Hereditary Spastic Paraplegia (HSP)
Hereditary Spastic Paraplegia (HSP)
refers to a group of degenerative spinal cord disorders
characterized by gradual, progressive weakness and stiffness (spasticity)
of the legs. Approximately 10,000 people in the United States are
afflicted with HSP.
In HSP, the motor nerves that innervate the lower limbs start to
deteriorate. This results in difficulty with balance, weakness and
stiffness of the legs, muscle spasms, and dragging of toes while
walking. Onset is generally during childhood but occasionally
symptoms do not appear until later in life. The severity and
progression of the disease also varies, even among families. Other
symptoms include shortened Achilles tendon, leg cramps,
hyperactive reflexes, and high arch feet (pes cavus).
HSP is classified as either uncomplicated or complicated.
Complicated HSP is rare and differs from uncomplicated HSP by the
presence of other neurological disorders such as ataxia, epilepsy,
optic and retinal neuropathies, mental retardation, ichthyosis and
many others. There is no specific test to diagnose HSP. HSP is
diagnosed only through careful elimination of other diseases that
share common symptoms. Treatment aims to alleviate the symptoms.
Physical therapy is important to maintain muscle strength and
prevent further muscle weakness. Although the disease is
progressive, rarely do patients ever become completely immobile.
Assisted devices such as a cane or walker, however, are usually
needed.
Within the two forms of HSP are also different types classified
according to their mode of inheritance. Autosomal, recessive and
X-linked forms of HSP have been found, the most common of which is
the autosomal dominant form. Genetic analysis to determine the
type of HSP present is now available. Genetic counseling and a
thorough genetic evaluation is strongly recommended to families
with a history of HSP or any other hereditary disorder. Certain
forms of X-linked diseases are more prevalent in one gender over
another. A new option, Preimplantation Genetic Diagnosis (PGD), is
now available to couples in this situation. PGD tests an embryo
for known genetic defects and is able to determine the gender of
an embryo prior to implantation. Implanting only specific embrya,
either male or female depending on prevalence of that specific
disease, will greatly reduce the chances of transmitting the
disease. A genetic counselor will determine whether PGD will be of
benefit to a couple
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Hamstring recession may offer some improvement
How to Keep From
Falling With Hsp
Treatment using
Medicines which reduce Spasticity
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