Loss of empathy is an early central symptom and diagnostic criterion of the behavioral variant frontotemporal dementia (bvFTD). Although changes in empathy are evident and strongly affect the social functioning of bvFTD patients, few studies have directly investigated this issue by means of experimental paradigms. The current study assessed multiple components of empathy (affective, cognitive and moral) in bvFTD patients. We also explored whether the loss of empathy constitutes a primary deficit of bvFTD or whether it is explained by impairments in executive functions (EF) or other social cognition domains. Thirty-seven bvFTD patients with early/mild stages of the disease and 30 healthy control participants were assessed with a task that involves the perception of intentional and accidental harm. Participants were also evaluated on emotion recognition, theory of mind (ToM), social norms knowledge and several EF domains. BvFTD patients presented deficits in affective, cognitive and moral aspects of empathy. However, empathic concern was the only aspect primarily affected in bvFTD that was neither related nor explained by deficits in EF or other social cognition domains. Deficits in the cognitive and moral aspects of empathy seem to depend on EF, emotion recognition and ToM. Our findings highlight the importance of using tasks depicting real-life social scenarios because of their greater sensitivity in the assessment of bvFTD. Moreover, our results contribute to the understanding of primary and intrinsic empathy deficits of bvFTD and have important theoretical and clinical implications.

Social cognition impairments are pervasive in the frontotemporal dementias (FTD). These deficits would be triggered by (a) basic emotion and face recognition processes as well as by (b) higher level social cognition (e.g., theory of mind, ToM). Both emotional processing and social cognition impairments have been previously reported in the behavioral variant of FTD (bvFTD) and also in other versions of FTDs, including primary progressive aphasia. However, no neuroanatomic comparison between different FTD variants has been performed. We report selective behavioral impairments of face recognition, emotion recognition, and ToM in patients with bvFTD and progressive non-fluent aphasia (PNFA) when compared to controls. Voxel-based morphometry (VBM) shows a classical impairment of mainly orbitofrontal (OFC), anterior cingulate (ACC), insula and lateral temporal cortices in patients. Comparative analysis of regional gray matter related to social cognition deficits (VBM) reveals a differential pattern of fronto-insulo-temporal atrophy in bvFTD and an insulo-temporal involvement in PNFA group. Results suggest that in spite of similar social cognition impairments reported in bvFTD and PNFA, the former represents an inherent ToM affectation whereas in the PNFA these deficits could be related to more basic processes of face and emotion recognition. These results are interpreted in the frame of the fronto-insulo-temporal social context network model (SCNM).

Factors predicting life expectancy in primary pulmonary hypertension have not been well defined. Thirty four cases of primary pulmonary hypertension that had been followed up until death or for at least five years were reviewed retrospectively. Patients were divided into three groups: 18 patients who died within five years of presentation to hospital; 12 who survived more than five years; and four who improved and who lived for more than five years. The age at onset was similar in the three groups and, like symptoms and sex, did not predict life expectancy. Right heart failure during the course of the disease was associated with a poor outcome. Radiographic evidence of cardiac enlargement and evidence of right heart strain on electrocardiogram at presentation was also predictive of survival for less than five years. Pulmonary arteriolar resistance was higher and cardiac output lower in those with the shortest survival times. There was no relation between pulmonary artery pressure and length of survival. Systemic resistance varied directly with pulmonary resistance and served to maintain systemic pressure. Presentation in or after pregnancy and patency of the foramen ovale were associated with longer survival. In four patients there was evidence of regression of the disease by cardiac catheterisation and lung histology. Primary pulmonary hypertension is a heterogeneous condition in which life expectancy varies widely.

ore than a century ago, the Phineas Gage case revealed that frontal lobe lesions can cause personality and social cognition impairment. Since its description, clinical observations 1 have highlighted important similarities between the symptoms of patients with prefrontal lesions (PFL) and patients with the behavioral variant of frontotemporal dementia (bvFTD). Both conditions share symptoms such as distractibility, personality changes, social inappropriateness, and markedly impaired moral judgments. 2,3 However, to our knowledge, no studies have compared PFL and bvFTD regarding any social cognition domain. Social cognition tasks are particularly sensitive in detecting impairment in frontal patients. 4 Moral reasoning is a social cognition domain affected in both conditions. 2,3 The brain areas usually affected in bvFTD 5,6 (the ventromedial prefrontal cortex [VMPC], orbitofrontal cortex, anterior temporal lobes, amygdala, and insula) are involved in moral cognition. 7 Moreover, patients with VMPC damage show abnormal moral judgments of harmful intentions in the absence of harmful outcomes. 3 However, to our knowledge, no studies of bvFTD have previously examined the processing of intentions and outcomes in moral judgment. This work compared the moral judgments of patients with bvFTD and patients with PFL by means of a well-characterized task 3 involving scenarios that disentangle the contributions of intentions and outcomes to moral judgment. Methods Participants All participants provided written informed consent in agreement with the Helsinki declaration. The ethics committee of the Institute of Cognitive Neurology approved this study. Eight patients with unilateral chronic cerebrovascular lesions confined to frontal structures were recruited. All patients were assessed at least 6 months after the lesion and none of them had aphasia or motor difficulties. Nineteen patients fulfilled the revised criteria for probable bvFTD. 8 All patients underwent neurological, neuropsychiatric, and neuropsychological examinations and were in the early or mild stages of the disease. Patients with bvFTD and patients with PFL with psychiatric disorders, other neurological diseases, or diffuse brain damage in neuroimaging were excluded. IMPORTANCE Several clinical reports have stated that patients with prefrontal lesions or patients with the behavioral variant of frontotemporal dementia share social cognition impairments. Moral reasoning is impaired in both conditions but there have been few investigations that directly compare this domain in the 2 groups. OBSERVATIONS This work compared the moral judgments of these patient groups using a task designed to disentangle the contributions of intentions and outcomes in moral judgment. For both disorders, patients judged scenarios where the protagonists believed that they would cause harm but did not as being more permissible than the control group. Moreover, patients with frontotemporal dementia judged harmful outcomes in the absence of harmful intentions as less permissible than the co...