Review
Neuropsychological characterization of hepatic encephalopathy

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Introduction

The neuropsychological features of hepatic encephalopathy (HE) were described centuries ago. Hippocrates stated that “those who are mad on account of phlegm are quiet, but those on account of bile are vociferous, vicious, and do not keep quiet” [1]. It can be assumed that Hippocrates was describing a patient with acute liver injury. Patients with fulminant hepatic failure often present with agitation and restlessness. The behaviour of cirrhotics with HE, however, is dominated by significant psychomotor slowing. The first comprehensive study of the neuropsychiatric manifestations of HE was performed by Sherlock et al. in 1954 [2]. The authors described the clinical presentation of 18 patients with liver disease and neurological signs. In all subjects, clouding of consciousness was accompanied with a loss of facial expression and speech disturbances together with a disorder of the motor system represented by asterixis, tremor, increased tendon reflexes, increased muscle tone and ataxic gait. In a subsequent paper the same group [1] vividly described the disturbances of visual perception in patients with HE. They observed visual agnosia, macropsia, distortion and prolongation of the images, spatial disorientation and a predominance of visual hallucinations. Auditory, tactile, olfactory and gustatory hallucinations were noted in rare cases. The patients' moods fluctuated, while varying degrees of personality change could be observed in all patients even in the early stage of the disease.

Section snippets

‘Subclinical’ vs. ‘minimal’ encephalopathy

Since the 1970s, several groups have shown that many patients with cirrhosis who present without clinical signs of encephalopathy do significantly worse in psychometric tests when compared to healthy controls [3], [4], [5], [6], [7], [8], [9], [10]. They were regarded as suffering from ‘latent’ or ‘subclinical’ HE, a term that has been recently replaced by the term ‘minimal HE’ [11], [12]. A common finding of all studies was a worse performance of cirrhotic subjects in tests of psychomotor

A standardized battery to diagnose minimal HE

We decided to standardize and evaluate a ‘paper and pencil’ test battery for the assessment of minimal HE – the psychometric hepatic encephalopathy score (PHES) – that in accordance with the data of Hamster [11], [15] included seven tests: the line tracing test, the serial dotting test, the digit symbol test, NCTs A and B, the digit span test and the cancelling d-test [23]. The NCTs were included considering their widespread use as psychometric tests in cirrhotic patients. The digit span and

Conclusion

The results of this study have led us to recommend a test battery including NCTs A and B, the line drawing test, the serial dotting test and the digit symbol test for the assessment of minimal HE [24]. This battery examines motor speed and accuracy, visual perception, visuo-spatial orientation, visual construction, concentration, attention and to a lesser extent memory. None of the tests is a true classical test of attention. However, a successful performance of all tests requires an unimpaired

Acknowledgements

This paper is dedicated to Hans Schomerus, who died on 25 December 2000, a few days after we had finished this manuscript. All those who met him knew him as an exemplary human being, physician and scientist. We lost a friend and an outstanding personality. We will never forget him.

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