Cognitive and Behavioral Dysexecutive Syndrome in Multiple System Atrophy (CogAMS)

Comparison of Cognitive and Behavioral Dysexecutive Syndrome Between Parkinsonian Form and Cerebellar Form of Multiple System Atrophy and Analysis of Correlates With an Imaging Study.

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 .

Study Overview

• Status: Completed
• Conditions: Multiple System Atrophy
• Intervention / Treatment: Other: MRI; Other: Clinical evaluation; Other: neuropsychological battery test; Other: Actigraphy

Detailed Description

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.

• Study Type: Observational
• Enrollment (Actual): 40

Contacts and Locations

Study Locations

• France
  • Bordeaux, France, 33000
    • CHU de Bordeaux

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 26 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

Patients suffering from MSA-P or MSA-C

Description

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

Study Plan

How is the study designed?

Design Details

• Observational Models: Case-Control
• Time Perspectives: Cross-Sectional

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
MSA-P	Other: MRI; Other: Clinical evaluation; Other: neuropsychological battery test; Other: Actigraphy; Actigraphy recording will be performed during 7 days after inclusion.
MSA-C	Other: MRI; Other: Clinical evaluation; Other: neuropsychological battery test; Other: Actigraphy; Actigraphy recording will be performed during 7 days after inclusion.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
Behavioral Dysexecutive Syndrome Inventory scores	At inclusion (day 0)

Secondary Outcome Measures

Outcome Measure	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.	Trail making Test; Stroop Test; Modfied Card Sorting Test; Brixton Test; Verbal fluency Test; Six Elements Test	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

Collaborators and Investigators

• Sponsor: University Hospital, Bordeaux
• Investigators: Principal Investigator: Alexandra FOUBERT-SAMIER, MD, University Hospital, Bordeaux

Study record dates

Study Major Dates

• Study Start (Actual): May 14, 2014
• Primary Completion (Actual): June 25, 2015
• Study Completion (Actual): June 26, 2015

Study Registration Dates

• First Submitted: July 1, 2014
• First Submitted That Met QC Criteria: July 3, 2014
• First Posted (Estimate): July 9, 2014

Study Record Updates

• Last Update Posted (Actual): February 26, 2019
• Last Update Submitted That Met QC Criteria: February 22, 2019
• Last Verified: February 1, 2019

More Information

Terms related to this study

• Keywords: multiple system atrophy; dysexecutive syndrome; imaging study
• Additional Relevant MeSH Terms: Cardiovascular Diseases; Vascular Diseases; Brain Diseases; Central Nervous System Diseases; Nervous System Diseases; Basal Ganglia Diseases; Movement Disorders; Synucleinopathies; Neurodegenerative Diseases; Pathological Conditions, Anatomical; Autonomic Nervous System Diseases; Primary Dysautonomias; Hypotension; Atrophy; Multiple System Atrophy; Shy-Drager Syndrome
• Other Study ID Numbers: CHUBX 2013/07