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Rehabilitation Program on Genetic and Degenerative Ataxia

Not Applicable
Not yet recruiting
Conditions
Genetic Disease
Degenerative Disease
Ataxia
Interventions
Other: PAMPERO program
Registration Number
NCT06089863
Lead Sponsor
Hospices Civils de Lyon
Brief Summary

Cerebellar ataxia is a pathology linked to the lesion of the cerebellum or the afferent and/or efferent cerebellar pathways. The aetiology can be an acquired cerebral lesion, following a chemical poisoning or a genetic degenerative lesion (for example : Friedreich's ataxia, spinocerebellar ataxias, etc.). As reported by the latest estimate available, genetic degenerative cerebellar ataxias affect approximately 6,000 patients in France (Orpha.net). Symptoms suffered by ataxic patients are : problems and gait disorders along with difficulties in coordination resulting in ataxia, uncoordinated movements.

These symptoms cause a decrease in the quality of life on patients with spinocerebellar ataxia. The symptoms improvement linked to the cerebellar syndrome is based on rehabilitation that can be supplemented by use of technical aids. Current scientific knowledge confirms that intensive rehabilitation by physiotherapy and occupational therapy in patients with degenerative ataxias improves cerebellar symptoms. Nevertheless, the choice rehabilitation technique stay at the appreciation of the therapist.

From the observation, the investigators have designed an intensive multidisciplinary rehabilitation program, called PAMPERO, with partner patients member of two genetic degenerative ataxia patient organisations. This 5-weeks program has been used in clinic during 3 years on 28 patients. It appears to be the only one in France.

The preliminary results show a positive effect on ataxia symptom. Nevertheless, the duration of the benefice over time and the effect on the quality of life stay unknown.

However, the quality of life is mainly affected by the participation restriction due to the risk of falling. The most frequent complaint from partner patient is the diminution of the social interaction resulting of the incapacity to move without risk.

The present protocol aimed at evaluating the Rehabilitation Program in collaboration with partner patient on the symptom intensity, activity and quality of life on genetic and degenerative ataxia.

This PAMPERO program's effect will be assessed by comparing the difference of Intensity of symptom measured by to Scale for the Assessment and Rating of Ataxia (SARA) at inclusion and 3 months after the end of rehabilitation.

Detailed Description

Not available

Recruitment & Eligibility

Status
NOT_YET_RECRUITING
Sex
All
Target Recruitment
48
Inclusion Criteria
  • ≥ 18 years
  • Patients with genetic and degenerative cerebellar ataxia
  • Diagnostic of cerebellar ataxia confirmed by anatomic MRI
  • Affiliated to a social insurgence regime or similar
  • Patients who have given their free, informed and express consent
  • Walking patients (who can walk unsupervised on flat ground with the help of proper technique : categories greater than 4 on the New Functional Ambulation Classification scale)

Non inclusion Criteria:

  • Patients who have already benefited from a hospitalization of more than 3 weeks for rehabilitation during the last 12 months
  • Patients participating simultaneously in another research whose objective would be the evaluation of a therapy, medicinal or not, likely to improve neurological or functional recovery
  • Pregnants, parturient or breastfeeding
  • Patients deprived of their liberty by a judicial or administrative decision
  • Psychiatric care patients
  • Patients admitted to Patients admitted to a health or social establishment for purposes other than research for purposes other than research
  • Major patients protected by the Law
Exclusion Criteria

Not provided

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
PAMPERO program groupPAMPERO programThis program is an intensive multidisciplinary program rehabilitation including Physical, Occupational, Speech, Psychomotor therapy and Adapted Physical Activity.
Primary Outcome Measures
NameTimeMethod
Scale for the Assessment and Rating of Ataxia (SARA)Inclusion ; 3 months after the end of rehabilitation

Intensity of symptom measured by to Scale for the Assessment and Rating of Ataxia (SARA). Scores are from 0 to 42. An higher score is associated with a worse outcome.

Secondary Outcome Measures
NameTimeMethod
Scale for the Assessment and Rating of Ataxia (SARA)Inclusion ; Immediately or 1 week after the end of rehabilitation ; 6 months after the end of rehabilitation

Intensity of symptom measured by to Scale for the Assessment and Rating of Ataxia (SARA). Scores are from 0 to 42. An higher score is associated with a worse outcome.

Score of the Mini-BESTest scaleInclusion ; Immediately or 1 week after the end of rehabilitation ; 3 months after the end of rehabilitation ; 6 months after the end of rehabilitation

Measure of the balance evaluate by Mini-BESTest scale (Mini-Balance Evaluation Systems Test). Scores are from 0 to 28. An higher score is associated with a better outcome.

Light, moderate and vigorous weekly activityInclusion ; Immediately or 1 week after the end of rehabilitation ; 3 months after the end of rehabilitation ; 6 months after the end of rehabilitation

Light, moderate and vigorous activity during one week continuously measured by actimeter ActiGraph wGT3X-BT with EUROCOC

Eyes open postural surface5 weeks

Measure of the balance evaluate by the Eyes open postural surface

Scale of Short Falls Efficacy Scale International (Short FES-I)Inclusion ; Immediately or 1 week after the end of rehabilitation ; 3 months after the end of rehabilitation ; 6 months after the end of rehabilitation

Fear of fall evaluated by to Scale of Short Falls Efficacy Scale International (Short FES-I). Scores are from 7 to 28. An higher score is associated with a worse outcome.

Number of weekly stepsInclusion ; Immediately or 1 week after the end of rehabilitation ; 3 months after the end of rehabilitation ; 6 months after the end of rehabilitation

Number of steps during one week continuously measured by actimeter ActiGraph wGT3X-BT with EUROCOC (European Certificate of Conformity)

Number of patients who stopped PAMPERO program5 weeks

Number of patients who stopped PAMPERO program

Reasons of PAMPERO program stops5 weeks

Reasons of PAMPERO program stops

Displacement length of the center of mass as a function of time eyes open5 weeks

Measure of the balance evaluated by the displacement length of the center of mass as a function of time eyes open

Time of weekly sedentary activityInclusion ; Immediately or 1 week after the end of rehabilitation ; 3 months after the end of rehabilitation ; 6 months after the end of rehabilitation

Time in minutes/day of sedentary activity during one week continuously measured by actimeter ActiGraph wGT3X-BT with EUROCOC

Short Form Health Survey (SF-36)Inclusion ; Immediately or 1 week after the end of rehabilitation ; 3 months after the end of rehabilitation ; 6 months after the end of rehabilitation

Measure of the quality of life evaluate by Short Form Health Survey (SF-36). Scores are from 0 to 100. An higher score is associated with a better outcome.

Number of falls during the study4 weeks ; up to 3 months ; up to 6 months

Risk of falling evaluated by the number of falls during and outside the rehabilitation normalized on 4 weeks

Time to occurrence of PAMPERO program stops5 weeks

Time to occurrence of PAMPERO program stops

Number of patients who completed the entire PAMPERO program5 weeks

Number of patients who completed the entire PAMPERO program (5 weeks)

Trial Locations

Locations (3)

Service de Rééducation Fonctionnelle (S.S.R.) de l'Hôpital Henry Gabrielle Hospices Civils de Lyon

🇫🇷

Saint-Genis-Laval, France

Service de Rééducation Fonctionnelle (S.S.R.) Val Rosay

🇫🇷

Saint-Didier-au-Mont-d'Or, France

Service de Médecine Physique et Réadaptation (M.P.R) de l'Hôpital Bellevue

🇫🇷

Saint-Étienne, France

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