scholarly journals The behavioral variant of frontotemporal dementia: a clinical case

2021 ◽  
Vol 13 (1) ◽  
pp. 67-73
Author(s):  
Yu. V. Kotsyubinskaya ◽  
V. A. Mikhailov ◽  
I. K. Stulov ◽  
N. I. Ananyeva ◽  
L. I. Sitnik

The paper is devoted to frontotemporal dementia (FTD), one of the most common forms of frontotemporal degeneration. The main symptoms of the disease include disinhibition, lack of empathy, obsessive-compulsive symptoms, apathy, cognitive impairment, appetite changes, and progressive changes in social behavior. In parallel, there are personality changes that are characterized by lower levels of self-awareness and by progressive psychological and social maladaptation of patients in society.The paper describes a clinical case of FTD in a female patient with marked behavioral changes and personality disorder. A 52-year-old woman was admitted to the alcoholism treatment department for alcohol intoxication and symptoms of mental confusion. According to her relatives, drinking too much alcohol every day, she was found to become rude, indifferent to others and her own duties, sharply limited the range of her activities and communication, and showed a decline in memory for current events. Psychopathological examination determined a distinct motivational-volitional decrease, the patient’s inability to mobilize mental activity, non-critical thinking, and indifference. X-ray diagnosis revealed the changes characteristic of frontotemporal neurodegeneration (atrophy of the frontal and temporal lobes prevails). The described case confirms that alcohol abuse can mask organic disorders that develop in systemic cerebral atrophy.

2019 ◽  
Vol 7 (5) ◽  
pp. 982-999 ◽  
Author(s):  
Richard M. McFall ◽  
James Allison ◽  
Richard J. Viken ◽  
William Timberlake

Basic learning theorists developed the response-disequilibrium model to resolve the long-standing psychological puzzle of how to specify in advance the circumstances that will yield reinforcement effects. The model explains the behavioral changes in reinforcement effects as predictable adaptations to external constraints on the free-baseline levels of those behaviors. Here we introduce response-disequilibrium therapy (RDx), a clinical intervention based on this model. We present a series of clinical case studies using RDx to treat obsessive-compulsive disorders, report highly favorable results in comparison with the standard therapy, and discuss the implications.


2011 ◽  
Vol 26 (S2) ◽  
pp. 487-487
Author(s):  
L. Fonseca ◽  
Á. Machado

IntroductionBehavioral and personality changes are the core symptoms of frontotemporal dementia. Suicide and suicide attempts have been reported in demented patients.Clinical caseWe present a case of an 80 years-old-male patient, with a suicide attempt at the age of 76 as the presentation symptom of FTD.Clincal studyThere are few studies of suicide or selfharm in frontotemporal dementia where such behavior might be expected to be more common. We are conducting a clinical study in FTD patients about the relation between FTD and suicide. The results of such study will be presented and discussed.DiscussionTo our knowledge, there are no reliable data or reports about suicide in FTD patients. Also, we didn’t find any case report of a suicide attempt as the first presentation symptom of FTD. We discuss the known data about this issue considering our clinical study and report.


2021 ◽  
Author(s):  
Leonardo de Souza ◽  
Maxime Bertoux ◽  
Luciano Mariano ◽  
Elisa Resende ◽  
Antônio Lúcio Teixeira ◽  
...  

Background: Mentalizing and emotion recognition are impaired in behavioral variant frontotemporal dementia (bvFTD). It is not clear whether these abilities are disturbed in progressive supranuclear palsy (PSP). Objective: To investigate social cognition (SC) between bvFTD and PSP. The neural basis of SC in PSP and bvFTD groups were also investigated by neuroimaging. Methods: Data from the notification sheet were collected and patients were classified according to current clinical and pathological criteria. Results: Groups did not differ on age, schooling and sex. Compared to controls, bvFTD and PSP patients had reduced scores in all tests of SC. bvFTD and PSP did not differ on measures of SC. PSP and bvFTD had cerebral atrophy in critical regions for SC. The cortical correlates of emotion recognition overlapped in bvFTD and PSP, correlating with frontal medial cortex, insula and limbic structures. PSP and bvFTD patients also displayed similar patterns of brain correlations (anterior temporal lobes) for social norms. The neural correlates of mentalizing were associated with frontal and temporal poles bilaterally, in both bvFTD and PSP. Conclusion:PSP patients exhibit impairment in mentalizing. PSP and bvFTD share clinical, cognitive and neuroimaging features.


2007 ◽  
Vol 9 (2) ◽  
pp. 153-160

Behavioral manifestations may dominate the clinical picture of the frontal variant of frontotemporal dementia (fv-FTD) for a long time before the appearance of true cognitive deficits. On the other hand, a deficit in the episodic memory domain represents the main manifestation of Alzheimer's disease (AD). Many behavioral disorders have been described in the clinical course of both FTD and AD; however, apathy and personality changes characterize frontal dementias, while depression dominates in AD, at least in the earlier stages. Depending on the distribution of neural damage, different patterns of noncognitive manifestations may be expected in different subtypes of FTD. Recent research on the social cognition deficit in FTD has offered new insights into the relationship between cognition and behavior, suggesting that some aspects of the behavioral changes in dementia may be generated by impairment in this domain.


