A progressive breakdown of the body in space.

A 74 year-old woman (MD), free of previous neurological history, presented with difficulty in handling cutlery, clothes, writing with what was initially described as an atypical apraxia in acts related to space. Initial neurological evaluation revealed mixed, asymmetric pyramidal, and extrapyramidal semiology. Νeuropsychological testing revealed dressing and constructional deficits, ideomotor apraxia and signs of executive dysfunction in absence of memory, language, and visual perception pathology. The final diagnosis was that of a corticobasal degeneration, where the rare occurrence of a progressively emerging syndrome of self-management loss within peripersonal space is observed.

The dichotomous view on IFG lesion and non-fluent aphasia.

A lesion in inferior frontal gyrus (IFG) is traditionally considered to be crucial for the occurrence of non-fluent aphasia. However, recent studies question the axiomatic causality between a lesion in this area and the expected non-fluent aphasic syndrome. The aim of the present study is to investigate the relationship between IFG lesions and non-fluent aphasia. To address this question, we present radiological and neuropsychological data of 49 chronic aphasic patients. Lesions were identified based on CT and/or MRI scans. Aphasia was assessed using the Boston Diagnostic Aphasia Examination-short form. Analysis indicated a statistically significant association between IFG lesion and non-fluent aphasic disturbances. Nevertheless, a large proportion of our patients did not confirm the traditional prediction, namely that non-fluent patients' lesions would include the inferior frontal gyrus and the opposite would be true for fluent patients. Our results stress the importance of taking into account the size of particular estimates when conducting group analyses. We also argue in favor of examining individual data in clinical practice, and further suggest that the traditional lesion to syndrome correspondence seems to be oversimplified and should be thoroughly revisited.

Big 5 personality changes in Greek bvFTD, AD, and MCI patients.

Patients with neurodegenerative disease show distinct patterns of personality change, some of which may be traced to focal neurological damage, whereas others may be mediated by cultural reactions to functional impairment. Although such changes are early and pervasive in behavioral variant frontotemporal dementia (bvFTD), and milder changes are seen in Alzheimer disease (AD), no study has examined all Big 5 factors of personality in mild cognitive impairment (MCI) patients. In addition, the influence of culture and ethnicity on disease-related personality changes has seldom been examined. Premorbid and current personality were measured in 47 Greek patients with bvFTD, AD, and MCI on the basis of informant reports using the Traits Personality Questionnaire 5, a 5-factor inventory in the Greek language that accounts for Greek cultural factors. bvFTDs showed greater decreases in conscientiousness compared with ADs and MCIs. ADs and MCIs showed increased neuroticism, whereas the bvFTD patients were rated as having become much less neurotic in the course of their disease. The pattern of personality change in MCIs was very similar to that of ADs, supporting recent evidence that personality changes occur as early as the MCI disease stage. In all the groups, personality changes were similar to those previously described in non-Mediterranean cultures, supporting the hypothesis that they may result directly from disease-specific neurological processes.

The Assessment of the Socioemotional Disorder in Neurodegenerative Diseases with the Revised Self-Monitoring Scale (RSMS).

BACKGROUND: Social cognition helps people to understand their own and others' behavior and to modulate the way of thinking and acting in different social situations. Rapid and accurate diagnoses of neurodegenerative diseases are essential, as social cognition is affected by these diseases. The Revised Self-Monitoring Scale (RSMS) is a scale that detects social-emotional cognition deficits. AIM: The aim of the current study is to examine how socioemotional parameters are affected by neurodegenerative diseases and whether the RSMS can discern these disorders based on the socioemotional parameters in the Greek population. METHODS/DESIGN: A total of 331 dementia subjects were included. Mini Mental State Examination (MMSE) and Addenbrooke's Cognitive Examination (Revised, ACE-R) measurements were used in order to assess the cognitive deficits. The Neuropsychiatric Inventory (NPI) was used for the evaluation of the neuropsychiatric symptoms. The RSMS and its two subscales was used in order to detect the socioemotional deficits. RESULTS: The RSMS and its two subscales (RSMS_EX and RSMS_SP) can effectively detect neurodegenerative diseases. The RSMS can detect bvFTD in Alzheimer's Disease (AD), AD in a healthy cohort, behavioral variant Frontotemporal Dementia (bvFTD) in a healthy cohort, bvFTD in Parkinson's Disease (PD) and Frontotemporal Semantic Dementia (FTD/SD) in a healthy cohort. It is a useful tool in order to detect frontotemporal dementias. RSMS correlated negatively with the NPI questionnaire total and the subcategories of apathy, disinhibition and eating disorders. The RSMS results are associated with the ACE-R score (specifically verbal fluency). CONCLUSIONS: The RSMS is a helpful tool in order to identify socioemotional deficits in neurodegenerative dementias. It is also a useful scale that can discern bvFTD and svPPA in AD patients. A worse RSMS score correlates with a worse ACE-R and NPI. It seems to be a useful scale that can reliably measure social behavior in non-reversible neurodegenerative disorders, such as AD, FTD (bvFTD, svPPA), PDD and PD. The results also apply to the Greek population.