What are leukodystrophies?
Leukodystrophies are a group of genetic diseases
affecting the myelin of the central nervous system.
Myelin, also called “white matter,” is made up of a
fatty substance whose purpose is to insulate the nerves
found in the brain (central nervous system) as well as
those that control all the muscles in our body (peripheral
nervous system). Its role is comparable with that of
insulation found on electric wires.
This insulating layer allows the electric signals to
travel correctly through our nerves. One thing that
leukodystrophies do is to deteriorate the mechanisms
that make it possible to form or maintain this very
important protective layer. There are several forms of
this disease. All leukodystrophies are degenerative,
which means that this sheath deteriorates at a rate that
differs from one form of the disease to another. That is
why the physical condition of children affected by this
disease can sometimes deteriorate very quickly.
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