The purpose of this project is to study the efficacy of three candidate molecules (Xenbilox, Tahor and Resveratrol) in order to decrease the production of oxysterols by reducing the synthesis of cholesterol and/or regulate the production of bile acids and/or enabling neuroprotective action within the motor neuron.
The primary objective of the study is: \- decrease the accumulation of metabolites which can have a negative impact on neurological and systemic function of patients with SPG5. The secondary objectives of the study are: * confirm the clinical and biological tolerance of the different candidate molecules under study * improve the serum bile acid profile of patients with SPG5
Study Type
INTERVENTIONAL
Allocation
RANDOMIZED
Purpose
TREATMENT
Masking
NONE
Enrollment
12
Pitié-Salpêtrière Hospital
Paris, France
Change of 27-hydroxycholesterol blood level after 2 months of treatment for each treatment
27-hydroxycholesterol will be measured on fasten blood samples for the 12 patients included before and after the 2-month treatment period for each treatment
Time frame: 2 months
Modification of the serum acid profile after a 2-month treatment measured in blood and urine samples
In blood and urines samples of the 12 patients, the following criteria will be assesed in order to observe an evolution of the acid profile: * total biliary acids * ratio of primary/secondary biliary acids * the ratio cholic acids/chenodeoxycholic acids * the ratio chenodeoxycholic acids/ lithocholic acids
Time frame: 2 months
Change of 25-hydroxycholesterol blood level after 2 months of treatment for each treatment
25-hydroxycholesterol will be measured on fasten blood samples for the 12 patients included before and after the 2-month treatment period for each treatment
Time frame: 2 months
Number of participants with adverse events related with digestive problems
During the study, adverse events related with digestive problems will be evaluate in order to asses the clinical tolerance to each treatment
Time frame: 18 months
Number of partcipants with adverse events related with standard biological parameters
Biological tolerance will be evaluate on patients blood samples at each visit. The following criteria will be assessed: * ionogram and fasting glucose * kidney and liver function with urea, creatinine,lipase, amylase, bilirubine, creatine phosphokinase, transaminases,sodium, potassium, calcium, phosphorus
Time frame: 18 months
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