Pain and temperature processing in dementia: a clinical and neuroanatomical analysis
Phillip D. Fletcher,Laura E. Downey,Hannah L. Golden,Camilla N. Clark,Catherine F. Slattery,Ross W. Paterson,Jonathan D. Rohrer,Jonathan M. Schott,Martin N. Rossor,Jason D. Warren +9 more
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TLDR
Using a semi-structured caregiver questionnaire and MRI voxel-based morphometry in patients with frontotemporal degeneration or Alzheimer’s disease, Fletcher et al. show that symptoms are underpinned by atrophy in a distributed thalamo-temporo-insular network implicated in somatosensory processing.Abstract:
Symptoms suggesting altered processing of pain and temperature have been described in dementia diseases and may contribute importantly to clinical phenotypes, particularly in the frontotemporal lobar degeneration spectrum, but the basis for these symptoms has not been characterized in detail. Here we analysed pain and temperature symptoms using a semi-structured caregiver questionnaire recording altered behavioural responsiveness to pain or temperature for a cohort of patients with frontotemporal lobar degeneration (n = 58, 25 female, aged 52-84 years, representing the major clinical syndromes and representative pathogenic mutations in the C9orf72 and MAPT genes) and a comparison cohort of patients with amnestic Alzheimer's disease (n = 20, eight female, aged 53-74 years). Neuroanatomical associations were assessed using blinded visual rating and voxel-based morphometry of patients' brain magnetic resonance images. Certain syndromic signatures were identified: pain and temperature symptoms were particularly prevalent in behavioural variant frontotemporal dementia (71% of cases) and semantic dementia (65% of cases) and in association with C9orf72 mutations (6/6 cases), but also developed in Alzheimer's disease (45% of cases) and progressive non-fluent aphasia (25% of cases). While altered temperature responsiveness was more common than altered pain responsiveness across syndromes, blunted responsiveness to pain and temperature was particularly associated with behavioural variant frontotemporal dementia (40% of symptomatic cases) and heightened responsiveness with semantic dementia (73% of symptomatic cases) and Alzheimer's disease (78% of symptomatic cases). In the voxel-based morphometry analysis of the frontotemporal lobar degeneration cohort, pain and temperature symptoms were associated with grey matter loss in a right-lateralized network including insula (P < 0.05 corrected for multiple voxel-wise comparisons within the prespecified anatomical region of interest) and anterior temporal cortex (P < 0.001 uncorrected over whole brain) previously implicated in processing homeostatic signals. Pain and temperature symptoms accompanying C9orf72 mutations were specifically associated with posterior thalamic atrophy (P < 0.05 corrected for multiple voxel-wise comparisons within the prespecified anatomical region of interest). Together the findings suggest candidate cognitive and neuroanatomical bases for these salient but under-appreciated phenotypic features of the dementias, with wider implications for the homeostatic pathophysiology and clinical management of neurodegenerative diseases.read more
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Primary progressive aphasias associated with C9orf72 expansions: Another side of the story.
Dario Saracino,Dario Saracino,Amandine Géraudie,Anne M. Remes,Anne M. Remes,Sophie Ferrieux,Marie Noguès-Lassiaille,Simona Bottani,Simona Bottani,Lorenzo Cipriano,Lorenzo Cipriano,Marion Houot,Aurélie Funkiewiez,Agnès Camuzat,Agnès Camuzat,Daisy Rinaldi,Marc Teichmann,Marc Teichmann,Jérémie Pariente,Philippe Couratier,Claire Boutoleau-Bretonnière,Sophie Auriacombe,Frédérique Etcharry-Bouyx,Richard Levy,Richard Levy,Raffaella Migliaccio,Raffaella Migliaccio,Eino Solje,Isabelle Le Ber,Isabelle Le Ber,Serge Belliard,Frédéric Blanc,Eve Benchetrit,Hugo Bertin,Anne Bertrand,Anne Bissery,Stéphanie Bombois,Marie-Paule Boncoeur,Alexis Brice,Mathieu Ceccaldi,Mathieu Chastan,Yaohua Chen,Marie Chupin,Olivier Colliot,Xavier Delbeuck,Christine Delmaire,Vincent Deramecourt,Mira Didic,Charles Duyckaerts,Maïté Formaglio,Emmanuel Gerardin,Véronique Golfier,Marie-Odile Habert,idier Hannequin,Lucette Lacomblez,Julien Lagarde,Géraldine Lautrette,Benjamin Le Toullec,Marie-Anne Mackowiak,Olivier Martinaud,Bernard-François Michel,Jacques Monteil,Assi-Hervé Oya,Florence Pasquier,Grégory Petyt,Adeline Rollin-Sillaire,Carole Roué-Jagot,Sabrina Sayah,Marie Sarazin,Christel Thauvin-Robinet,Catherine Thomas-Antérion,François Sellal,Martine Vercelletto,David Wallon +73 more
TL;DR: In this paper, the authors comprehensively analyzed a unique series of 16 patients affected by PPA-C9orf72 repeat expansions, including non-fluent/agrammatic variants, with apraxia of speech being the main defining feature.
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A Neuron-Glial Model of Exosomal Release in the Onset and Progression of Alzheimer's Disease.
TL;DR: In this paper, the authors proposed a model for Alzheimer's Aβ accumulation based on Ca 2+-dependent exosome release from astrocytes and exploited the model to assess how temperature dependence of exosomes release could interact with Aβ neurotoxicity.
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Behavioral Variant Frontotemporal Dementia and Social and Criminal Transgressions.
TL;DR: A review of the literature on the frequency of social and criminal transgressions in Behavioral variant frontotemporal dementia and the neurobiological disturbances that underlie them found a high frequency of transgressions among patients with bvFTD due to impairments in neurocognition, social emotions, and moral reasoning.
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Self-awareness in Dementia: a Taxonomy of Processes, Overview of Findings, and Integrative Framework
TL;DR: In this paper, a taxonomy of self-awareness processes is presented, discussing how these can be structured across different levels of cognitive complexity, indicating the relative preservation of capacities such as body ownership and agency, despite impairments in higher level cognitive processes, such as autobiographical memory and emotional regulation.
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AI-Assisted In-House Power Monitoring for the Detection of Cognitive Impairment in Older Adults.
Yuriko Nakaoku,Soshiro Ogata,Shunsuke Murata,Makoto Nishimori,Masafumi Ihara,Koji Iihara,Misa Takegami,Kunihiro Nishimura +7 more
TL;DR: In this article, the authors investigated whether unobtrusive in-home power monitoring technologies could be used to predict cognitive impairment and found that the former had better predictive ability compared to the latter.
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