Title: Il trattamento del Dolore
1Il pilastro fragile neuromotolesioni dal deficit
alla strategia dellarto inferiore Charcot-Marie-T
ooth
La riabilitazione primi passi verso le
evidenze Angelo Schenone Dipartimento di
Neuroscienze, Oftalmologia e Genetica Università
di Genova
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3CMT1A possible therapeutic strategies based on
molecular evidence
First line
Protection of SC and axon
Second line
Blocking influx of Ca
Neurotrophic factors
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24FURTHER PROBLEMS
252010-2012
- 92 patients (23 per center)
- Multicentre, prospective, randomised, single
blind, controlled study - 3 months run in time
- 3 months of treatment
- Two groups
- A) Treadmill, Stretching and Proprioceptive
exercises (TreSPE) (46 pts) - B) Stretching and Proprioceptive exercises
(SPE) (46 pts) - Evaluations before (T0) and after therapy (T1)
- FU phase (6 months).
26AIMS
- Clarify if aerobic exercise, based on a tightly
controlled program at the treadmill, is well
tolerated by CMT1A patients and, eventually, may
improve their ability to walk - Understand whether the respiratory function and
cardiopulmonary response to exercise is affected
in CMT1A - Study the maintenance of improvement, if any, in
a six months follow up (FU) time - Evaluate the impact of rehabilitation on the
quality of life.
27CLINICAL PLAN
Group Months Months Months Months Months
Group 0 - 3 3 - 6 6 - 9 9 - 15 15 24
1 Operators Training Run in time TreSPE Follow up Data Elaboration
2 Operators Training Run in time SPE Follow up Data Elaboration
screening
T1 T2 T3
DONE BY A BLIND EXAMINER
28INCLUSION CRITERIA
- Clinical diagnosis of familial CMT1A. All
patients will undergo genetic testing before
screening and only patients with a known gene
mutation will be recruited. - Age between 18 and 65 years.
- Ability to accomplish the primary outcome measure
(10m timed walking test) without support, with or
without AFO - Ability to walk on treadmill on a horizontal
plane for 20 minutes at a speed of 1.5 km/ h,
with or whithout support at the bars - Score at the Mobility Scale between 2 and 10
- Score at the Borg Scale between 21 and 45
- Signed informed patient consent to partecipate
29EXCLUSION CRITERIA
- Patients affected by HNPP or any other type of
CMT. - Patients with vestibular affections, psychiatric,
cardiovascular and lung disorders or severe
arthropathic changes in the lower limbs
preventing the performance of exercise resistance
training - Other associated causes of neuropathy (diabetes,
endocrine disorders, vasculitis, herniated disc,
use of potentially neurotoxic molecules as
antineoplasti drugs or alcohol consumption) - Non-ambulating patients or patients requiring
even monolateral support to walk - Other neurological disorders
30OUTCOME MEASURES
- 10 - meter walk test (10MWT)
- 6 minute walk test (6MWT)
- 12 item walking scale (12WS)
- Foot extension and foot flexion strength
determined with a myometer - SF 36
- Ankle angle
- Berg Scale
- Mobility Scale
- Lung function (MEP, FVC, MIP)
- Peak oxygen consumption during an incremental
exercise test on treadmill - Time to holding
- FSS
- Modified Borg Dyspnoea Scale
- Measuring assessing the metabolic syndrome
31TREATMENT
Treadmill Training for 40 5 warm up 20
aerobic exercise (gradually incremented to 30
from session to session) 5 warm down RePh
for 20 Positive Espiratory Pressure (PEP)
Bottle for 10 Postural training according to
the Meziers Technique for the other
10 Stretching for 20 both at the bed and in
static position Proprioceptive and postural
kinesitherapy
Tr treadmill PR proprioceptive exercise RePh
respiratory exercise STR stretching
exercise BE balance exercise Treatments in red
will be carried out by physioterapist
1 Treatments in black will be carried out by
physioterapist 2
0 - 20 20 - 40 40 - 60 60 - 80 80 - 100 100 - 120
Pt 1 TrA TrA PR B RePhC STR D BE E
Pt 2 STR D PR B Tr A Tr A BE E RePhC
Pt 3 STR D BE E PR B RePhC TrA TrA
Pt 4 PR B STR D BE E RePhC
Pt 5 BE E STR D RePhC PR B
Pt 6 BE E STR D RePhC PR B
32- Treadmill, stretching, proprioceptive exercises
- MRC
- Tinetti Balance Scale
- Physical Performance Battery
- Ankle Angle
- Oxygen Consumption and Lung Function Tests
- Peak Treadmill Velocity and Slope
- 6MTW
- CMTNs
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37Genoa - Prof. A. Schenone
Rome Prof. L. Padua Dep. Neurosciences,
Ophthalmology and Genetics Don
Gnocchi Foundation University of Genoa
Milan Dr. D. Pareyson
Verona Prof. G.M. Fabrizi I.R.C.C.S. Foundation
Dep. Neurological and Visual Sciences Neurologica
l Institute "Carlo Besta"
University of Verona