1995; Abrahams et al. 2000). On the contrary, other studies interpreted difficulties in comprehension as an evidence of aphasia (Doran et

al. 1995). According to Tsermentseli et al. (2012), however, there is currently insufficient evidence to support the idea that language deterioration is related to executive dysfunction rather than to an aphasic syndrome. Emotional processing and social cognition have also been investigated in ALS. Papps et al. (2005) found a failure to show the normative pattern of enhanced recognition memory for emotional words in ALS patients. In addition, one study showed that in early stages Inhibitors,research,lifescience,medical of the disease emotional responses of ALS patients tend to be altered toward positive valence and toward a more balanced arousal state: they express more positive verbal emotional judgements and rate exciting pictures as less arousing and exciting than controls (Lule Inhibitors,research,lifescience,medical et al. 2005). In a group of nondemented ALS patients Girardi et al. Inhibitors,research,lifescience,medical have found

a deficit of Theory of Mind, that is, an impairment in inferring the mental state of another on the basis of a simple social cue, that is over and above the presence of executive dysfunctions and suggests a profile of cognitive and behavioral dysfunction indicative of a subclinical FTD syndrome. Behavior Inhibitors,research,lifescience,medical impairment is now recognized as another typical feature of ALS and cognitive and behavioral impairments can coexist in approximately 25% of ALS patients (Newsom-Davis et al. 1999; Murphy et al. 2007). Up to 63% of patients are apathetic, irritable, inflexible, restless, and disinhibited (Lomen-Hoerth et al. 2003; Murphy et al. 2007; Phukan et al. 2007). Emotional lability, that is, the patho-logical occurrence Inhibitors,research,lifescience,medical of sudden episode of laughing or crying, has been estimated in 10–20% of ALS patients (Newsom-Davis et al. 1999). However, those episodes are not necessarily in line with the emotional state of the patient.

The prevalence of ALSbi, that is, behavioral impairment that does not meet diagnostic criteria for FTD, varies depending on methodology and diagnostic criteria. One feature which is consistent across many studies is the presence of apathy. Grossman et al. (2007) used the FrSBe (Frontal System Behavioral Scale) to assess changes in apathy, medroxyprogesterone disinhibition, and executive dysfunction in ALS patients; results showed a high incidence of behavioral changes, particularly regarding apathy (55%), and emphasized the usefulness of the scale for detecting behavioral functioning in these patients. Bulbar-onset disease was significantly related to apathy Mdm2 inhibitor screening library ratings indicating that patients with bulbar-onset ALS are more likely to develop behavioral symptoms than those with limb-onset disease.