Hereditary spastic paraplegia (HSP), also called familial spastic paraparesis (FSP), refers to a group of inherited disorders that are characterized by progressive weakness and spasticity (stiffness) of the legs.
Hereditary spastic paraplegia affects less than 200,000 people in the North American population. There are no differences in rate relating to gender. Average age at onset is 24 years old.
Early in the disease course, there may be mild gait difficulties and stiffness. These symptoms typically slowly progress so that eventually individuals with HSP may require assistance of a cane, walker, or wheelchair. Though the primary features of HSP are progressive lower extremity spasticity and weakness, more complicated forms may be accompanied by other symptoms. These symptoms include:
- Conditions that affect the optic nerve and retina of the eye
- Ataxia (lack of muscle coordination)
- Cognitive impairment
- Peripheral neuropathy
The diagnosis of HSP is primarily by neurological examination and testing to rule out other disorders. Several genetic mutations have been identified which underlie some of the forms of HSP, and specialized genetic testing and diagnosis are available at some medical centers.
The prognosis for individuals with HSP varies. Some individuals are very disabled and others have only mild disability. The majority of individuals with uncomplicated HSP have a normal life expectancy.
There are no specific treatments to prevent, slow, or reverse HSP. Symptomatic treatments used for spasticity are sometimes helpful. Regular physical therapy is important for muscle strength and to preserve range of motion.