An overview on pure autonomic failure.

Pure autonomic failure (PAF) is a neurodegenerative disease affecting the sympathetic component of the autonomic nervous system and presenting as orthostatic hypotension (OH). It is a rare, sporadic disease of adults. Although OH is the primary symptom, the autonomic dysfunction may be more generalised, leading to genitourinary and intestinal dysfunction and sweating disorders. Autonomic symptoms in PAF may be similar to those observed in other autonomic neuropathies that need to be ruled out. PAF belongs to the group of α synucleinopathies and is characterised by predominant peripheral deposition of α-synuclein in autonomic ganglia and nerves. However, in a significant number of cases, PAF may convert into another synucleinopathy with central nervous system involvement with varying prognosis: Parkinson's disease (PD), multiple system atrophy (MSA), or dementia with Lewy bodies (DLB). The clinical features, the main differential diagnoses, the risk factors for "phenoconversion" to another synucleinopathy as well as an overview of treatment will be discussed.

Association of a MIND Diet with Brain Structure and Dementia in a French Population.

BACKGROUND: Adherence to the Mediterranean-DASH Intervention for Neurodegenerative Delay (MIND) diet, which combines higher consumption of vegetables, berries, nuts, whole grains, olive oil, fish, beans and poultry, with lower consumption of meat, sugars and saturated fats, is a promising strategy to prevent dementia. However, evidence in populations with non-US food culture, especially from Europe, is limited. OBJECTIVES: To evaluate the association of a French-adapted MIND diet score with gray matter volumes, white matter microstructure and incident dementia. DESIGN AND SETTING: This longitudinal study included participants from the population-based Three-City Bordeaux cohort (≥65 years), with a follow-up from June 2001 to February 2018. PARTICIPANTS: Dementia-free participants at dietary assessment, in 2001-2002, who underwent systematic detection of incident dementia (over up to 7 visits). A subset of the cohort was included in an ancillary MRI study in 2010-2011. MEASUREMENTS: A French-adapted MIND diet score (range, 0-15) was computed from a 148-item Food Frequency Questionnaire and a 24-hour recall administered at home. Incident dementia and its subtypes were adjudicated by an expert committee; and gray matter volumes and white matter microstructure were assessed by 3D-T1 MRI and diffusion-MRI. RESULTS: Among 1,412 participants (mean age, 75.8 [SD, 4.8]; 63% women), followed for a median of 9.7 years (maximum 16.3 years), 356 (25.2%) developed incident dementia. In multivariable-adjusted Cox model, a higher French MIND diet score was associated with lower risks of dementia and AD (hazard ratios for 1-point of score = 0.89 [95% confidence interval, 0.83-0.95] and 0.88 [0.81-0.96], respectively). In Tract-Based Spatial Statistics analysis of 175 participants included in the MRI sub-study, a higher MIND diet score was associated with lower diffusivity values in the splenium of the corpus callosum (P < .05 after Family-Wise Error-correction). In contrast, there was no significant association of the adapted MIND diet score with gray matter volumes in Voxel-Based Morphometry analysis. CONCLUSION: In this cohort of French older adults, higher adherence to the French MIND diet was associated with a lower dementia risk and with preserved white matter microstructure. These results provide further evidence for a role of the MIND diet in the prevention of dementia.

New insights into orthostatic hypotension in multiple system atrophy: a European multicentre cohort study.

