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Progressive Supranuclear Palsy (PSP) is a relentlessly progressive neurodegenerative disorder, clinically characterized by parkinsonism with prominent axial involvement and postural instability, bulbar symptoms, supranuclear ophthalmoplegia, and executive dysfunction. Abnormal neuronal and glial tau aggregations affecting the basal ganglia and selective brainstem structures result in dysfunction of the five frontosubcortical circuits and brainstem functions. There is no effective treatment for PSP. One of the key feature in the aggregation of tau is its phosphorylation by kinases such as glycogen synthase kinase 3b (GSK3b). Recent reports have shown that valproic acid was able to inhibit the activity of GSK3b and could exert a neuroprotective effect through this inhibition. The investigators thus decided to conduct this controlled study to assess the putative neuroprotective effects in patients with PSP.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| valproic acid | Experimental | Depakine |
|
| Placebo | Placebo Comparator | Placebo |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| valproic acid | Drug | Depakine |
| |
| Placebo |
| Measure | Description | Time Frame |
|---|---|---|
| PSPRS score (specific score for PSP) | This score will be measured every three months during the two-year follow up of the study |
| Measure | Description | Time Frame |
|---|---|---|
| Neuropsychological evaluation | inclusion, one year and two years follow up |
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Inclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Pascal Derkinderen | Nantes University Hospital | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Service de Neurologie A, Hôpital Gabriel Montpied-BP | Clermont-Ferrand | 63009 | France | |||
| Service de Neurologie et Pathologie du mouvement Hôpital Roger Salingro, CHRU de Lille |
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| ID | Term |
|---|---|
| D013494 | Supranuclear Palsy, Progressive |
| ID | Term |
|---|---|
| D001480 | Basal Ganglia Diseases |
| D001927 | Brain Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
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| ID | Term |
|---|---|
| D014635 | Valproic Acid |
| ID | Term |
|---|---|
| D010421 | Pentanoic Acids |
| D014631 | Valerates |
| D000144 | Acids, Acyclic |
| D002264 | Carboxylic Acids |
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| Drug |
Placebo |
|
| Lille |
| 59000 |
| France |
| Service de Neurologie, CHU Nantes | Nantes | 44093 | France |
| Service de Neurologie, CHU Poitiers | Poitiers | 86021 | France |
| D009069 | Movement Disorders |
| D009886 | Ophthalmoplegia |
| D015835 | Ocular Motility Disorders |
| D003389 | Cranial Nerve Diseases |
| D024801 | Tauopathies |
| D019636 | Neurodegenerative Diseases |
| D010243 | Paralysis |
| D009461 | Neurologic Manifestations |
| D005128 | Eye Diseases |
| D012816 | Signs and Symptoms |
| D013568 | Pathological Conditions, Signs and Symptoms |
| D009930 |
| Organic Chemicals |
| D005232 | Fatty Acids, Volatile |
| D005227 | Fatty Acids |
| D008055 | Lipids |