated residual basal ganglia damage. This case highlights the need to identify reversible causes of AHD and treat them rapidly in order to halt progressive damage. The general prognosis of patients with AHD remains poor, and flow interruption in portosystemic shunts must always be taken into account. Rapid diagnosis and treatment could ameliorate the poor prognosis.
Highlights
Neurologic complications of hepatic failure, most commonly cognitive impairment and extrapyramidal syndrome, are well documented
The toxicity may be attributable to the effect of metabolites that are otherwise detoxified in the liver, since most cases result from either liver failure or portosystemic shunts that bypass the detoxification processes [1]
We report an unusual course of events in a patient with acquired hepatocerebral degeneration (AHD), with a dramatic acute presentation of extrapyramidal syndrome in the presence of splenic vein thrombosis and gastric bleeding which significantly improved over a few weeks
Summary
Neurologic complications of hepatic failure, most commonly cognitive impairment and extrapyramidal syndrome, are well documented. We report an unusual course of events in a patient with AHD, with a dramatic acute presentation of extrapyramidal syndrome in the presence of splenic vein thrombosis and gastric bleeding which significantly improved over a few weeks. This case may shed light on the pathogenesis underlying the disorder. A 44-year-old woman born in Kazakhstan was admitted for evaluation of massive hematemesis and syncope Her past medical history was remarkable for chronic hepatitis C infection at age 28 years that was successfully treated with interferon. On follow-up EGD performed 9 months after splenic artery embolization, there was no evidence of bleeding or gastric varices. The first follow-up MRI study was accompanied by magnetic resonance spectroscopy (MRS; 2D PRESS TR/TE 2000/144ms), which showed low peaks of N-acetyl aspartate (NAA) and creatine compared with normal thalamus tissue (Figure 4)
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