2014 ◽  
Author(s):  
Joseph P. Barsuglia ◽  
Michelle J. Mather ◽  
Hemali V. Panchal ◽  
Aditi Joshi ◽  
Elvira Jimenez ◽  
...  

2013 ◽  
Vol 7 (2) ◽  
pp. 197-205 ◽  
Author(s):  
Corina Satler ◽  
Carlos Tomaz

ABSTRACT Anosognosia, impairment insight and unawareness of deficits are used as equivalent terms in this study. Objective: To investigate the relationship between the presence of anosognosia symptoms and cognitive domains, functional abilities, and neuropsychiatric symptoms in patients with probable Alzheimer's disease (pAD) and elderly controls (EC). Methods: Twenty-one pAD (14 women) and twenty-two EC (16 women) were submitted to a neuropsychological battery of tests assessing global cognitive status, and specific cognitive functions: memory, executive and attention functions, verbal fluency and visuoconstructive abilities. Additionally, functional abilities (FAQ) and neuropsychiatric symptoms (NPI) were measured. Results: The linear regression statistical test found general anosognosia to be associated with subjective memory complaints, age and Arithmetic-DRS in the EC group. On the other hand, cognitive and functional abilities scores (Arithmetic- DRS, IQCODE and FAQ) were the best predictors in pAD patients, particularly for behavioral awareness. Conclusion: These results indicated that different variables are associated with self-awareness for pAD patients and EC, but for both groups executive functions appear to play an important role, contributing particularly to awareness of behavioral changes.


2020 ◽  
pp. 089198872094423
Author(s):  
Thaís Bento Lima-Silva ◽  
Eneida Mioshi ◽  
Valéria Santoro Bahia ◽  
Mário Amore Cecchini ◽  
Luciana Cassimiro ◽  
...  

Introduction: There is a shortage of validated instruments to estimate disease progression in frontotemporal dementia (FTD). Objectives: To evaluate the ability of the FTD Rating Scale (FTD-FRS) to detect functional and behavioral changes in patients diagnosed with the behavioral variant of FTD (bvFTD), primary progressive aphasia (PPA), and Alzheimer disease (AD) after 12 months of the initial evaluation, compared to the Clinical Dementia Rating scale−frontotemporal lobar degeneration (CDR-FTLD) and the original Clinical Dementia Rating scale (CDR). Methods: The sample consisted of 70 individuals, aged 40+ years, with at least 2 years of schooling, 31 with the diagnosis of bvFTD, 12 with PPA (8 with semantic variant and 4 with non-fluent variant), and 27 with AD. The FTD-FRS, the CDR, and the 2 additional CDR-FTLD items were completed by a clinician, based on the information provided by the caregiver with frequent contact with the patient. The Addenbrooke Cognitive Examination-Revised was completed by patients. After 12 months, the same protocol was applied. Results: The FTD-FRS, CDR-FTLD, and CDR detected significant decline after 12 months in the 3 clinical groups (exception: FTD-FRS for PPA). The CDR was less sensitive to severe disease stages. Conclusions: The FTD-FRS and the CDR-FTLD are especially useful tools for dementia staging in AD and in the FTD spectrum.


Alcohol ◽  
2019 ◽  
Vol 79 ◽  
pp. 37-45 ◽  
Author(s):  
Thaddeus M. Barney ◽  
Andrew S. Vore ◽  
Anny Gano ◽  
Jamie E. Mondello ◽  
Terrence Deak

2017 ◽  
Vol 114 (19) ◽  
pp. 5029-5034 ◽  
Author(s):  
Grietje Krabbe ◽  
S. Sakura Minami ◽  
Jon I. Etchegaray ◽  
Praveen Taneja ◽  
Biljana Djukic ◽  
...  

Frontotemporal dementia (FTD) is the second most common dementia before 65 years of age. Haploinsufficiency in the progranulin (GRN) gene accounts for 10% of all cases of familial FTD. GRN mutation carriers have an increased risk of autoimmune disorders, accompanied by elevated levels of tissue necrosis factor (TNF) α. We examined behavioral alterations related to obsessive–compulsive disorder (OCD) and the role of TNFα and related signaling pathways in FTD patients with GRN mutations and in mice lacking progranulin (PGRN). We found that patients and mice with GRN mutations displayed OCD and self-grooming (an OCD-like behavior in mice), respectively. Furthermore, medium spiny neurons in the nucleus accumbens, an area implicated in development of OCD, display hyperexcitability in PGRN knockout mice. Reducing levels of TNFα in PGRN knockout mice abolished excessive self-grooming and the associated hyperexcitability of medium spiny neurons of the nucleus accumbens. In the brain, PGRN is highly expressed in microglia, which are a major source of TNFα. We therefore deleted PGRN specifically in microglia and found that it was sufficient to induce excessive grooming. Importantly, excessive grooming in these mice was prevented by inactivating nuclear factor κB (NF-κB) in microglia/myeloid cells. Our findings suggest that PGRN deficiency leads to excessive NF-κB activation in microglia and elevated TNFα signaling, which in turn lead to hyperexcitability of medium spiny neurons and OCD-like behavior.


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