Amyotrophic lateral sclerosis (ALS), a progressive motor neuron disease, affects movement and communication abilities and emotional processing. Subjective ratings of emotional stimuli depicting social interactions and facial expressions differed significantly between ALS patients and healthy controls in a previous study with a reduction of negative emotional valence (pleasantness) and lower subjective arousal (excitement) in ALS patients. In the present study, sixty similar emotional slides were presented to 13 ALS patients, 15 matched healthy controls and six tetraplegic patients. Subjective reports of valence and arousal as well as brain responses to the affective pictures using functional magnetic resonance imaging (fMRI) were measured. The picture series was presented twice with a 6-months interval to investigate effects of disease progression. ALS patients presented an increased brain response in the right supramarginal area and a reduced brain response in extrastriate visual areas at both measurements compared with healthy controls. Within the ALS patients' group a reduction of brain responses in the anterior insula at the follow-up was correlated with the subjective arousal. The reduced response in the anterior insula is tentatively interpreted as indicating reduced arousal during the course of the disease at the neural and behavioural level. The reduction of activity in extrastriate visual areas might be similarly interpreted. The increased brain response in the right supramarginal area of ALS patients might represent an altered sensitivity to social-emotional cues.

Amyotrophic lateral sclerosis (ALS) is a fatal disease, leaving the patient in a partially or completely deafferented state. In an explorative study, we investigated responses to visual socio-emotional stimuli in ALS patients. Pictures from the International Affective Picture System (IAPS) were verbally judged by 12 moderately affected ALS patients with a spinal onset and a slow progression and 18 age-matched controls, and data were compared with psychophysiological responses. Verbal emotional judgments of patients were more positive than ratings of controls. Regarding arousal, patients neutralized extreme pictures, in that they rated calm pictures as more exciting than controls and exciting pictures as more calm. These changes of emotional processing were unrelated to depression or frontal lobe dysfunction. There were no major differences between patients and controls concerning physiological responses to emotional stimuli. We conclude that emotional responses of ALS patients tend to be altered towards positive valence and towards a more balanced arousal state in early stages of the disease. These findings contradict assumptions of a generally negative impact of the disease on the emotional disposition and may indicate compensatory cognitive or neuroplastic changes.

The findings suggest an ongoing compensatory process within the higher order motor-processing system of amyotrophic lateral sclerosis patients, probably to overcome loss of function in primary motor and motor imagery-specific networks. The increased activity in precentral and frontoparietal networks in motor imagery might be used to control brain-computer interfaces to drive communication and limb prosthetic devices in patients with loss of motor control such as severely disabled amyotrophic lateral sclerosis patients in a locked-in-like state.

Multiparametric MRI suggests a progressive functional deficit in secondary/higher order sensory processing areas in ALS, probably associated with reduction of re-afferent information flow due to progressive immobility. The changes described might also represent an expression of the disease process itself. Evidence for compensatory processes in multimodal associative cortices was found.

Smooth pursuit and saccadic eye movements of schizophrenic patients were examined. In a pendulum (0.5 Hz) tracking task schizophrenic inpatients had a slightly lower smooth pursuit gain than outpatients and controls, who showed no significant differences. The number of saccades, counter-saccades and velocity arrests occurring in a 20 s tracking epoque was the same in patients and controls, but patients made larger saccades. When tracking a stepping target by saccadic eye movements, schizophrenic inpatients, and to a lesser extent outpatients, exhibited longer reaction times than controls and had a higher incidence of "non-fixation" (saccades away from the target while the target is stationary). Schizophrenic patients also showed a significantly larger proportion of dysmetric saccades (undershooting the target). While similar changes of reaction time and non-fixation score were observed in manic-depressives and alcoholics, dysmetria was more often found in schizophrenics and possibly constitutes the expression of a specific impairment of attention.