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FTD Turkish characte..

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  • 1. Frontotemporal lobar degeneration: demographic and clinical characteristics in a Turkish dementia population Görsev G. Yener, M.D. Neurology, Dokuz Eylül University Izmir, Turkey gorsev.yener@deu.edu.tr
  • 2. FTLD Prevalence  Not clear (4-16%)  Common cause pre-senile dementia 1:1 with AD 45-64 years  (Ratnavalli Neurology 2002) more common than AD below 60 years  (Knopman Neurology 2004)
  • 3. FTLD in Izmir, Turkey Demographic data Genetics/Pathological Syndrome Neary Criteria – FTD, PA, SD MND association
  • 4.  1994-2004 Izmir DEU Dementia Clinic N=1169  Demented= 66 % AD (possible / probable) 67 % Vascular dementia 15 % Fronto-temporal dementia 4 % Lewy body dementia 9 %
  • 5. FTLD Clinical Heterogeneity  Genetic & sporadic cases  Histology varies  Motor overlap with PSP, CBD, ALS
  • 6. Neary Criteria  Frontotemporal Lobar Degeneration  Frontotemporal Dementia  Progressive Non-Fluent Aphasia  Semantic Dementia Neary et al. 1998, Neurology
  • 7. Dokuz Eylül University Dementia and Movement Disorders Clinics  1994-2001  35 FTLD, 25 PSP, 5 CBD cases  Demographic and clinical features  Webster  MMSE (subitems)
  • 8. FTLD: Gender ** Turkish (N=35) (% male) Age(Years) ** 39% 40% 72% 59% 0 20 40 60 80 FTD SD PNFA MND 40% 67% 64% 0 20 40 60 80 FTD SD PNFA US (N=353) (% male) Johnson et al. Arch Neurol. 2005;62:925-930. Yener et al 2002
  • 9. FTLD: Age at Onset ** 58 59 63 50 55 60 65 FTD SD PNFA US (N=353) Age(Years) Johnson et al. Arch Neurol. 2005;62:925-930. 61 72 61 50 55 60 65 70 75 FTD SD PNFA Turkish (N=35) ? Yener et al 2002
  • 10. Presenile onset (%) -Turkish 26% 100% 48%46% 0 20 40 60 80 100 FTLD (N= 35)PSP (N= 25)CBD (N= 5)AD (N= 390) **
  • 11. 40-60 % In Netherland series 38% (15 million screened) Family Hx (+) , RR=3.5 Family history
  • 12. Family Hx (%)-Turkish population 10% 24%20% 25% 54% 0 0 0 0 0 1 1 FTLD (N= 35) PSP (N=25)CBD (N=5) AD (N= 390) VAD (N=65) **
  • 13. FTLD Pathological Syndrome Core Features Frontotemporal predominance Gliosis, spongiosus, neuronal loss
  • 14. Variable Histological Features Neuronal inclusions with ubiquitin  (Clinical: MND, ALS, Paget, Inclusion body myositis) Neuronal inclusions with tau  (Clinical: FTDP-17, PSP, CBD) Other inclusions  (Neuronal intermediate filament inclusion dementia) No inclusions (DLDH)  (Clinical: FTLD)
  • 15. Pick Bodies Cerebral cortex (Described by Alzheimer in 1911)
  • 16. Time (minutes) Distance travelledbythe tauspecies(m) B.A. d 0 20 40 60 80 100 0N4R-EGFP 0N4RpCIneo 0N4R-EGFP 0N4R-untagged Courtesy of Tim Hutton, Cambridge, UK
  • 17. Tau mutations
  • 18. Bigio, et al, 2004, J Neuropath Exp Neurol, 63(8): 801 811 Neuronal ubiquitinated intranuclear inclusions in familial and non-familial FTD-MND associated with ALS
  • 19. FTD with ubiquitinated neuronal inclusions and visuospatial impairment Meiner et al Neurology 2005;65:478–480
  • 20. Ubiquitin and Tau  Ubiquitin is a marker for a given protein to be sent to a proteasome for degradation  Ubiquitin proteins are also needed for the degradation of tau
  • 21. FTD Neuropathology Subtypes  FTD-Ub related to MND subtype  CBD, PSP major tau subtype  DLDH less common with new staining techniques
  • 22. Clinical Features  Subtypes  MND  Parkinsonism  MMSE
  • 23. FTLD subtypes in the US and Turkey PNFA (n=11) 31% SD (n=3) 9% FTD (n=21) 60% PNFA (n=87) 25% SD (n=66) 19%FTD (n=200) 56% Johnson et al. Arch Neurol. 2005;62:925-930. 3 sites (N=353) 1 site (N=35)Yener et al 2002
  • 24. MND and FTD are associated  FTD is associated with MND (15%)  Most ALS patients develop FTD  FTLD-ALS chr 9 and 17  Hypometabolism in frontal lobes in ALS
  • 25. Chang et al, Neurology 2005;65:75–80 Brain atrophy in ALS and ALS/FTLD
  • 26. FTLD ; 78% FTD_MND ; 22% 0% 10% 20% 30% 40% 50% 60% 70% 80% FTLD FTD_MND Turkish FTLD and MND
  • 27. Webster scores-Turkish FTLD subtypes 4,4 0,5 1 0,6 0,00 1,00 2,00 3,00 4,00 5,00 6,00 7,00 8,00 FTD SD PNFA FTD- MND Clinical Features-Parkinsonism **
  • 28. Clinical Features  Parkinsonism appears earlier in FTLD  MND - more often parkinsonism  No tau mutation was found in FTD- MND group.
  • 29. MMSE Scores in Turkish FTLD 14,1014,10 24,30 23,60 0 20 FTLD PSP CBD MND-FTD
  • 30. MMSE Figure copying (%) 63% 0% 69% 33% 0 20 40 60 80 FTLD (n= 27) PSP(n= 25)CBD (n= 5) ND-FTD (n= 8)
  • 31. Clinical patterns PSP~CBD Both higher Webster and MMSE scores MMSE figure copy is discriminative
  • 32. Clinical patterns Webster scores FTD-MND > FTLD MMSE scores FTD-MND = FTLD preserved figure copying FTD-MND > FTLD
  • 33. Clinical patterns FTLD subtypes  total MMSE PNFA < SD < FTD  figure copy SD < PNFA < FTD
  • 34. Conclusions  FTLD common in presenile ages  Turkish FTLD subtypes have similar profile to the US FTD> PNFA > SD
  • 35. Conclusions  Higher family Hx than other dementias (54%)  FTLD-MND association (22%)  FTD-MND has higher parkinsonism scores