Frontotemporal Dementia: An Overview


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  • Frontotemporal Dementia: An Overview

    1. 1. Frontotemporal Dementia An Overview October 13, 2012 Brian S. Appleby, M.D. Staff, Lou Ruvo Center for Brain Health, Cleveland Clinic
    2. 2. Disclosures• Co-PI, memantine for frontotemporal dementia, Forest Pharmaceuticals
    3. 3. Objectives• Describe the illnesses within the frontotemporal lobar degeneration (FTLD) spectrum• Characterize the common types of FTLD• Illustrate case examples of FTLD
    4. 4. Frontotemporal Lobar Degeneration Semantic PPA dementia PSPAGD FTD FTD-MND CBDFUS NIFID HSD IBMPFD
    5. 5. Papageorgiou S, et al. Alzheimer Dis Assoc Disord, 2009AD FTLD
    6. 6. Survival Time Garcin B, Neurology 2009
    7. 7. Appleby BS, Dementi Geriatr Cog Disord 2008
    8. 8. CJD FTD SD PPA AD CBDZBI=Zarit Burden Interview Johns Hopkins FTD/YOD Clinic
    9. 9. Hemispheric Asymmetry (MRI and PET)
    10. 10. Frontotemporal dementia (55%)Primary progressive aphasia Semantic dementia(25%) (20%)
    11. 11. Frontotemporal Dementia• Mean age of onset: 55-65 years-of-age• Male>Female• Prominent frontal lobe symptoms - Disinhibition - Poor insight/judgment - Loss of social graces - Perseverative behaviors - Apathy
    12. 12. Primary Progressive Aphasia• Progressive non-fluent aphasia• Decreased speech output• Speech apraxia• Changes in grammar use• Neuropathology is often progressive supranuclear palsy or corticobasal degeneration Josephs KA, Brain 2006
    13. 13. Semantic Dementia Animal Bird
    14. 14. Treatment
    15. 15. Cholinesterase Inhibitors Irwin D, Am J Alzheimers Dis Other Disord 2010
    16. 16. Memantine• Increases brain FDG-PET metabolism in FTD and SD (Chow 2011, 2012)• No improvement in behavior/cognition (Diehl-Schmid 2008, Vercelletto 2011)• Transient improvement in neuropsych symptoms in FTD and PPA (Swanberg 2007, Boxer 2009)• Currently in multisite RCT
    17. 17. Antipsychotics• Often used because of behavioral symptoms• Mounting evidence of hypersensitivity to EPS in FTD (Mendez 2001, Pijnenburg 2003, Czarnecki 2008)• Think of overlap of FTLD with “Parkinson’s-Plus” disorders
    18. 18. Benzodiazepines“Do you really want to give a disinhibitingmedication to a demented person with nofrontal lobes?”
    19. 19. Antidepressants• Loss of serotonergic neurons->replete with serotonergic drugs• Trazodone (Lebert 2004)• SSRIs (Swartz 1997, Moretti 2003, Herrmann 2011)• Paroxetine: no effect, worsened cognition (Deakin 2004)
    20. 20. Non-Pharmacological Interventions• Environment (locked behavioral dementia unit)• Caregiver support (FTD support groups)• SSDI Compassionate Allowances• Elder care lawyer involvement early• Driving (different concerns)• Travel letters
    21. 21. Case #1• 58 y.o. AAM attorney with h/o dyslexia with a 2 yr h/o cognitive decline and personality change• Distracted, poor concentration, low mood, fatigued• Only reads comic books and watches cartoons, often the same ones repeatedly
    22. 22. ExamGeneral: Asked to leave room severaltimes to walk around. Buccalstereotypies (i.e., blowing)Speech: Sparse, poverty of contentAffect: Flat, no brighteningMMSE: 19/30Brain MRI: Mild generalized atrophy
    23. 23. Further Work-up
    24. 24. Case #2• 60 y.o. WM with no past neuropsych hx• Initial complaint is stuttering/stammering• Phonemic paraphrasic errors on exam• MoCA=28/30• “f”=2 words, “animals”=18• At next visit, has complaints of poor concentration and distractibility
    25. 25. Case #3• 60 y.o. WF with h/o rheumatic fever, GERD, vit D def, osteopenia, and liver/brain hemangiomas• 1 yr h/o progressive strabismus with diplopia (repaired with return 1 mo later), parkinsonism, dysarthria, and short-term amnesia, fatigue, anxiety, panic attacks
    26. 26. ExamSpeech: hypophonic, sparse, dysarthricThought Process: bradyphrenicAffect: stable, flat without brighteningMMSE: 7/30UPDRS II: 43Neuro: vertical gaze impairment, choppysaccades, hypomimia, axial rigidity
    27. 27. Case #4• 50 y.o. female from Spain with 4 yr h/o gradual executive dysfxn, short-term amnesia, progressive non-fluent aphasia, parkinsonism, and myoclonus• Paces frequently, apathetic, crying when frustrated, seen responding to internal stimuli, and sometimes thinks others are stealing from her
    28. 28. ExamGait: slow, shuffling, leans to leftSpeech: Effortful, paraphrasic errorsMMSE: 5/303MS: 17/100Clock: 1/5UPDRS II: 44•Myoclonus with speech and action•Left-sided neglect, finger agnosia
    29. 29. ExamPentagons Clock