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FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
FTD Turkish characte..
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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

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