Cognitive and Behavioral Dysexecutive Syndrome in Multiple System Atrophy

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.

Inclusion criteria

• Patients suffering from "probable" MSA according to clinical consensus criteria (Gilman et al, 2008),
• Age >30
• Written informed consent
• Patient covered by the national health system
• The presence of an informant
• Blood negative dosage of the hormone béta-hCG for the women old enough to procreate

Exclusion criteria

• UMSARS IV score > 4 points
• Pregnant woman (blood positive dosage of the hormone béta-hCG) or breast feeding
• Patient under tutelage
• Patient unable to give consent
• Against indication to perform an MRI