Progressive supranuclear palsy (PSP) is a rare neurodegenerative disease from the parkinsonian syndrome group.

PSP is characterised by the association of a non-doparesponsive parkinsonian syndrome with axial signs. The latter predominantly manifest as a psycho-motor slowness, an apathy and frontal executive deficits. Swallowing impairments may additionally provoke life-threatening situations. Today the treatment is mostly symptomatic as no cure is available. Given the limited treatment options and its clinical characteristics, PSP deeply impact on the patients' quality of life (QoL) as well as on their caregivers'. Nevertheless a limited number of studies have focused on these aspects. A better understaning of the factors determining both patient and cargiver QoL may help optimising their care.

the principal objective of this study is to identify the determinants of PSP patients' QoL.

The secondary objectives are : i) to identify the determinants (medical, behavioural, socio-economic, environmental …) of PSP patients' caregivers' QoL and burden ; ii) to validate in French language the QoL scale specific for PSP available in English (PSP-QoL).

This is a multidisciplinary transversal study. 2 subject groups will be included : i) PSP patients ; ii) caregiver of PSP patients (designated by the patient as being the person closest to them), Data collected : i) from the patient : socio-demographic, social and professionnal environment, clinical (disease duration and severity, neuropsychological evaluation), therapeutic, mood, anxiety, coping, body image, QoL ; ii) from the caregiver: socio-demographic, social and professionnal environment, connection with the patient, data relative to their own health, mood, anxiety, coping, QoL, burden.

Progress : patient information, designation of a caregiver, consent collection, collection of data Statistical analysis : To address the principal objective 'patient' QoL scores will be confronted to the other collected variables (Student's t-test, correlation coefficient). The results will be adjusted to the confounding factors using multivariate analyses.

Progressive supranuclear palsy (PSP) is a rare neurodegenerative disease from the parkinsonian syndrome group. It represents 5 to 10% of all parkinsonian syndromes and affects 3000 to 10000 persons in France.

PSP is characterised by the association of a non-doparesponsive parkinsonian syndrome with axial signs such as early gait instability and falls, oculomotor signs such as a vertical gaze limitation and both cognitive and behavioural disturbances. The latter predominantly manifest as a psycho-motor slowness, an apathy and frontal executive deficits. Swallowing impairments may additionally provoke life-threatening situations. Today the treatment is mostly symptomatic as no cure is available. Given the limited treatment options and its clinical characteristics, PSP deeply impact on the patients' quality of life (QoL) as well as on their caregivers'. Nevertheless a limited number of studies have focused on these aspects. A better understaning of the factors determining both patient and cargiver QoL may help optimising their care.

Objectives : the principal objective of this study is to identify the determinants (motor, behavioural, socio-economic, environemental…) of PSP patients' QoL.

The secondary objectives are : i) to identify the determinants (medical, behavioural, socio-economic, environmental …) of PSP patients' caregivers' QoL and burden ; ii) to validate in French language the QoL scale specific for PSP available in English (PSP-QoL).

Methods : This is a multidisciplinary transversal study. Population : 2 subject groups will be included : i) PSP patients ; ii) caregiver of PSP patients (designated by the patient as being the person closest to them), Data collected : i) from the patient : socio-demographic, social and professionnal environment, clinical (disease duration and severity, neuropsychological evaluation), therapeutic, mood, anxiety, coping, body image, QoL ; ii) from the caregiver: socio-demographic, social and professionnal environment, connection with the patient, data relative to their own health, mood, anxiety, coping, QoL, burden.

Progress : patient information, designation of a caregiver, consent collection, collection of data Statistical analysis : To address the principal objective 'patient' QoL scores will be confronted to the other collected variables (Student's t-test, correlation coefficient). The results will be adjusted to the confounding factors using multivariate analyses. To address the secondary objective, 'caregiver' QoL and burden scores will be confronted to the other collected variables through the same procedure. The transcultural validation of the PSP-QoL scale will be obtained using standard methodology: forward-backward method, acceptability test, study of metric properties.

Expected results and impact: This work will help understanding the social handicap in PSP and may lead to new therapeutic strategies.

Relevance of the project: No data on PSP-related QoL or caregiver burden are available in France In addition, the factors determining these aspects of the disease are poorly understood. The motor handicap obviously reduces patients' QoL and increases the caregivers' burden. However the impact of the cognitive and behavioural disturbances and particularly the reduction of social interactions on the QoL of patients and caregiver burden have been less studied.

Inclusion Criteria (patients)

Female or male, age ≥ 40 years at the time of onset of symptoms

• diagnosis of possible or probable PSP according to the criteria of Litvan (1996b)
• agreeing to participate in the study
• subject with no severe cognitive impairment (MMS> 20/30)
• subject who does not have psycho-behavioral comorbidity deemed to be severe enough to make his assessment impossible
• subject not presenting a measure of legal protection

Exclusion Criteria (patients)

• Age <40 years at the onset of symptoms
• Diagnosis other than PSP (including other Parkinsonian syndromes)
• Presence of another concomitant neurological disease
• subject with severe cognitive impairment (MMS≤20 / 30)
• subject presenting psycho-behavioral comorbidities considered sufficiently severe to make it impossible to evaluate
• subject under legal protection
• subject not affiliated to a social security scheme

Inclusion Criteria (caregivers)

• Female or male, age ≥ 18 years
• designated by the patient as the person closest to him / her
• agreeing to participate in the study
• subject affiliated to a social security scheme

Exclusion Criteria (caregivers)

• Age <18 years
• Not designated by the patient as the person closest to him / her
• Not agreeing to participate in the study
• subject not affiliated to a social security scheme

PSP patients with their caregivers