Familial Spastic Paraplegia
Background
Familial Spastic Paraplegia: Hereditary Spastic Paraplegia; Strumpell disease
Familial Spastic Paraplegia (FSP) describes a group of largely progressive conditions predominantly affecting the legs. As the name suggests there are three main features: the legs become stiff (spasticity); there is a variable amount of weakness (paraplegia) and there is a strong genetic element.
At a clinical level there are two forms of this condition: pure and complicated. The pure form is not associated with additional features, whereas the complicated form is clinically very variable with many different additional features. The pure form is far commoner then the complicated variety. However, it is now established that the situation at a genetic level is even more complicated with several different genes proving responsible for the pure form of FSP.