OBJECTIVES: Orthostatic hypotension (OH) is a key feature of multiple system atrophy (MSA), a fatal progressive neurodegenerative disorder associated with autonomic failure, parkinsonism and ataxia. This study aims (1) to determine the clinical spectrum of OH in a large European cohort of patients with MSA and (2) to investigate whether a prolonged postural challenge increases the sensitivity to detect OH in MSA. METHODS: Assessment of OH during a 10 min orthostatic test in 349 patients with MSA from seven centres of the European MSA-Study Group (age: 63.6 ± 8.8 years; disease duration: 4.2 ± 2.6 years). Assessment of a possible relationship between OH and MSA subtype (P with predominant parkinsonism or C with predominant cerebellar ataxia), Unified MSA Rating Scale (UMSARS) scores and drug intake. RESULTS: 187 patients (54%) had moderate (> 20 mm Hg (systolic blood pressure (SBP)) and/or > 10 mm Hg (diastolic blood pressure (DBP)) or severe OH (> 30 mm Hg (SBP) and/or > 15 mm Hg (DBP)) within 3 min and 250 patients (72%) within 10 min. OH magnitude was significantly associated with disease severity (UMSARS I, II and IV), orthostatic symptoms (UMSARS I) and supine hypertension. OH severity was not associated with MSA subtype. Drug intake did not differ according to OH magnitude except for antihypertensive drugs being less frequently, and antihypotensive drugs more frequently, prescribed in severe OH. CONCLUSIONS: This is the largest study of OH in patients with MSA. Our data suggest that the sensitivity to pick up OH increases substantially by a prolonged 10 min orthostatic challenge. These results will help to improve OH management and the design of future clinical trials.

Parkinson's disease incidence and prevalence assessment in France using the national healthcare insurance database.

BACKGROUND AND PURPOSE: The incidence and prevalence of Parkinson's disease are important for public health planning yet there is a lack of representative, up-to-date estimations for France. METHODS: For this cross-sectional study, subjects with suspected Parkinson's were identified in the EGB database, a 1/97 random sample of the national healthcare insurance database, linked to the national hospital-discharge summary database. Incidence and prevalence were estimated using a specific definition that included those with a diagnosis (hospitalization or listed as a long-term chronic disease for full reimbursement) and a sensitive definition that also included those with an indicative drug reimbursement profile. Estimations were extrapolated to the national population, standardizing on age and gender. RESULTS: According to either the specific or the sensitive definitions, the annual incidence of Parkinson's disease during the study period was respectively 36 and 49 per 100,000 person-years and prevalence in 2010 was 308-410 per 100,000 persons in the population as a whole. According to the age groups 55-64, 65-74, 75-84 and ≥85 years incidence was respectively 33-46, 139-172, 301-363 and 442-560 per 100,000 person-years amongst men and 32-55, 81-117, 203-270 and 251-313 per 100,000 person-years amongst women. The 2010 prevalence stratified by the same age groups was 293-376, 898-1161, 2524-3011 and 3760-4578 per 100,000 persons amongst men and 199-351, 618-889, 1910-2433 and 2504-3263 per 100,000 persons amongst women. CONCLUSIONS: The specific and sensitive definitions of disease bracket the true values; the relatively small range indicates that the current study provides good estimations of incidence and prevalence of Parkinson's disease for recent years in France.

Change in leisure and social activities and risk of dementia in elderly cohort.

OBJECTIVE: To investigate the association of the change in practice of leisure and social activities with dementia risk taking into account the evolution of cognitive performances. DESIGN, SETTING, AND PARTICIPANTS: From the PAQUID prospective cohort re-examined every 2 years until the 20-year follow-up since 1988, 1461 subjects were seen at 10th year of follow-up. Engagement in 10 leisure and social activities was collected at baseline and at the 10-year follow-up visit for 805 subjects. Four categories of change in activity engagement were considered: subjects who remained active; remained inactive; became inactive and became active. Adjustment on confounders (age, gender, educational level, diabetes, stroke and depression) and rate of evolution of cognitive performances was made with the multivariate Cox proportional hazards model. MAIN OUTCOME MEASURES: Time to incident cases of dementia occurring between the 10th and the 20th year of follow-up. RESULTS: A total of 258 incident dementia cases were documented. The risk of dementia was lower for subjects remaining or becoming active (cumulative risk of dementia: 30%) compared to those remaining or becoming inactive (52% and 42%, respectively) (p<0.0001). Multivariate adjustment including rate of cognitive decline during the first decade of FU did not change these relationships. CONCLUSION: This prospective cohort study suggests a significant association between change in leisure and social activities during old age and risk of dementia.

[Relationship between educational level and dementia: social factor and age-related chronic disease]. / Relation entre facteur social et maladie chronique liée à l'âge: l'exemple du niveau d'études et des démences.

