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Görsev G. Yener , M.D. Neurology , Dokuz Eylül University Izmir, Turkey gorsev.yener@deu.tr

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. FTLD Prevalence. Not clear ( 4- 16%) Common cause pre-senile dementia

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Görsev G. Yener , M.D. Neurology , Dokuz Eylül University Izmir, Turkey gorsev.yener@deu.tr

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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. FTLDin 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) US (N=353) (% male) ** ** Age (Years) Johnson et al. Arch Neurol. 2005;62:925-930. Yener et al 2002

  9. FTLD: Age at Onset US (N=353) Turkish (N=35) ** ? Age (Years) Johnson et al. Arch Neurol. 2005;62:925-930. Yener et al 2002

  10. Presenile onset (%) -Turkish **

  11. Family history • 40-60 % • In Netherland series 38% • (15 million screened) • Family Hx (+) , RR=3.5

  12. Family Hx (%)-Turkish population **

  13. FTLD Pathological SyndromeCore 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. a b c d A. B. Distance travelled by the tau species (m) Time (minutes) 0N4R-EGFP 0N4R-untagged Courtesy of Tim Hutton, Cambridge, UK

  17. Tau mutations

  18. Neuronal ubiquitinated intranuclear inclusions in familial and non-familial FTD-MND associated with ALS Bigio, et al, 2004, J Neuropath Exp Neurol, 63(8): 801 811

  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 3 sites (N=353) Johnson et al. Arch Neurol. 2005;62:925-930. 1 site (N=35) Yener et al 2002

  24. MND and FTDare 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. Brain atrophy in ALS and ALS/FTLD Chang et al, Neurology 2005;65:75–80

  26. Turkish FTLD and MND

  27. Webster scores-Turkish FTLD subtypes 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

  30. MMSE Figure copying (%)

  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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