What other names is HSP known under?
The most common names are: Hereditary Spastic Paraparesis/Paraplegia(HSP),
Familial Spastic Paraparesis/Paraplegia (FSP) and Strumpell-Lorrain's disease.
What is HSP?
Hereditary Spastic Paraparesis is an inherited progressive, neurological disorder. It's cause is the degeneration of
the ends of the longer nerves within the spinal cord.
What are the symptoms?
For all forms of HSP, the primary symptoms are spasticity (stiffness), muscle spasm and variable degrees of weakness
in the muscles of the legs (paraparesis). Secondary symptoms include poor balance and loss of function in lower limbs
due to underuse. Because of the progressivity, these symptoms become worse through time. In the beginning, walking becomes
difficult and is in time characterized by toedropping and dragging of the foot. Eventually, the use of mobility aids such
as canes, crutches or wheelchair may be necessary. For HSP onset at childhood, the progression usually slows down after the
body stops growing. Bladder problems might occur in the late stages of the condition.
For complicated HSP, some known additional symptoms are optic neuropathy, retinopathy (disease of the retina),
dementia, ataxia (lack of muscle control), mental retardation and deafness. Some of these combinations of symptoms are only
present with one person or family history of HSP.
When is the onset of symptoms?
Although the majority of patients experience symptoms onset at the age of 20 through 49, symptom onset can happen as
early as infancy or as late as age 85.
What is the prognosis?
The prognosis can not be generalized, because most HSP cases have a pattern of their own. Some are severely disabling,
while others are less disabling. The progressivity worsens the degree of disability through time.
What is the frequency?
The exact frequency of HSP in the US is not known, but in Europe it has been estimated at anywhere from 1 to 13 cases
per 100,000 people. Assuming a frequency of 5 per 100,000 people, less that 15,000 people in the US have HSP.
How does HSP affect the individual's life?
The affect of HSP on social life, work capacity, self-care etc. is highly individual and may vary from none to severe.
What treatment is there?
There is no cure for HSP in itself. The progressivity cannot be stopped, slowed or reversed. Therefore, the treatment
is symptomatic. To reduce spasticity and preserve range of motion, physical therapy, with thorough stretching of affected
muscles, is recommended. To increase strength and mobility, weight training and aquatic exercises are prescribed. Medications
to reduce spasticity and urinary urgency are often helpful.
