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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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slide1
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
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)

ftld in izmir turkey
FTLDin Izmir, Turkey

Demographic data

Genetics/Pathological Syndrome

Neary Criteria – FTD, PA, SD

MND association

slide4
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 %

ftld clinical heterogeneity
FTLD Clinical Heterogeneity
  • Genetic & sporadic cases
  • Histology varies
  • Motor overlap with PSP, CBD, ALS
neary criteria
Neary Criteria
  • Frontotemporal Lobar Degeneration
    • Frontotemporal Dementia
    • Progressive Non-Fluent Aphasia
    • Semantic Dementia

Neary et al. 1998, Neurology

dokuz eyl l university dementia and movement disorders clinics
Dokuz Eylül University Dementia and Movement Disorders Clinics
  • 1994-2001
  • 35 FTLD, 25 PSP, 5 CBD cases
    • Demographic and clinical features
    • Webster
    • MMSE (subitems)
ftld gender
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

ftld age at onset
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

family history
Family history
  • 40-60 %
  • In Netherland series 38%
    • (15 million screened)
  • Family Hx (+) , RR=3.5
ft l d pathological syndrome core features
FTLD Pathological SyndromeCore Features
  • Frontotemporal predominance
  • Gliosis, spongiosus, neuronal loss
variable histological features
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)
slide16

Pick Bodies Cerebral cortex

(Described by Alzheimer in 1911)

slide19

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

slide21

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

slide22

FTD with ubiquitinated neuronal inclusions and visuospatial impairment

Meiner et al Neurology 2005;65:478–480

ubiquitin and tau
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
ftd neuropathology subtypes
FTD Neuropathology Subtypes
  • FTD-Ub related to MND subtype
  • CBD, PSP major tau subtype
  • DLDH less common with new staining techniques
clinical features
Clinical Features
  • Subtypes
  • MND
  • Parkinsonism
  • MMSE
ftld subtypes in the us and turkey
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

mnd and ftd are associated
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
slide28

Brain atrophy in ALS and ALS/FTLD

Chang et al, Neurology 2005;65:75–80

webster scores turkish ftld subtypes
Webster scores-Turkish FTLD subtypes

Clinical Features-Parkinsonism

**

clinical features1
Clinical Features
  • Parkinsonism appears earlier in FTLD
  • MND - more often parkinsonism
  • No tau mutation was found in FTD-MND group.
clinical patterns
Clinical patterns

PSP~CBD

  • Both higher Webster and MMSE scores
  • MMSE figure copy is discriminative
clinical patterns1
Clinical patterns
  • Webster scores
      • FTD-MND > FTLD
  • MMSE scores
      • FTD-MND = FTLD
  • preserved figure copying
      • FTD-MND > FTLD
clinical patterns2
Clinical patterns

FTLD subtypes

  • total MMSE PNFA < SD < FTD
  • figure copy SD < PNFA < FTD
conclusions
Conclusions
  • FTLD common in presenile ages
  • Turkish FTLD subtypes have similar profile to the US
      • FTD> PNFA > SD
conclusions1
Conclusions
  • Higher family Hx than other dementias (54%)
  • FTLD-MND association (22%)
  • FTD-MND has higher parkinsonism scores