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study withdrawn by the sponsor for logistical reasons
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Type 1 myotonic dystrophy (MD1) is a genetic and hereditary disease that primarily affects muscle tissue, resulting in myotonia (difficulty relaxing after contraction) and atrophy (progressive muscle weakening with decreased muscle volume). It also affects eyes, heart, endocrine system, gastrointestinal system and central nervous system. Specific cognitive abilities are impaired in patients with MD1 such as attention, visio-spatial or visio-building abilities as well as executive dysfunctions.
Currently, the cognitive assessment of MD1 patients is based on classical neuropsychological tests, which are time-consuming and require a MD1 expert neuropsychologist. Moreover, it is usually very difficult for MD1 patients to accept performing these tests, and when they agree to perform them, they usually give up before the end. This finding is more frequent in MD1 patients with high level of cognitive impairment.
In order to overcome these difficulties in assessing cognitive functions of MD1 patients, the investigators decided to use innovative tools such as virtual reality, which allow individuals to experience a sensory-motor and cognitive experience in a digitally world through a helmet, glasses and joysticks.
The start-up My Cyber Royaume from Lille, in collaboration with the reference center of neuromuscular diseases from Nice coordinated by Pr Sacconi, have developed a software "Good Diag NMD" which uses virtual reality to assess cognitive disorders, more specifically executive functions in patients with type 1 myotonic dystrophy.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Type 1 myotonic dystrophy | Other | Type 1 myotonic dystrophy patients doing classical neuropsychological test and on "Good Diag NMD" software |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Type 1 myotonic dystrophy | Diagnostic Test | To compare classical neuropsychological test and on "Good Diag NMD" software in Type 1 myotonic dystrophy patients |
|
| Measure | Description | Time Frame |
|---|---|---|
| Evaluate the feasibility of the use "Good Diag NMD" software in the evaluation of cognitive executive dysfunctions in patients with dystrophy myotonia 1 | The feasibility "Good Diag NMD" software in patient with type 1 myotonic dystrophy (DM1) will be determined as the rate of patients having completed the Good Diag NMD session in full. Satisfactory feasibility is considered if this rate of patients is greater or equal to 50%. | 0 months |
| Measure | Description | Time Frame |
|---|---|---|
| Establish a possible relationship between the genotype of DM1 patients and the scores obtained during "Good Diag NMD" session | Genotype of DM1 patients will be determined by molecular biology technique, ie the size of GTC triplet expansion will indicate the of severity of the genetic abnormality . Then, we will analyse a possible correlation of the genotype with the scores obtained during "Good Diag NMD" session. |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Sabrina SACCONI | Centre Hospitalier Universitaire de Nice | Principal Investigator |
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| ID | Term |
|---|---|
| D009223 | Myotonic Dystrophy |
| ID | Term |
|---|---|
| D009136 | Muscular Dystrophies |
| D020966 | Muscular Disorders, Atrophic |
| D009135 | Muscular Diseases |
| D009140 | Musculoskeletal Diseases |
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| 0 months |
| To compare the satisfaction score of type 1 myotonic dystrophy patients between classic neuropsychological tests and the Good Diag NMD session | The satisfaction will be measured with a satisfaction questionnaire called "Assessment questionnaire" (value between 0 to 50) | 0 months |
| Compare the scores of "Good Diag NMD" session (scores of perseveration, inhibition and flexibility) with the scores of the classical neuropsychological tests | The following correlations will be examined studied:
| 0 months |
| Establish a possible correlation between the severity of the disease, the severity of muscular impairment and the scores of Good Diag NMD session | 0 months |
| D020967 | Myotonic Disorders |
| D020271 | Heredodegenerative Disorders, Nervous System |
| D019636 | Neurodegenerative Diseases |
| D009422 | Nervous System Diseases |
| D009468 | Neuromuscular Diseases |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |