Eneida Mioshi, PhD Department of Psychiatry University of ... · 1 Eneida Mioshi, PhD Department of...
Transcript of Eneida Mioshi, PhD Department of Psychiatry University of ... · 1 Eneida Mioshi, PhD Department of...
1
Eneida Mioshi, PhD Department of Psychiatry University of Cambridge School of Clinical Medicine
Knopman et al, Brain, 2008
Disease stage?
Outcome
measures?
Power?
Longitudinal
changes?
2
FRS – Frontotemporal Dementia
Rating Scale
(Also known as FTD-FRS)
3
Very mild
Mild
Moderate
Severe
Very severe
Profound
3.35
1.68
-0.59
-3.09
-6.66
Mioshi et al, Neurology, 2010 4
FRS
Mioshi et al, Neurology, 2010
Disease progression
differs across
FTD variants
5
FRS
CDR can
underestimate disease
severity in FTD
Mioshi et al, Neurology, 2010 6
*
Baseline Follow up-2
-1
0
1
2
Sem Dem
PNFA
bv-FTDLogi
t sco
re d
eclin
e
Mioshi et al, Neurology, 2010 7
Significant decline for all variants
FRS
• 130 patient-carer dyads
• Patients Demographics • 84 males • 63.8±9.1 years • 4.7 years disease duration •34 (26%) bulbar; 96 (74%) limb
RESULTS • Stages:
• Mild (30.8%) • Moderate (63.1%) • Severe (6.2%)
Hsieh et al, ALSFTD, 2013 8
FRS
De Silva et al (in prep)
ALSFRS-R
ALSFRS-R
FTDFRS
FTDFRS
9
FRS
FTLD
(n=252) AD (n=243)
Early susceptibility to
decline in frontal-
dependent abilities
Early susceptibility
to decline in
memory
Different profiles on CDR-FTLD for AD and FTLD
Mioshi & Knopman (in prep)
Late decline in
language and
behaviour
10
Lima-Silva et al, Dementia&Neuropsychologia, 2013
CDR 1 = Mild
11
R = - 0.636
p < 0.001
Mioshi et al, ADAD, 2012
Global improvements
Naming
Activity engagement
Family carer burden
Family carer appraisal of
problems
12
FRS
Mixed ANOVA
Time: F(1,20)
= 5.851, p< 0.05
Group: F(1,20)
= 40.380, p< 0.001
Behaviour: n.s.
Time*group: F(1,20)
= 5.631, p< 0.05
Mioshi et al (in prep)
How much did it bother you?
13
DISINHIBITION
APATHY
FRS: disease staging; longitudinal changes; FTDMND
GENFI: validation in
other European
countries/languages
TRx-237-007; FRM-0334 Reviewing scoring system
Other considerations for
non-pharmacological
trials 14
Australia John Hodges Olivier Piguet Matthew Kiernan Sharpley Hsieh Jashelle Caga Colleen McKinnon Claire O’Connor Cassandra Kaizik James Burrell Emma Devenney
USA David Knopman Brazil Thais Lima-Silva Monica Yassuda Paulo Caramelli Ricardo Nitrini
UK
Michael Hornberger James Rowe Kate Dawson Rachel Winson Julieta de la Llosa Camilla Zugman
All patients and their family carers
All FRS Collaborators
15
15