The natural history of temporal variant frontotemporal dementia

Department of Neurology, University of California at San Francisco, San Francisco, CA 94143-1207, USA.
Neurology (Impact Factor: 8.29). 05/2005; 64(8):1384-90. DOI: 10.1212/01.WNL.0000158425.46019.5C
Source: PubMed


The temporal variant of frontotemporal dementia (tvFTD) features asymmetric anterior temporal/amygdala degeneration as well as ventromedial frontal, insular, and inferoposterior temporal involvement. Left temporal atrophy has been linked to loss of semantic knowledge, whereas behavioral symptoms dominate the right temporal variant.
To investigate the first symptoms and the timing of subsequent symptoms in patients with left versus right tvFTD.
Twenty-six patients with tvFTD were identified. Six had right > left temporal atrophy (right temporal lobe variant [RTLV]) and were matched with six having comparable left > right temporal atrophy (left temporal lobe variant [LTLV]). Clinical records were reviewed to generate individualized symptom chronologies.
In all patients, first symptoms involved semantics (4/6 LTLV, 1/6 RTLV), behavior (4/6 RTLV, 1/6 LTLV), or both (1 LTLV, 1 RTLV). Semantic loss began with anomia, word-finding difficulties, and repetitive speech, whereas the early behavioral syndrome was characterized by emotional distance, irritability, and disruption of physiologic drives (sleep, appetite, libido). After an average of 3 years, patients developed whichever of the two initial syndromes--semantic or behavioral--that they lacked at onset. A third stage, 5 to 7 years from onset, saw the emergence of disinhibition, compulsions, impaired face recognition, altered food preference, and weight gain. Compulsions in LTLV were directed toward visual, nonverbal stimuli, whereas patients with RTLV were drawn to games with words and symbols.
The temporal variant of frontotemporal dementia follows a characteristic cognitive and behavioral progression that suggests early spread from one anterior temporal lobe to the other. Later symptoms implicate ventromedial frontal, insular, and inferoposterior temporal regions, but their precise anatomic correlates await confirmation.

