AKINETIC MUTISM AS A CLASSIFICATION CRITERION FOR THE DIAGNOSIS OF CREUTZFELDT-JAKOB-DISEASE

Objectives-Among the classification criteria for the diagnosis of Creu tzfeldt-Jakob disease, akinetic mutism is described as a symptom which helps to establish the diagnosis as possible or probable. Akinetic mu tism has been anatomically divided into two forms-the mesencephalic fo rm and the frontal form. The aim of this study was to delimit the symp tom of akinetic mutism in patients with Creutzfeldt-Jakob disease from the complex of symptoms of an apallic syndrome and to assign it to th e individual forms. Methods-Between April and December 1996, 25 akinet ic and mute patients with Creutzfeldt-Jakob disease were consecutively examined. The patients were classified according to the definition of akinetic mutism by Cairns and secondly in accordance with the feature s constituting the complete picture of an appalic syndrome (defined by Gerstenbrand). Results-From 25 patients with definite Creutzfeldt-Jak ob disease, 24 patients showed impoverishment of speech and, after a m ean duration of four (range 1.1-11.2) months, almost complete absence of voluntary movements and speech. Seven patients were classified as b eing mute and akinetic and assigned to the mesencephalic form whereas 13 patients were classified as apallic. One patient was mute without b eing akinetic and four patients were comatose. Conclusion-Diffuse brai n damage underlies akinetic mutism in patients with Creutzfeldt-Jakob disease. The term can be used as a classification criterion for the di agnosis of Creutzfeldt-Jakob disease; however, it should be applied ve ry carefully and delimited clearly from the apallic syndrome.