mri in als · mri in als . background . braak et al., nat rev neurol 2013 . proposed staging of...
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MRI in ALS
Federica Agosta, MD, PhD
Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific
Institute, Vita-Salute San Raffaele University, Milan, Italy
MRI IN ALS Background
Braak et al., Nat Rev Neurol 2013
Proposed staging of pTDP-43 pathology in ALS
Stage 1 Motor cortex, bulbar and spinal somatomotor neurons
Stage 2 Reticular formation, precerebellar nuclei
Stage 3 Prefrontal cortex and basal ganglia
Stage 4 Anteromedial areas of the temporal lobe and the hippocampal formation
MRI IN ALS GM damage / Motor cortex &
clinical correlations
Agos
ta e
t al.,
Plo
sON
E 20
12
p=0.05
p=0.01
p=0.05
T-value
0
-1.68
-4
Anterior
Posterior C-statistic ALS vs. controls 0.75
Progression rate: r=-0.33, p=0.03
ALS vs controls
Bede et al., JNNP 2012
Bulbar and limb-onset ALS vs controls
Vers
trae
te e
t al.,
JNN
P 20
12
t values Agosta et al., Hum Brain Mapp 2007
Agos
ta e
t al.,
Plo
sON
E 20
12
MRI IN ALS GM damage / Extramotor damage & cognition
MND-PLUS vs MND-MOTOR
MND-PLUS vs controls
MND-MOTOR vs controls
Agosta et al., Hum Brain Mapp 2016
MRI IN ALS WM damage / CST
Filip
pini
et a
l., N
euro
logy
201
0
CST FA vs UMN score and disease duration
CST FA decrease in patients without UMN signs at the time of MRI
Sach et al., Brain 2004
Independent predictors of survival: ALSFRS deterioration rate
(p=0.01, HR=2.2, 95% CI=1.2-3.9) CST FA
(p=0.06, HR=0.94, 95% CI=0.89-1.00) Agosta et al., Eur J Neurosci 2010
r=-0.42 p=0.04
Disease progression rate
CST
aver
age
FA
CST damage vs progression rate and survival
MRI IN ALS Extramotor WM damage
Bilateral Partial R2=0.25
Bilateral Partial R2=0.30
Right Partial R2=0.30
Right Partial R2=0.29
Left Partial R2=0.46
Left Partial R2=0.43
Corpus callosum
Right ILF
Left IFOF
Sarro et al., AJNR 2011
Cognitive impairment vs WM damage in ALS
Canu et al., PlosONE 2013
PLS without cognitive deficits vs controls
PLS with cognitive deficits vs controls
FA vs semantic fluency
FA vs action naming
MRI IN ALS Functional connectivity
Douaud et al., Brain 2011
Increased FC Reduced structural connectivity
ALS vs controls
Increased FC in PLS vs controls Sensorimotor Network
Frontal Network
Left frontoparietal Network
Agosta et al., Neurobiol Aging 2013
ALSFRS-R Progr-rate FA CC-PMC
FA CC-SMA
Fluency FA CC-body
Agosta et al., Neurobiol Aging 2013
DMN
Salience
Executive
Right fronto- parietal
Left fronto- parietal
RS networks ALS vs controls
DMN: WCST score vs connectivity of L precuneus (β=-0.087) and L angular gyrus (β=-0.040) Right fronto-parietal: WCST score vs connectivity of R angular gyrus (β=-0.055) and L PCC (β=-0.022)
MRI IN ALS Functional connectivity
MRI IN ALS Network analysis
Verstraete et al., Hum Brain Mapp 2013
Affected subnetwork after 6 months
Carew et al., Neurology 2012
MRI IN ALS Spinal cord
35
40
45
50
55
5 10 15 20 25 30 35 40
Valsasina et al., JNNP 2007
Cord
FA
ALSFRS
r = 0.74 p < 0.001
Controls ALS
0
20
40
60
80
100
0 0.6 1.2 1.8
Cord MD [/1000 mm2 s-1] Cord FA
0
5
10
15
20
25
30
0 0.2 0.4 0.6 0.8 1.0
MRI in ALS Peripheral NS
Gere
vini
et a
l., R
adio
logy
201
6
STIR 3D T2 VISTA
T2 STIR
T1
Nerve root increased signal
Supraspinatus muscle edema
Adipose tissue deposition between trapezius and supraspinatus muscle
Nerve root T2 signal vs disease progression:
r = 0.40
Healthy control
Healthy control
STIR
T2
Neuroimaging Research Unit
Department of Neurology
G. Comi, N. Riva
Department of Neuroradiology
A. Falini
Physicians, Neuropsychologists, & fellows F. Imperiale R. Messina F. Pirro P. Preziosa G. Riccitelli
M. Absinta F. Agosta L. Cacciaguerra E. Canu F. Caso
A. D’Ambrosio E. De Meo P.M. Ferraro S. Fumagalli S. Galantucci
E. Sarasso M.A. Rocca L. Sarro F. Savoldi E. G. Spinelli
Technicians L. Dall’Occhio A. Meani P. Misci M. Petrolini
S. Sala M. Sibilia E. Silvestri R. Vuotto
Physicists S. Basaia M. Copetti E. Molteni
E. Pagani L. Storelli P. Valsasina
OSR DIVISION OF NEUROSCIENCE
Director: M. Filippi