Abstract

FRONTOTEMPORAL DEMENTIA WITH parkinsonism linked to chromosome 17 (FTDP-17) is characterized by motor, psychiatric and cognitive symptoms. Executive dysfunction and behavioral disturbances such as disinhibition, socially inappropriate behavior, and unsafe driving habits1 are crucial for differential diagnosis and are usually described as changes in personality. In most cases of frontotemporal dementia, personality assessment is limited to the detection of behavioral abnormalities by means of symptom-oriented scales such as Neuropsychiatric Inventory. To our knowledge, personality changes in FTDP-17 have never been described in the paradigm of individual differences to show which personality traits are altered by the disease. The use of personality questionnaires enables direct observation of personality changes during progression of the disease, and the results can be interpreted in the context of personality dimensions according to a given personality theory. Herein we present the case of a 58-year-old man diagnosed with FTDP-17 due to the P301L mutation in the tau gene (MAPT) who developed early personality changes, 7 years before the onset of motor symptoms. Psychometric assessment of personality was administered using the Minnesota Multiphasic Personality Inventory (MMPI), a common patient-derived test, and an informant questionnaire based on Big Five personality model derived from the International Personality Item Pool,2 the International Personality Item Pool–Questionnaire, Polish Version (IPIP-QPV).3 The MMPI was performed at the age of 53, 5 years before motor symptoms onset. Results (L-52, F-65, K-41, 1-Hs 86, 2-D 75, 3-Hy 87, 4-Pd 47, 5-Mf 51, 6-Pa 65, 7-Pt 81, 8-Sc 67, 9-Ma 47, 0-Si 80) showed lack of foresight, poor insight, egocentrism, somatization tendencies, fatigue, depressed mood and introversion. Slightly elevated F score may be associated with somatic complaints (Hy4-115). The IPIP-QPV was completed by the patient's wife when he was 58. At the time of this assessment the patient scored 24 on Mini Mental State Examination and 11 on the Blessed Dementia Rating Scale, which highlights the discrepancy between relatively mildly affected cognitive status and moderate impairment in daily living activities, mostly due to executive dysfunction, personality and emotional changes. The results (min. −20, max. 20 for each trait) are presented here as: before the disease onset/at the time of testing: extraversion −14/−13, openness to experience 7/−6, agreeableness 3/−1, conscientiousness 5/1, neuroticism −1/−4. The IPIP-QPV indicated that introversion was independent of the disease progression. Interestingly, the patient became not only less open to experience, less agreeable and conscientious, but also less neurotic. Low openness to experience and decreased agreeableness seem connected to dysexecutive syndrome with impaired mental flexibility, set shifting and inhibition, while decreased neuroticism may be related to poor insight and disinhibition. This report shows that analyzing personality changes in dementia with reference to psychological theories can provide complementary information to cognitive and behavioral data. Personality assessment shows the dynamic aspect of individual personality during disease progression and provides useful data for patient care.

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