What Is Hereditary Spastic Paraplegia?
Hereditary spastic paraplegia (HSP), also known as familial paraparesis or Strümpell-Lorrain syndrome, is a group of inherited disorders. It affects the leg muscles, causing them to become progressively weaker and stiffer over time.
HSP occurs when an abnormal gene is passed from parents to a child. This genetic change causes degeneration of the long nerve pathways in the spinal cord and leads to nerve conduction to the legs being affected over time.
How Do the Symptoms Progress?
The degree of stiffness and weakness in the leg muscles varies from person to person and between subtypes. The age at which the disorder begins can also vary over a wide range.
- The first symptoms are usually mild walking difficulties caused by muscle stiffness (spasticity).
- Progression of the disease is typically slow; however, walking may become more difficult over time.
- Uncontrollable trembling in the legs, increasing spasticity, and reduced balance and coordination may occur.
Weakness and increasing stiffness in the leg muscles can cause the feet to point downward and the legs to stiffen during walking. This makes it harder to lift the feet off the ground; it may increase the risk of tripping and falling and may lead to compensatory movements such as hitching the hip up further in order to keep walking. Muscle tightness, leg cramps and muscle spasms may also occur.
The Role of Orthoses and Physiotherapy
There is no known cure for HSP, and as the disorder progresses the person may increasingly need a walking aid or a wheelchair in daily life. Nevertheless, appropriate supportive approaches may contribute to maintaining quality of life.
- Orthotic support: Leg orthoses or ankle-foot orthoses selected in line with the specialist physician's assessment may help make walking easier and reduce the risk of falls by adjusting foot position. In cases where the feet point downward and tripping increases, short leg braces (AFO) may support walking.
- Physiotherapy: Regular exercise programs may help increase strength, prevent muscle tightness and preserve existing walking ability.
- Medical treatments: Medications such as muscle relaxants, prescribed by a physician, may reduce the frequency of spasticity and the pain associated with cramps and spasms.
Orthotic applications and supportive treatments are planned according to the person's needs in line with the specialist physician's prescription and medical report. The type of orthosis and the physiotherapy program are determined according to the stage of the disorder and the person's functional status.
Key Points
- HSP is an inherited and progressive disorder affecting the leg muscles.
- The main symptoms are spasticity, weakness and increasingly difficult walking.
- Orthotic support and physiotherapy may help preserve walking ability, increase strength, reduce pain and prevent muscle tightness.
- All applications are carried out in line with the specialist physician's assessment and prescription.
This content is for informational purposes; you should consult your physician for diagnosis and treatment.
