Cognitive processing in completely paralyzed patients with amyotrophic lateral sclerosis

Cognitive functions have never been studied before in completely paralyzed patients, probably due to the lack of the appropriate method. In this study, three male patients in the last stage of amyotrophic lateral sclerosis (ALS) are examined. They were totally locked-in, i.e., incapable of any voluntary response including eye movements. Given this complete lack of motor expression, the method of event-related brain potentials (ERPs) was used. The ERP recording was carried out at the patients' bedside. In one patient indications of preserved cognitive abilities including adequate language comprehension were obtained. The data of the second patient also demonstrate a high degree of intactness of complex cognitive functions, but some ERP phenomena were consistently abnormal, which may be interpreted as a consequence of disease-related degenerative processes. In the third patient no electrocortical response which might indicate higher cortical processing was found. This first ERP study of totally paralyzed patients shows that some of them can possess high, perhaps even normal, information processing capacity after a long period of severe immobility and artificial ventilation.