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    • "On the other hand, patients with predominantly right temporal atrophy present with clear loss of empathy and ability to read social cues, more mental rigidity, and other behavioral symptoms that overlap with and often meet FTDC diagnostic criteria for bvFTD, while their language symptoms are subtle or absent (Seeley et al. 2005). Thus, in this study, 14 predominantly temporal patients with substantial semantic loss for object knowledge were categorized as svPPA, and 13 patients without substantial loss of object knowledge with predominantly behavioral presentation were classified as rtFTD (Seeley et al. 2005; Josephs et al. 2009). Patients with nonfluent variant primary progressive aphasia (nfvPPA) were excluded because too few were available over the course of enrollment to complete quantitative statistical analysis. "
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    ABSTRACT: IntroductionThe Behavioral Inhibition System (BIS) and the Behavioral Activation System (BAS) have been theorized as neural systems that regulate approach/withdrawal behaviors. Behavioral activation/inhibition balance may change in neurodegenerative disease based on underlying alterations in systems supporting motivation and approach/withdrawal behaviors, which may in turn be reflected in neuropsychiatric symptoms.MethodA total of 187 participants (31 patients diagnosed with behavioral variant of FTD [bvFTD], 13 semantic variant of primary progressive aphasia [svPPA], 14 right temporal variant FTD [rtFTD], 54 Alzheimer's disease [AD], and 75 older healthy controls [NCs]) were included in this study. Changes in behavioral inhibition/activation were measured using the BIS/BAS scale. We analyzed the correlation between regional atrophy pattern and BIS/BAS score, using voxel-based morphometry (VBM).ResultsADs had significantly higher BIS scores than bvFTDs and NCs. bvFTDs activation-reward response (BAS-RR) was significantly lower than ADs and NCs, though their activation-drive (BAS-D) was significantly higher than in ADs. Both AD and rtFTD patients had abnormally low activation fun-seeking (BAS-FS) scores. BIS score correlated positively with right anterior cingulate and middle frontal gyrus volume, as well as volume in the right precentral gyrus and left insula/operculum.ConclusionsAD, bvFTD, and rtFTD patients show divergent patterns of change in approach/withdrawal reactivity. High BIS scores correlated with preservation of right-predominant structures involved in task control and self-protective avoidance of potentially negative reinforcers. Damage to these regions in bvFTD may create a punishment insensitivity that underlies patients’ lack of self-consciousness in social contexts.
    Brain and Behavior 05/2015; 5(9). DOI:10.1002/brb3.350 · 2.24 Impact Factor
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    • "and impulsivity, lack of insight, and emotional blunting, callousness , and a loss of empathy (Gustafson, 1987; Neary et al., 2005). Notably, patients with semantic dementia with right temporal lobe atrophy also tend to demonstrate similar behavioural symptoms early in the course of illness (Bozeat et al., 2000; Seeley et al., 2005; Snowden et al., 2001), and all three subtypes can include these behavioural abnormalities (Neary et al., 1998; Rosen et al., 2006). One of the core diagnostic features of bvFTD is a loss of empathy , exhibited by decreased social interest, diminished responsiveness to the feelings of others, and increased coldheartedness (Perry and Miller, 2001; Rascovsky et al., 2011). "
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    ABSTRACT: Objectives: Behavioural variant frontotemporal dementia (bvFTD) is a debilitating neurodegenerative disorder characterized by frontal and temporal lobe atrophy primarily affecting social cognition and emotion, including loss of empathy. Many consider empathy to be a multidimensional construct, including cognitive empathy (the ability to adopt and understand another's perspective) and emotional empathy (the capacity to share another's emotional experience). Cognitive and emotional empathy deficits have been associated with bvFTD; however, little is known regarding the performance of patients with bvFTD on behavioural measures of emotional empathy, and whether empathic responses differ for negative versus positive stimuli. Methods: 24 patients with bvFTD and 24 healthy controls completed the Multifaceted Empathy Test (MET; Dziobek et al., 2008), a performance-based task that taps both cognitive and emotional facets of empathy, and allows for the discrimination of responses to negative versus positive realistic images. MET scores were also compared with caregiver ratings of patient behaviour on the Interpersonal Reactivity Index, which assesses patients' everyday demonstrations of perspective taking and empathic concern. Results: Patients with bvFTD were less accurate than controls at inferring mental states for negative and positive stimuli. They also demonstrated lower levels of shared emotional experience, more positive emotional reactions, and diminished arousal to negative social stimuli relative to controls. Patients showed reduced emotional reactions to negative non-social stimuli as well. Lastly, the MET and IRI measures of emotional empathy were found to be significantly correlated within the bvFTD group. Conclusions: The results suggest that patients with bvFTD show a global deficit in cognitive empathy, and deficient emotional empathy for negative, but not positive, experiences. Further, a generalized emotional processing impairment for negative stimuli was observed, which could contribute to the emotional empathy deficit. This work highlights potential treatment targets and a means to assess the impact of novel therapies on socioemotional impairment in bvFTD.
    Neuropsychologia 11/2014; 67. DOI:10.1016/j.neuropsychologia.2014.11.022 · 3.30 Impact Factor
    • "In addition to predominant right and subsequently left anterior and inferior temporal lobe atrophy, structural brain imaging also shows involvement of the right amygdala and hippocampus, parahippocampal and fusiform gyri, and less prominent atrophy in left amygdala and parahippocampal gyrus. With disease progression, atrophy extends into the ventromedial frontal, insular, and inferolateral temporal cortex (Henry et al., 2014; Seeley et al., 2005). Figure 1. "
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    ABSTRACT: Frontotemporal dementia (FTD) is a progressive, neurodegenerative syndrome that results in changes in personality, behavior, and language. The right temporal variant, a relatively under-studied and perhaps under-diagnosed clinical subtype of FTD, manifests as behavioral changes, loss of person-specific knowledge, and eventual deficits in word-finding and semantic knowledge. Thorough assessment is necessary in order to distinguish right temporal variant FTD from other dementias. Speech-language pathologists (SLPs) play an essential role in accurate diagnosis, provision of appropriate resources and referrals, and administration of effective treatments.
    Perspectives on Neurophysiology and Neurogenic Speech and Language Disorders 10/2014; 24(4):157-163. DOI:10.1044/nnsld24.4.157
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