Tal Shany-Ur

Background: Theory of mind (ToM) is crucial for socioemotional interaction. ToM deficits may explain behavioral changes in dementia, especially Alzheimer's disease (AD) and frontotemporal dementia (FTD). Objective: This study examined the psychometrics of a new ToM test in healthy adults, identified ToM differences in dementia syndromes, and assessed if ToM scores predict neuropsychiatric function and real-life behavior. Methods: The UCSF Cognitive and Emotional Perspective Taking Test (CEPT) was evaluated in 195 healthy adults (age: 42.69 ± 16.20) and in a clinic cohort of 304 participants (age: 64.07 ± 9.2). Participants included healthy controls, AD, behavioral-variant frontotemporal dementia (bvFTD), semantic variant primary progressive aphasia (svPPA), non-fluent PPA (nfvPPA), and progressive supranuclear palsy (PSP) patients. CEPT's psychometrics were assessed, and ToM differences and predictions of neuropsychiatric symptoms were analyzed using regression models. Results: In controls, CEPT showed good validity and reliability. In patients, CEPT scores correlated with executive and emotional measures, but not language measures, showing good construct validity. Cognitive ToM was most impaired in AD and bvFTD, with less impairment in svPPA and PSP, and all patient groups showed impaired emotional ToM. ToM performance predicted real-life neuropsychiatric behavior, including anxiety, apathy, disinhibition, and aberrant motor behaviors. Conclusions: ToM deficits appear early in dementia syndromes and predict neuropsychiatric behavior. Assessing ToM and social cognition with ecologically valid tasks may help identify altered social cognition in early neurodegeneration.

Introduction: More precise subtyping within dementia syndromes leads to better prediction of pathology, supporting individualized, disease-specific treatments. Notably, studies highlight that identification of the right-temporal or semantic behavioral variant frontotemporal dementia (sbvFTD) subtype relies in part on measuring emotion recognition abilities. Methods: To evaluate the effectiveness of current tools, we compared two dynamic video-based affect labeling tests—the Dynamic Affect Recognition Test (DART) and The Awareness of Social Inference Test-Emotion Evaluation Test (TASIT-EET)—against the static image-based Name Affect subtest of the Comprehensive Affect Testing System (CATS-NA) test. A total of 555 persons with dementia (PwD), in the early stages of neurodegenerative disease (Clinical Dementia Rating ≤ 1; Mini Mental State Examination ≥ 20), diagnosed with Alzheimer’s disease syndrome (AD) (n=154), progressive supranuclear palsy syndrome (PSPS) (n=88), non-fluent variant primary progressive aphasia (nfvPPA) (n=77), semantic variant PPA (n=53), behavioral variant frontotemporal dementia (bvFTD) (n=124), semantic bvFTD (n=65), and 133 healthy older participants underwent emotion testing and structural MRI. Results: All emotion labeling tests differentiated PwD from healthy controls (DART, AUC=0.81; TASIT-EET, AUC=0.84; CATS-NA, AUC=0.72), and FTD with social cognition deficits (sbvFTD, bvFTD, and svPPA) from other PwDs (DART, AUC=0.64; TASIT-EET, AUC=0.66; CATS-NA, AUC=0.63). Dynamic tests outperformed CATS-NA in differentiating sbvFTD from bvFTD and svPPA (DART, AUC=0.79; TASIT-EET, AUC=0.74; CATS-NA, AUC=0.60), whereas DART outperformed TASIT-EET in differentiating sbvFTD from svPPA (DART, AUC=0.73; TASIT-EET, AUC=0.66). Multiple linear regression analysis showed that TASIT-EET performance was predicted by visual memory (Benson-delayed) and verbal semantic (BNT, Animal Fluency) functions (p<0.01) and CATS-NA performance was predicted by visuospatial (CATS-Face matching, Number location) (p<0.001) and executive functions (Modified Trail making speed) (p<0.05), while DART was predicted by only working memory functions (Digit span backward) (p<0.05). DART corresponded to the expected structural anatomy of emotion, including right predominant insula, anterior temporal, and orbitofrontal lobes. While both TASIT-EET and CATS-NA shared that pattern of brain anatomy, TASIT-EET correlated with more left temporal structures than DART, and CATS-NA associated with more dorsal structures than DART. Finally, all emotion labeling tests correlated with real-life empathy deficits measured by a standardized informant-based survey. Conclusion: Tasks showing dynamic audio-visual emotion displays showed better effectiveness for diagnostic differentiation of FTD syndromes than static image-based tasks, and the DART showed better clinical and anatomic precision than the TASIT-EET. Emotion identification deficits are a core feature of dementia syndromes like sbvFTD, but occur in the context of additional cognitive deficits. Therefore, careful selection of tests that reflect the key underlying neural circuits related to emotion, and which minimize demand from other cognitive domains, will result in more accurate diagnoses.

