Diapositiva 1

AN EXTENSIVE NEUROPHYSIOLOGICAL ASSESSMENT IN
AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL
DEMENTIA:
DIFFERENCES AND COMMON POINTS
E. Alvisi1, V. Versiglia1, A. Costa2, E. Sinforiani3, S. Bernini3, C. Cereda4, G. Sandrini5, A. Moglia2, M. Ceroni2, E. Alfonsi1
1National
Neurological Institute C. Mondino Foundation, Electromiography Department, University of Pavia; 2University of Pavia, National Neurological Institute C. Mondino
3National Neurological Institute C. Mondino Foundation, Neuropsycology Department; Experimental Neurobiology Laboratory, National Neurological Institute C.Mondino,
5Neurorehabilitation Unit, National Neurological Institute C. Mondino, University of Pavia.
A) TIMELINE OF MOST IMPORTANT EVENTS IN ALS HISTORY
Methods: each subject was clinically assessed with:
- Neurological examination
- ALSFRS-r scale
- Disease progression rate (DPR)
- Body mass index (BMI)
- Forced vital capacity (FVC)
- Neurophysiological study (electroneurography,
electromiography with a multiMUAPs signal detection, magnetic
stimulation). Difference between continous variable were
calculated by t-Student test, between cathegorical variables by
Pearson Chi-Square.
Medelec Synergy
SYNC5-C
Objectives: To assess the differences and a possible clinical
and instrumental link between amyotrophic lateral sclerosis
(ALS), frontotemporal dementia (FTD) and ALS-FTD patients
compared to healthy age-sex matched controls.
Materials: we enrolled all consecutive ALS and FTD patients
according to El -Escorial revised-Awaji /Neary criteria
undergoing
neurophysiological
examination
in
our
Electromiography Department from November 2013 to May
2014 (n=50, 27 ALS, 6 FTD, 4 ALS-FTD, 13 controls).
Introduction: Although evidence of such an overlap at
pathological and genetic levels is now substantial between
frontotemporal dementia (FTD) and motorneuron disease (MND)
(a), the frequency and severity of clinical and subclinical motor
neuron dysfunction in FTD is unknown. In further support of
commonality, patients who present with MND may develop
cognitive symptoms and some may satisfy the diagnostic criteria
for FTD diagnosis. Similarly patients with FTD may subsequently
develop MND.
B) AWAJI CRITERIA: CRITERIA FOR DETECTION OF NEUROGENIC CHANGE
C) UNSTABLE MUP AND MULTIMUAPs analysis
ENG
- Conduzioni sensitive del nervo surale sx;
conduzioni motorie del nervo sciatico
popliteo interno (SPI) con studio onda F,
conduzioni motorie del nervo ulnare con
studio onda F
Latenza motoria distale; ampiezza del potenziale
d‟azione motoria composta (cMAPs); ampiezza
del potenziale d‟azione
sensitivo composto
(SNAP); Velocità di conduzione motoria massima
(MCVmax); Velocità di conduzione sensitiva
antidromica massima (SCVmax); latenza minima
dell‟onda F
EMG
Analisi MultiMUAPs: tibiale anteriore; primo
- Studio con ago-elettrodo coassiale di interosseo dorsale, paravertebrali toracici medi;
distretti muscolari in ambito spinale e genioglosso di sinistra. Registrazione di 20 PUM
bulbare
per
ciascun
distretto
muscolare
e
loro
valutazione di area e durata media
TMS
-
Stimolazione magnetica transcranica
Valutazione del tempo di conduzione centrale
(TCMC) e periferico (TCMP), per derivazione dal
primo interosseo dorsale e dall‟abduttore alluce
di sin: valutazione area del PEM corticale e
periferico (RATIO); SOGLIA CORTICALE;
valutazione dell‟“hot spot” della corteccia
motoria; RECLUTAMENTO PEM CORTICALI
fino a 6 punti sovrasoglia
D) ENG-EMG AND TMS STUDY
E)BARS GRAPHS – UP: CLINICAL PARAMETERS ; DOWN: ENG PARAMETERS
MultiMUAPs study: PUMs duration and area were significantly greater at tibialis anterior and first dorsal interosseous muscle in FTD and ALS
patients (p = 0.001)
Genioglossus muscle and thoracic paravertebral highlight PUM of longer duration in ALS. FTD shows increase in the PUM duration of borderline
significance at the level of the thoracic paraspinal muscles. (p = 0.05)
TCMC: increased bilaterally compared to the controls in the upper limbs.
SOGLIA MEP CORTICALE: markedly lower in ALS patients compared to the controls.
Primo (1) vs secondo (2)
Spinale (0) vs Bulbare (1)
RATIO TIB 1
SX
2
14
0,29
0,18
15
0,51
0,33
RATIO TIB 1
DX
2
14
0,25
0,2
15
0,44
0,3
0,04
0,06
MUAPs pvt sx 0
dur
1
25
12,4
2,97
6
10,52
1,44
MUAPs lin sx 0
dur
1
25
8,41
1,46
6
9,34
0,76
0,04
0,05
Bibliografia
1) M.De Carvalho, R.Dengler et al. Electrodiagnostic criteria for diagnosis of ALS-Clinical Neurophysiology 119 (2008) 497503
2) B Falck , E Stalberg. Multi-MUP EMG analysis in clincial routine-Neurol Neurochir Pol 1996; 30 3; 55-70
3) S.Vucic et al.Transcranial magnetic stimulation and amyotrophic lateral sclerosis: pathophysiological insights. J Neurol
Neurosurg Psychiatry 2013 84: 1161-1170
XLV CONGRESSO NAZIONALE 11-14 OTTOBRE 201 – CAGLIARI
Results: Almost all parameters considered to
recognize specific and subclinical alterations and
electroneurography, the central motor pathways
study and multiMUAPs analysis seems to be very
sensitive allowing a precise quantification of
electromiographic
parameters
despite
the
apparent motor system stability in FTD patients.
Conclusion: suggested by Awaji criteria it is
important
to
perform
an
extensive
neurophysiological examination in ALS suspected
patients but it is to consider it also in
frontotemporal dementia patients, considering
„the indisputable overlap‟ between these two
disorders.
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