Il trattamento del Dolore

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Il 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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CMT1A 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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FURTHER PROBLEMS
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2010-2012

• 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).

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AIMS

1. 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
2. Understand whether the respiratory function and
   cardiopulmonary response to exercise is affected
   in CMT1A
3. Study the maintenance of improvement, if any, in
   a six months follow up (FU) time
4. Evaluate the impact of rehabilitation on the
   quality of life.

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CLINICAL 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
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INCLUSION CRITERIA

1. Clinical diagnosis of familial CMT1A. All
   patients will undergo genetic testing before
   screening and only patients with a known gene
   mutation will be recruited.
2. Age between 18 and 65 years.
3. Ability to accomplish the primary outcome measure
   (10m timed walking test) without support, with or
   without AFO
4. 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
5. Score at the Mobility Scale between 2 and 10
6. Score at the Borg Scale between 21 and 45
7. Signed informed patient consent to partecipate

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EXCLUSION CRITERIA

1. Patients affected by HNPP or any other type of
   CMT.
2. Patients with vestibular affections, psychiatric,
   cardiovascular and lung disorders or severe
   arthropathic changes in the lower limbs
   preventing the performance of exercise resistance
   training
3. Other associated causes of neuropathy (diabetes,
   endocrine disorders, vasculitis, herniated disc,
   use of potentially neurotoxic molecules as
   antineoplasti drugs or alcohol consumption)
4. Non-ambulating patients or patients requiring
   even monolateral support to walk
5. Other neurological disorders

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OUTCOME MEASURES

1. 10 - meter walk test (10MWT)
2. 6 minute walk test (6MWT)
3. 12 item walking scale (12WS)
4. Foot extension and foot flexion strength
   determined with a myometer
5. SF 36
6. Ankle angle
7. Berg Scale
8. Mobility Scale
9. Lung function (MEP, FVC, MIP)
10. Peak oxygen consumption during an incremental
    exercise test on treadmill
11. Time to holding
12. FSS
13. Modified Borg Dyspnoea Scale
14. Measuring assessing the metabolic syndrome

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TREATMENT
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
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• 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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Genoa - 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