Abstract
THE CLINICAL diagnosis of hepatolenticular degeneration is made on the basis of the evaluation of the motor phenomena, the evidence of hepatic dysfunction and the presence of the ring of a pigment at the margin of the cornea. The classic descriptions of Wilson,1Westphal2and Strumpell3and the numerous subsequent reports make it possible to delimit this disease from other pathologic entities in the majority of cases. However, a detailed analysis of the dyskinetic symptoms is still wanting and the importance of liver function tests for diagnostic consideration is still under discussion. The specificity of the clinical picture, as well as that of the pathologic changes in the brain stem, needs to be stressed again in the light of recent observations on nervous disorders associated with various hepatic diseases in man and in animals. A comparison of these conditions with hepatolenticular degeneration can be established only after
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