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.
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
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)
PSP patients with their caregivers
Cohort
Prospective
PSP-QoL
Other interventionPSP-QoL is a patient-reported outcome measure for progressive supranuclear palsy (PSP). The Supranuclear Palsy Quality of Life scale (PSP-QoL) may be a helpful patient-reported scale for clinical trials and studies in PSP. PSP-QoL consists of 28 items scored on a 3- or 5-point Likert scale, with the total score ranging from 0 to 100. Each item is scored from either 0 to 4, with the exception of four items, which are scored from 0 to 2, with higher scores indicating more-severe disability or movement abnormality. Items are in six categories: daily activities (by history); behavior; bulbar; ocular motor; limb motor; and gait/midline. The scale includes comments and/or instructions for each item and word anchors to explain the ratings.
score of PSP-QoL questionnaire
the score obtained by group will be compare Progressive Supranuclear Palsy (PSP) Quality of Life questionnaire by Schrag and collaborators in 2006. 14 items, 5 degree from no problem, slight problem, moderate problem, marked problem to extreme problem (1 to 5 points) minimum score:14 maximum score : 70
Click on the lead sponsor to view all of their trials.
