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 3,000 to 10,000 persons in France. PSP is characterised by a doparesistant parkinsonism with axial signs such as early gait instability and falls, oculomotor signs such as a vertical gaze palsy, dysphagia and dysarthria, and both cognitive and behavioural disturbances. The latter predominantly manifest as psycho-motor slowness, apathy and frontal executive deficits. Swallowing impairments and falls may lead to life-threatening situations and death occurs 6-9 years after disease onset. Apart from L-dopa which may transiently and inconsistently improve motor symptoms no effective symptomatic, disease-modifying or neuroprotective therapy is presently available to reduce disability in any way. Therefore these patients often receive mostly non-medical care such as physiotherapy and speech therapy. In addition to dopaminergic degeneration there is evidence of cholinergic deficits in PSP correlated with gait and balance impairments . This stands in contrast with the limited number of studies of cholinergic augmentation strategies in PSP. Trials of cholinesterase inhibitors in PSP have produced rather conflicting results: donepezil improves cognition but deteriorates some motor functions whereas a case series of 5 PSP patients treated with rivastigmine found an improvement in several cognitive aspects and no deterioration of motor functions .On the other hand in Parkinson's disease there is convincing evidence of a positive effect of rivastigmine on cognition , apathy and falls Investigators' hypothesis is that rivastigmine (an acetyl- and butyryl-cholinesterase inhibitor) may reduce gait and postural impairment in PSP and may therefore limit the number of falls and their consequences both in terms of injuries sustained (fractures etc...) and on the patients' autonomy. In addition investigators hypothesise that rivastigmine may also reduce the cognitive and behavioural impairment associated with PSP. Taken together these improvements are likely to produce a significant effect on the patients' quality of life and their caregiver burden.
Study Type
INTERVENTIONAL
Allocation
RANDOMIZED
Purpose
TREATMENT
Masking
QUADRUPLE
Enrollment
106
Service de Neurologie et Pathologie du Mouvement Hôpital de La Timone
Marseille, France
Efficacy will be assessed at 4 months as the change from baseline of the number of falls and near falls over the past 2 week
Efficacy will be assessed at 4 months as the change from baseline of the number of falls and near falls over the past 2 weeks using fall postcards sent weekly and filled daily by the subject and caregiver. The number of falls and near-falls will be expressed both as the total number of occurrences over that period and as the number of occurrence adjusted to the distance and number of hours walked (assessed using continuous accelerometer recordings)
Time frame: baseline, 2 weeks, 4 months
Change from baseline at 4 months measured by the global ans sub scores of the PSPRS scale (Progressive Supranuclear Palsy Rating Scale)
Time frame: Baseline and 4 months
Change from baseline at 4 months measured by the Clinical Global Impression scale (patient and caregiver)
Time frame: Baseline and 4 months
Apathy assessed using the Lille Apathy Rating Scale (LARS) both subject and caregiver versions
Time frame: Baseline and 4 months
Assessment of the Quality of life of patient using the PSP-Quality of Life Scale (PSP-QoL)
Time frame: Baseline and 4 months
Assessment of the Quality of life of patient using the EQ-5D
Time frame: Baseline and 4 months
Assessment of the Quality of life of the caregiver using the PSP-Quality of Life Scale (PSP-QoL)
Time frame: Baseline and 4 months
Assessment of the Quality of life of the caregiver using the EQ-5D
Time frame: Baseline and 4 months
Caregiver burden assessed using the Caregiver Reaction Assessement (CRA)
Time frame: Baseline and 4 months
Safety: recording of adverse events at each study visit and at the phone calls
Vital signs, laboratory tests and ECGs will be performed at each study visit to monitor any biological or cardiac adverse event
Time frame: 4 months
This platform is for informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional.