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The main objective of the study is to compare the score to the Behavioral Dysexecutive Syndrome Inventory (BDSI) between Parkinsonian Multiple system Atrophy MSA-P patients and cerebellar Multiple System Atrophy (MSA-C) patients matched on disease duration, age (± 7 years) and sex .
Multiple system atrophy (MSA) is a rare neurodegenerative disorder which is characterized by a variable combination of parkinsonism, cerebellar dysfunction, autonomic failure, and additional signs. According to the consensus criteria for the diagnosis of MSA, the presence of dementia is considered an exclusion criteria. However, several studies have reported cognitive impairment in patients with MSA, dominated by a dysexecutive syndrome. Some studies have compared the cognitive profile of MSA-P patients and MSA-C but any have studied the behavioral dysexecutive syndrome in this population. In 2010, the GREFFEX has established criteria for dysexecutive syndrome and described two distinct parts: the dysexecutive syndrome and cognitive behavioral dysexecutive syndrome. Our clinical impression is that according to the type of MSA, the dysexecutive syndrome seems to be different : the MSA-P patients seem to have a cognitive dysexecutive syndrome and the MSA-C patients a behavioral dysexecutive syndrome.
We want to compare the BDSI score between MSA-P patients and MSA-C patients matched on disease duration, age and sex The specific involvement of the cerebellum in behavioral disorders could explain the behavioral differences experienced by clinicians caring for these patients but never demonstrated. The presence of these disorders could have an impact on the relationship between the patient and the caregiver. In addition, few studies have correlated with cognitive imagery data. Therefore, the exact areas involved in cognitive and behavioral MSApatients remain poorly understood.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| MSA-P |
| ||
| MSA-C |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Clinical evaluation | Other |
| ||
| neuropsychological battery test |
| Measure | Description | Time Frame |
|---|---|---|
| Behavioral Dysexecutive Syndrome Inventory scores | At inclusion (day 0) |
| Measure | Description | Time Frame |
|---|---|---|
| To compare the subscores of the BDSI between MSA-P patients and MSA-C patients. | At inclusion (Day 0) | |
| To compare the cognitive tests exploring the cognitive dysexecutive syndrome between MSA-P patients and MSA-C patients. |
|
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Inclusion criteria:
Exclusion criteria:
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Patients suffering from MSA-P or MSA-C
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| Name | Affiliation | Role |
|---|---|---|
| Alexandra FOUBERT-SAMIER, MD | University Hospital, Bordeaux | Principal Investigator |
| Rodolphe THIEBAUT, MD, PhD | University Hospital, Bordeaux | Study Chair |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| CHU de Bordeaux | Bordeaux | 33000 | France |
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| ID | Term |
|---|---|
| D019578 | Multiple System Atrophy |
| ID | Term |
|---|---|
| D054969 | Primary Dysautonomias |
| D001342 | Autonomic Nervous System Diseases |
| D009422 | Nervous System Diseases |
| D001480 | Basal Ganglia Diseases |
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| ID | Term |
|---|---|
| D056044 | Actigraphy |
| ID | Term |
|---|---|
| D008991 | Monitoring, Physiologic |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
| D061725 | Accelerometry |
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| Other |
|
| MRI | Other |
|
| Actigraphy | Other | Actigraphy recording will be performed during 7 days after inclusion. |
|
| At inclusion (Day 0) |
| To research correlations between scores UMSARS and cognitive and behavioral scores for MSA-P patients and MSA-C patients. | Assessment using the Behavioral Dysexecutive Syndrome Inventory | At inclusion (Day 0) |
| To compare atrophied brain areas, the anatomic and functional abnormalities of neural networks between MSA-P patients and MSA-C patients. | At inclusion (Day 0) |
| To research correlations between imaging abnormalities and cognitive test performance in MSA patients. | At inclusion (day 0) |
| Actigraphy | Sleep analysis : Sleep / wake cycles and sleep fragmentation | During 7 days after inclusion |
| D001927 | Brain Diseases |
| D002493 | Central Nervous System Diseases |
| D009069 | Movement Disorders |
| D000080874 | Synucleinopathies |
| D019636 | Neurodegenerative Diseases |
| D008919 |
| Investigative Techniques |