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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| PAMPERO program group | Experimental | This program is an intensive multidisciplinary program rehabilitation including Physical, Occupational, Speech, Psychomotor therapy and Adapted Physical Activity. |
|
| Usual Care intervention group | No Intervention | Usual Rehabilitation of the patient after the inclusion. 0 to 2 physical therapy sessions and 0 to 2 Speech therapy sessions each week usually constitute this program. This rehabilitation highly depends on the patients usual preferences. Some patients have no rehabilitation in the daily care while some have more. |
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| PAMPERO program | Other | This program is an intensive multidisciplinary program rehabilitation including Physical, Occupational, Speech, Psychomotor therapy and Adapted Physical Activity. The duration of this program is 5 weeks and each week is divided as follow :
|
| Measure | Description | Time Frame |
|---|---|---|
| Scale for the Assessment and Rating of Ataxia (SARA) | 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. | Inclusion ; 3 months after the end of rehabilitation |
| Measure | Description | Time Frame |
|---|---|---|
| Scale for the Assessment and Rating of Ataxia (SARA) | 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. | Inclusion ; Immediately or 1 week after the end of rehabilitation ; 6 months after the end of rehabilitation |
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Inclusion Criteria:
Non inclusion Criteria:
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Lucas PIGNON | Contact | 04 78 86 50 58 | +33 | lucas.pignon@chu-lyon.fr |
| Christelle MAROLHO | Contact | 04 72 11 57 68 | +33 | christelle.marolho@chu-lyon.fr |
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Service de Rééducation Fonctionnelle (S.S.R.) Val Rosay | Saint-Didier-au-Mont-d'Or | 69370 | France |
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| ID | Term |
|---|---|
| D001259 | Ataxia |
| D030342 | Genetic Diseases, Inborn |
| D002524 | Cerebellar Ataxia |
| ID | Term |
|---|---|
| D020820 | Dyskinesias |
| D009461 | Neurologic Manifestations |
| D009422 | Nervous System Diseases |
| D012816 | Signs and Symptoms |
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|
| Score of the Mini-BESTest scale |
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. |
| Inclusion ; Immediately or 1 week after the end of rehabilitation ; 3 months after the end of rehabilitation ; 6 months after the end of rehabilitation |
| Eyes open postural surface | Measure of the balance evaluate by the Eyes open postural surface | 5 weeks |
| Displacement length of the center of mass as a function of time eyes open | Measure of the balance evaluated by the displacement length of the center of mass as a function of time eyes open | 5 weeks |
| Scale of Short Falls Efficacy Scale International (Short FES-I) | 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. | Inclusion ; 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 weekly steps | Number of steps during one week continuously measured by actimeter ActiGraph wGT3X-BT with EUROCOC (European Certificate of Conformity) | Inclusion ; Immediately or 1 week after the end of rehabilitation ; 3 months after the end of rehabilitation ; 6 months after the end of rehabilitation |
| Time of weekly sedentary activity | Time in minutes/day of sedentary activity during one week continuously measured by actimeter ActiGraph wGT3X-BT with EUROCOC | Inclusion ; 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 weekly activity | Light, moderate and vigorous activity during one week continuously measured by actimeter ActiGraph wGT3X-BT with EUROCOC | Inclusion ; Immediately or 1 week after the end of rehabilitation ; 3 months after the end of rehabilitation ; 6 months after the end of rehabilitation |
| Short Form Health Survey (SF-36) | 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. | Inclusion ; 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 falls during the study | Risk of falling evaluated by the number of falls during and outside the rehabilitation normalized on 4 weeks | 4 weeks ; up to 3 months ; up to 6 months |
| Number of patients who completed the entire PAMPERO program | Number of patients who completed the entire PAMPERO program (5 weeks) | 5 weeks |
| Number of patients who stopped PAMPERO program | Number of patients who stopped PAMPERO program | 5 weeks |
| Time to occurrence of PAMPERO program stops | Time to occurrence of PAMPERO program stops | 5 weeks |
| Reasons of PAMPERO program stops | Reasons of PAMPERO program stops | 5 weeks |
| Service de Médecine Physique et Réadaptation (M.P.R) de l'Hôpital Bellevue | Saint-Etienne | 42100 | France |
|
| Service de Rééducation Fonctionnelle (S.S.R.) de l'Hôpital Henry Gabrielle Hospices Civils de Lyon | Saint-Genis-Laval | 69230 | France |
|
| D013568 |
| Pathological Conditions, Signs and Symptoms |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D002526 | Cerebellar Diseases |
| D001927 | Brain Diseases |
| D002493 | Central Nervous System Diseases |