Dementia is an age-related chronic syndrome, whose the first cause is a neurodegenerative disease: Alzheimer's disease (AD). In spite of some controversies, educational level is now considered as a major risk factor for dementia and AD. The protective effect of a high level of education could be related to a preservation of cognitive reserve and a reinforcement of brain reserve. Moreover, subjects with a high level of education have a better access to health care and a better management of vascular risk factors. With the general improvement of the educational level, the age-related incidence of AD and dementia should decrease in the future.

Validation of the French version of the MSA health-related Quality of Life scale (MSA-QoL).

INTRODUCTION: Multiple system atrophy (MSA) has considerable impact on health-related quality of life. The MSA health-related Quality of Life scale (MSA-QoL) is a patient-reported questionnaire, which has been recently designed to evaluate the quality of life in MSA. The objective of the present study was to validate the French version of the MSA-QoL questionnaire. METHODS: One hundred and thirty-six consecutive MSA patients were included in the study. Four patients with more than 10% missing responses were excluded from the final analysis. Data quality, scaling assumptions, acceptability, reliability and validity were assessed similar to the original validation of the English version. RESULTS: Missing responses were low, item and subscale scores were evenly distributed and floor and ceiling effects were negligible. Item-total correlations were higher than the recommended greater than 0.30 and internal consistency was high for all subscales. Test-retest reliability was good for all subscales. Validity was supported by moderate interscale correlations between the subscales and the predicted correlations with other scales assessing motor disability, activities of daily living, quality of life and mood. DISCUSSION: The French version of the MSA-QoL displays robust psychometric properties similar to the English version. CONCLUSION: The French version of MSA-QoL seems suitable for assessing quality of life in French speaking MSA patients.

A long-term follow-up of weight changes in subthalamic nucleus stimulated Parkinson's disease patients.

Deep brain stimulation of the subthalamic nucleus (STN-DBS) constitutes the mainstay treatment in advanced Parkinson's disease (PD) with motor fluctuations. Despite its efficacy on motor signs and quality of life, emergent adverse events have been recently reported. Among them, weight gain (WG) is a recognized adverse event of subthalamic nucleus deep brain stimulation (STN-DBS) in Parkinson's disease (PD). Also, WG is poorly known at the long-term and predisposing factors have not yet been identified. We conducted a cross-sectional study of WG in 47 STN-DBS PD patients between 1999-2006. Data on disease history, motor status and dopaminergic drug treatment were retrospectively collected at surgery and 1 year post-surgery. Weight at disease diagnosis and at surgery, as well as the current weight and height were gathered by an autoquestionnaire. Moreover, the weight before surgery was obtained and verified in medical files in more than 90% of our patients. Sixty-six patients who underwent surgery between 1999-2006 were included, but six were deceased, four refused to participate and nine were lost for follow-up. So, 47 (71%) were retained in our analysis. A total of 78.7% of patients gained weight. On average 4.7 years follow up after surgery, the mean weight gain was +7.2±8.1kg compared to the preoperative assessment (p<0.001) and the mean BMI gain was +2.7±3.0kg/m(2) compared to pre-surgery values (p<0.001). The patients gained more weight after surgery than they had lost during disease evolution before surgery. Women and patients with a more severe UPDRS-III "off" drug score before surgery significantly gained more weight. Our study provides further evidence that the WG is a problem after STN-DBS and concerns a majority of patients at the long term. It may expose them to complications that should be considered for prevention and the patient's information before surgery.

[Guillain-Barré syndrome secondary to cranial surgery: direct or fortuitous relationship?]. / Syndrome de Guillain-Barré secondaire à une craniectomie: association fortuite ou relation directe?

The Guillain-Barré syndrome, an acute polyradiculonevritis (PRN), usually appears after a post-infectious immuno-allergic reaction. We report a case of acute PRN following cranial surgery. Post-surgical Guillain-barré syndrome is rarely described. We reviewed the pathophysiological mechanisms possibly involved in this disease that have to be evoked in patients with abnormal post-surgical neurological symptoms.