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Portal–systemic encephalopathy in a non-cirrhotic patient

K Fukushima¹, M Kurozumi², M Kadoya², S Ikeda¹

¹ Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan
² Department of Radiology, Shinshu University School of Medicine, Matsumoto, Japan

Correspondence to:
fuku{at}hsp.md.shinshu-u.ac.jp

We report a rare case of non-cirrhotic intrahepatic portal–systemic venous shunt. The patient presented with repeated neuropsychiatric symptoms. Our case reminds us that such portal–systemic encephalopathy should be considered even in cases without any chronic liver diseases, as a differential diagnosis of non-specific neuropsychiatric symptoms.

A 77-year-old woman was transferred to our hospital with repeated episodes of consciousness disturbance. She had no history of liver disease, surgery, alcoholism, drug abuse or trauma. For a few years, she had shown episodes of altered consciousness that occurred following constipation. Five days before transfer to our hospital, she was admitted to a local hospital because of disorientation, abnormal behaviour and hand tremor. During the episode, EEG showed the frequent triphasic waves (fig 1A).

View this figure (33K): in this window | in a new window | PowerPoint for Teaching   Figure 1 EEG showing triphasic waves (A). T1 axial scan (B) showing signal change in the globus pallidus (arrowheads).

 

On admission, her symptoms had already improved, and examination did not show any abnormalities. Findings on blood tests, including liver enzymes, were unremarkable, and were negative for hepatitis B and C virus, antinuclear antibody and antimitochondria M2 antibody. However, her blood ammonia level was increased (159 µg/dl; normal 30–80), and T1 weighted brain MRI showed high signal intensity in the globus pallidus (fig 1B). Abdominal Doppler sonography showed dilatation of the right portal vein (anterior superior branch) and middle hepatic vein. Both vessels were directly connected via an abnormal knob-like vessel, which presented with shunt blood flow (fig 2A). Portal–systemic encephalopathy caused by intrahepatic portal–systemic venous shunt (IPSVS) was diagnosed. Three dimensional CT angiography (fig 2B) confirmed the diagnosis. The shunt vessel was successfully embolised by catheter intervention using coils, and her blood ammonia level normalised. The patient has remained in good health with no residual neuropsychiatric symptoms.

View this figure (108K): in this window | in a new window | PowerPoint for Teaching   Figure 2 Abdominal Doppler sonography (A) and three dimensions CT angiography (B). Right portal vein (RPV), anterior superior branch of the RPV (P8) and middle hepatic vein (MHV) were dilated. P8 and MHV were connected by an abnormal shunt vessel (*).

 

IPSVS is most often induced subsequent to portal hypertension as a result of liver cirrhosis, causing hepatic encephalopathy (portal–systemic encephalopathy).¹ IPSVS in patients without chronic liver disease is rare, and the pathogenesis of this disease remains unclear.^{1 2} One explanation is the persistence of portal–hepatic venous systems during embryonic development.³ Aging, high protein diet and constipation leads to hepatic encephalopathy in such non-cirrhotic IPSVS cases.^{1 4} The patients show repeated episodes of neuropsychiatric symptoms, and are often misdiagnosed as having dementia or psychotic disorders.¹ Our findings indicate that portal–systemic encephalopathy should be considered even in non-cirrhotic cases.

This article has been adapted from Fukushima K, Kurozumi M, Kadoya M, Ikeda S. Portal–systemic encephalopathy in a non-cirrhotic patient Journal of Neurology, Neurosurgery and Psychiatry 2008;79:96

Competing interests: None.

1. Watanabe, A. Portal-systemic encephalopathy in non-cirrhotic patients: classification of clinical types, diagnosis and treatment. J Gastroenterol Hepatol 2000;15:969–79.[CrossRef][Medline]
2. Pocha, C, & Maliakkal, B. Spontaneous intrahepatic portal-systemic venous shunt in the adult: case report and review of the literature. Dig Dis Sci 2004;49:1201–6.[CrossRef][Medline]
3. Tanoue, S, Kiyosue, H, Komatsu, E, et al. Symptomatic intrahepatic portosystemic venous shunt: embolization with an alternative approach. AJR Am J Roentgenol 2003;181:71–8.[Abstract/Free Full Text]
4. Kerlan, RK, Jr, Sollenberger, RD, Palubinskas, AJ, et al. Portal–systemic encephalopathy due to a congenital portocaval shunt. AJR Am J Roentgenol 1982;139:1013–15.[Free Full Text]

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