CLINICAL CHARACTERISTICS AND POSSIBLE MECHANISMS UNDERLYING BEHAVIORAL AND PERSONALITY CHANGES IN FTDP-17
Journal Title: Acta Neuropsychologica - Year 2013, Vol 11, Issue 2
Abstract
Although the clinical and neuroanatomical characteristics of behavioral and personality changes have been well-defined in frontotemporal dementia syndromes, relatively little is known about the behavioral and personality changes in frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17). Thus, the aim of this study was to longitudinally characterize the behavioral and personality abnormalities associated with FTDP-17.We examined three patients with a clinical diagnosis of FTDP-17 confirmed by genetic testing. In all three patients the P301L MAPT mutation was detected. All patients underwent neuroimaging (MRI and SPECT). Behavior and personality changes were assessed longitudinally with the Blessed Dementia Rating Scale, the Frontal Behavioral Inventory and/or the Neuropsychiatric Inventory. All three patients demonstrated marked and progressive changes in behavior and personality, as revealed by clinical observation and longitudinal assessment. These abnormalities included mainly disinhibition, indifference, and apathy, as well as compulsive-like and utilization behavior. Nonetheless, as the disease progressed, negative symptoms dominated the clinical presentation of all cases.The common behavioral and personality abnormalities in FTDP-17 with MAPT seem to overlap with the behavioral variant FTD. Also, early behavioral and personality changes in FTDP-17 with MAPT are likely associated with the initial locus of neurodegeneration, and may represent an effect of the disease-related dynamic imbalance between the mutually inhibitory inter- and intrahemispheric processes, which may also possibly account for symptom change as the disease progresses, eventually resulting in an unified behavioral profile of negative symptoms.
Authors and Affiliations
Michał Harciarek, Emilia Sitek, Ewa Narożańska, Seweryna Konieczna, Dariusz Wieczorek, Bogna Brockhuis, Piotr Lass, Mirosława Dubaniewicz-Wybieralska, Maria Barcikowska, Jarosław Sławek
Keywords
Related Articles
- EP ID EP55428
- DOI -
- Views 108
- Downloads 0
WORKING MEMORY IN FEMALE VICTIMS OF DOMESTIC VIOLENCE
[b]Background.[/b] The purpose of this study was to evaluate selected cognitive functions in victims of domestic violence, and to examine the correlation between these functions and the intensity of symptoms of posttraum...
SPEECH AND LANGUAGE DYSFUNCTION IN CHILDREN WITH EPILEPSY
Speech and language dysfunction are a common developmental problem observed in children with epilepsy. These patients need a complex assessment and chronic therapy, conducted by neurologists, psychologists and spe...
Cognitive abilities in a patient with anorexia nervosa
The aim of our research was to examine the cognitive and intellectual functioning of a patient with anorexia nervosa, and to verify the persistence of cognitive functioning deficiencies five years after the regression of...
LANDAU-KLEFFNER-TYPE SYNDROME IN CHILDREN WITH DRUG-RESISTANT SYMPTOMATIC EPILEPSY
Purpose: To compare the severity and features of language disorders with the localization of brain lesions in children with epilepsyinducing cortical dysplasias.Results: The cognitive performance of 20 children with cort...
LIVING IN THE PAST: THE NEUROTIC PERSONALITY
The basic function of personality is to enable the individual to adapt to the anticipated state of affairs. In order for this to be successful, it is necessary to maintain an orientation toward the future. This requires,...