Hereditary Spastic Paraplegia

Treatment

The treatment of hereditary spastic paraplegia (HSP) consists of symptomatic and supportive medical management as well as other supportive services, including physical therapy. Although no treatment is currently available that may slow or alter the disease's progression, therapy with baclofen (Lioresal®), a voluntary (i.e., skeletal) muscle relaxant, may help to reduce spasticity in some HSP patients. Baclofen may be administered by mouth (orally) or delivered directly to the spinal canal (intrathecally). Therapy with another skeletal muscle relaxant, dantrolene (Dantrium®), may also improve spasticity in some patients. Other medications that may provide some benefit include zanaflex (Tizanidine®), a medication that has been approved to treat muscle spasms, or the benzodiazepines diazepam (Valium®) or clonazepam (Klonopin®). In addition, in patients with bladder control problems, treatment with oxybutynin (Ditropan®), an involuntary (i.e., smooth) muscle relaxant and spasmolytic agent, may help to relieve spasticity of the bladder.

Some patients may be candidates for chemodenervation. This therapeutic approach may help to reduce muscle overactivity without systemic side effects. For example, some patients benefit from injections of botulinum toxin type A (BOTOX®) into the hip adductors or ankle plantar flexors. In addition, in selected candidates, injections of phenol into the obturator nerve may be of some benefit. In some patients with slowly progressive disease, neuro-orthopedic surgery to lengthen the ankle plantar flexors or hip adductors may be appropriate.

Although physical therapy does not prevent or reduce degenerative changes of the spinal cord, experts indicate that receiving regular therapy may play an important role in helping HSP patients maintain and increase muscle strength and range of motion. In addition, in those with childhood onset, early intervention with such therapy may lead to increased functional mobility and ambulation.

With disease progression, many HSP patients may benefit from the use of certain ambulatory aids, such as canes, crutches, leg braces, or walkers. Less commonly, some patients may require wheelchairs or motorized carts.