Objectives: We examined the long-term maintenance of treatment outcomes in patients with acquired brain injuries who participated in community-based neuropsychological rehabilitation (NR) programs, in a prospective, within-subject, longitudinal, partial double-blind cohort study.Methods: One hundred forty-three patients (39 females, mean age 33.5 years) who had experienced a brain injury (BI) (mean time since injury 3.95 years) were referred to a postacute community-based NR institute. Patients participated in one of the three programs aimed at improving their functional outcome: comprehensive–holistic neuropsychological rehabilitation, vocational-focused neuropsychological rehabilitation, and individual neuropsychological rehabilitation. Self-reported data regarding employment, community integration, perceived quality of life (PQoL), and mood were collected at program start and end, and annually for 3 years post program completion. Group placement was based on clinical considerations, such that the study did not aim to compare the programs, but rather to assess their long-term benefits.Results: Employment status and stability, community integration, and PQoL improved significantly after program completion and continued to improve for the following 3 years. The proportion of individuals with mood disturbances did not change during or after the programs.Conclusions: A clear consensus regarding BI rehabilitation is that long-term maintenance of treatment outcomes is imperative to its efficacy. Our findings suggest that postacute NR programs provide participants with various tools, skills, and psychological perspectives that they continue to gain from and generalize to real life after program completion, reflecting transformational processes with stable long-term benefits.

Corticobasal syndrome and dementia with Lewy bodies are clinical presentations with unique and overlapping features but distinct pathological substrates. We report the case of an 80 year-old man who presented with apraxia, rigidity, slowness, right arm myoclonus, a 10-year history of probable REM-sleep behavior disorder, and later developed visual hallucinations. At autopsy, he had pathological features of corticobasal degeneration, and Lewy body disease confined to the brainstem. This report highlights the importance of considering co-existing pathologies when a clinical presentation defies categorization, and demonstrates that salient features of dementia with Lewy bodies may result from pathology limited to the brainstem.

Editors' Note: Based on his experience, Dr. Verslegers disagrees with “Long-term use of daily sumatriptan injections in severe drug-resistant chronic cluster headache,” which presented arguments to use sumatriptan injections on a daily basis in refractory patients with cluster headache. Instead, he suggests that ergotamine should be employed when classical treatments fail. Drs. Leone and Proietti Cecchini, authors of the study, argue that ergots are less specific than triptans, have more side effects such as nausea and vomiting, increase the risk of cardiovascular events, and can lead to ergotism when taken frequently. The authors also suggest that neurostimulation procedures can be beneficial in treating intractable chronic cluster headache. Commenting on “Cognition and neuropsychiatry in behavioral variant frontotemporal dementia by disease stage,” Bertoux et al. point to the issue …

Behavioral variant frontotemporal dementia (bvFTD) is a clinical syndrome associated with frontal-predominant neurodegeneration.^1-3 Despite numerous detailed accounts, no systematic understanding of the nature, frequency, and mechanisms of bvFTD clinical symptoms has arisen, in part owing to tremendous diversity across patients.