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Semantic and frontotemporal dementias

Glasgow, November 2009. Semantic and frontotemporal dementias. Julie Snowden. Cerebral Function Unit Greater Manchester Neuroscience Centre Salford Royal Foundation Trust. Historical Background. Clinical descriptions 1900s Pathology 1920s

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Semantic and frontotemporal dementias

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  1. Glasgow, November 2009 Semantic and frontotemporal dementias Julie Snowden Cerebral Function Unit Greater Manchester Neuroscience Centre Salford Royal Foundation Trust

  2. Historical Background Clinical descriptions 1900s Pathology 1920s Focal cerebral atrophy 1980s Localisationist Holistic Gestalt psychology Specialisation of function Neuropsychology

  3. Spatial orientation (mental map) Praxis Alzheimer’s disease (AD) Executive functions Social cognition Parietal Frontal Visual perception Occipital Temporal Language Frontotemporal lobar degeneration (FTLD) Episodic memory (events)

  4. Frontotemporal Lobar Degeneration Progressive aphasia Fronto-temporal dementia Semantic dementia

  5. Overview of Frontotemporal Lobar Degeneration • Onset in middle years (45-65) • Insidiously progressive • Affects both men and women • High familial incidence • Median duration 8 years (3-20)

  6. Clinical features • Few neurological signs  striatal signs but • May be associated with MND • Routine EEG normal • Focal change on structural / functional imaging

  7. Frontotemporal dementia • Breakdown in social, interpersonal conduct • Neglect of self-care and responsibilities • Emotional blunting, loss of insight • Repetitive, stereotyped behaviours • Hyperorality, dietary changes

  8. Plans/Goals Checking Organisation Attention Abstraction Flexibility New plan

  9. Planning Organisation Attention Abstraction Checking Flexibility Inertia/purposeless overactivity Disorganised behaviour Inattentive, distractible Concrete Poor self-monitoring, impaired insight Inflexible Loss of sympathy and empathy

  10. Wisconsin Card Sorting Test

  11. Weigls blocks Perseverative Dismantling behaviour shape, colour, motif

  12. Brixton spatial anticipation test 2 3 4 5 1 6 7 9 10 8 Burgess & Shallice, 1997

  13. Picture sequencing Verbal fluency • Animals • “Billy the budgie • and Spot the dog” • F words • “Frank – my brother” Concrete, rule violations

  14. Design fluency in FTD novel, abstract, 4-lines Perseverations, rule violations, concrete

  15. Dorsolateral Apathy, inertia Blunting Executive impairment severe Phenotypic variation in FTD Orbital Disinhibited, fatuous Executive impairment mild

  16. AD copy FTD copy

  17. MRI coronals SPECT coronal sagittal FTD

  18. MRI coronals SPECT coronal sagittal AD

  19. Cognition in FTD and AD

  20. Qualitative performance characteristics in FTD • Impulsivity, inattention • Economy of effort, lack of persistence • Concreteness of thought • Perseverative response • Unconstrained by goal – rule violations

  21. Non-tau Ubiquitinated neurones microvacuolation Tau Pick bodies in neurones Pick cells Neurofibrillary tangle-like in neurones and glia Frontotemporal lobar degeneration Ubiquitin protein: TDP-43 protein (Arai et al. 2006)

  22. Parietal Frontal Occipital Temporal Frontotemporal lobar degeneration (FTLD) Semantic dementia: overview • Circumscribed, multimodal disorder of meaning • Bilateral, often asymmetric degeneration of the anterior temporal lobes -especially inferior temporal gyri • Part of spectrum of FTLD SD

  23. Semantic dementia Impaired Word meaning naming comprehension phonology syntax speech fluency Preserved

  24. Language Naming “A nice little dog” Superordinate, coordinate, descriptive, omission errors No TOT effect/effortful word search No benefit from phonemic cueing Comprehension “rabbit ..what’s rabbit?” Partial/degraded knowledge Which is the rabbit? X 

  25. Impaired Recognition of: faces, objects nonverbal sounds smells, tastes tactile stimuli Perception of sensory stimuli Semantic loss - multimodal Preserved

  26. Perceptual matching  Category matching X

  27. SD drawing

  28. Nature of memory impairment word and object meaning facts, world knowledge memory for autobiographical events Impaired Preserved

  29. SEMANTIC DEMENTIA Bilateral temporal lobe atrophy

  30. Behaviour in semantic dementia • Narrowed behavioural repertoire • Preoccupations • Personal hobby e.g. painting, dress making • Word and number games, jigsaws, Sudoku • Preference for routine • Clockwatching, time bound More compulsive than frontotemporal dementia

  31. Narrowed world view • Self-centredness • Reduced sympathy and empathy

  32. Questions • What determines what is lost and retained? • What is the relationship between knowledge in different modalities? – amodal vs. multi-modal models • What is the relationship between knowledge and experience (semantic-episodic memory)?

  33. rabbit ? Animal

  34. Margaret Thatcher

  35. Elvis Presley

  36. Famous face and name recognition id = provides identifying information fam = reports familiarity

  37. MR and SPECT imaging Left > right Right > left

  38. Name and Face Identity % correct Temporal lobe atrophy

  39. Name and Face Familiarity % Temporal lobe atrophy

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