Hereditary Spastic Paraplegia (HSP)
HSP is also known as familial paraparesis or Strumpell-Lorrain syndrome and refers to a group of inherited disorders that affect the leg muscles, causing them to progressively weaken and stiffen over time.
HSP is an inherited condition, whereby an abnormal gene has been carried over from the parents, leading to changes and deterioration of the long nerves in the spine.
The degree of stiffness and weakness in the leg muscles varies between the types of paraparesis. The onset of the disorder can differ greatly from person to person. The initial signs are mild gait difficulties caused by stiffness (spasticity) in the leg muscles. The progression of the disease is typically slow, however gait becomes more difficult, often with uncontrollable shaking of the legs, increased spasticity in the leg muscles, reduced balance and coordination.
A person may have weakness and increased spasticity (stiffness) in the leg muscles often causing the feet to point downwards and the legs to stiffen in gait. This often leads to difficulties clearing the feet and may cause tripping or falling or compensations such as hip hitching to help ambulate. A person may develop tightness in the leg muscles as a result of spasticity and may suffer from leg cramps and muscle spasms.
There is no cure for HSP and a person may increasingly require the assistance of a walking aid or wheelchair as the disorder progresses to help them in their daily life. However, treatments such as muscle relaxants may reduce the incidence of spasticity, and pain associated with cramps and spasms. Orthotic input and physiotherapy may help to maintain and improve the ability to walk, improve strength, reduce pain and help to prevent muscle tightness.
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