Neuronal correlates of coping in Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a rare degenerative and as of now untreatable disorder of the upper and lower motor neurons marked by progressive paralysis of the whole body (Rowland & Shneider, 2001; Logroscino et al., 2008). Epidemiological data suggest a median survival time of 32 month after symptom onset (del Aguila et al., 2003) with respiratory failure being the main cause of death (Kurian et al., 2009).

Despite its rapid progression and fatal course, patients diagnosed with ALS display comparatively low levels of depression (see Kurt et al., 2007 for a review), report good average mood, and high subjective quality of live (QoL; Chiò et al., 2004; Matuz, 2008).

Whereas classic models of coping (Lazarus, 1999) emphasize task orientation as a means of coping with major life events, the concept of psychological consistency focuses on the regulation of negative emotions associated with a state of inconsistency, defined as a mismatch between one's expecta¬tions and perceptions (Znoj & Grawe, 2000). One of the most far reaching means of the regulation of emotions involves change in schemas controlling the perception of the self and one's relation to others (response shift, Spranger & Schwartz, 1999), thereby allowing adaptation when the stressor itself cannot be changed, as is the case in ALS. To elucidate the regulation of emotions in patients over the course of ALS a psychophysiological approach is proposed.

The event related potential N400 is a negative deflection following the presentation of semantically incongruent contents (Chewilla et al., 1995), shown to occur in word processing (van Petten & Luka, 2006), and, more recently, in the processing of visually presented action sequences (Reid & Striano, 2008, Shibata et al., 2009).

It is proposed that patients who cope successfully, as indicated by low levels of depression and high subjective QoL, gradually change their self-schemas to incorporate their disease, so that verbal contents referring to the disease become semantically congruent to the self-schema. This integration is expected to show as an attenuated N400 response. Once this strategy of modifying one's self-schema to incorporate the disease is adopted it is expected to be maintained as it is negatively rewarded by a reduction in negative feelings of inconsistency.