eMedicine Specialties > Physical Medicine and Rehabilitation > Disorders of the Motor Unit

Hereditary Spastic Paraplegia: Treatment & Medication

Author: Nam-Jong Paik, MD, PhD, Chief, Associate Professor of Rehabilitation Medicine, Rehabilitation Medicine, Seoul National University Bundang Hospital
Coauthor(s): Jae-Young Lim, MD, Assistant Professor, Department of Rehabilitation Medicine, Division of Musculoskeletal Rehabilitation, Bundang Hospital, Seoul National University College of Medicine
Contributor Information and Disclosures

Updated: Jul 29, 2008

Treatment

Rehabilitation Program

Physical Therapy

Regular physical therapy (PT) is important for maintaining and improving range of motion (ROM) and muscle strength. Furthermore, PT is necessary to maintain aerobic conditioning of the cardiovascular system. Although PT does not reduce the degenerative process within the spinal cord, individuals with HSP must maintain an exercise regimen performed at least several times each week, as guided by their physical therapist. Exercise can help the patient to retain or improve muscle strength, minimize atrophy of the muscles caused by disuse, increase endurance, reduce fatigue, prevent spasms and cramps, and maintain or improve ROM. Exercise also has a positive psychological effect, helping to reduce stress and produce feelings of well-being.

Patients with HSP may experience spasticity and weakness (ie, increased muscle tone and reduced muscle strength). Because of the increased resistance to passive stretching, spasticity may make it difficult for patients to exercise certain muscles. Antispasmodic drugs may help the patient to reduce the spasticity and may allow weakened muscles to be targeted in order to improve the effectiveness of PT.

Different types of exercised incorporated into PT programs for patients with HSP may include strengthening, stretching, and aerobic exercises, as follows:

• Strengthening exercises - These help to strengthen muscles that have not yet weakened. Strengthened muscles help to compensate for muscles that have weakened, decreasing the rate of functional impairment. Exercise may also help to slow the development of disuse atrophy, which occurs in muscles that are not being used (eg, in calf muscles of people who use wheelchairs). Back-strengthening exercises may help to reduce or eliminate back pain associated with HSP. Such pain is probably not due to HSP itself but to strain on the back resulting from HSP (eg, poor gait, poor posture, use of a mobility device).
• Stretching exercises - These help to maintain or increase ROM and to reduce such problems as tendinitis, bursitis, and muscle cramps.
• Aerobic exercises - These improve cardiovascular fitness, reduce fatigue, and increase endurance and general fitness. Walking, bicycle riding, water aerobics, and swimming are among many excellent forms of aerobic exercise.

Medical Issues/Complications

Currently, no specific treatment exists to prevent, retard, or reverse progressive disability in patients with HSP. Nonetheless, treatment approaches used for chronic paraplegia from other causes are useful.

Possible complications associated with HSP include the following:

• Gastrocnemius-soleus contracture
  • This condition is more common when symptoms begin in childhood rather than in adulthood.
  • It also occurs when PT has not been sufficient.
• Cold feet
  • Many people with HSP complain of cold feet. This is a common complaint in many disorders of the upper or lower motor neurons.
  • Cold feet may be related to abnormal thermoregulation of cutaneous vessels; however, circulation is usually preserved.
• Fatigue
  • Fatigue is a common symptom of HSP. One obvious cause is the extra effort required for walking, because of muscle weakness in the legs.
  • Various medications prescribed for HSP cause drowsiness or fatigue. Many patients with HSP may not get their required amount sleep, because of leg cramps or spasms or as a result of the frequent need to urinate during the night.
  • A less obvious cause of fatigue may be the fact that because of a more sedentary lifestyle, people with HSP often are not aerobically fit and therefore have reduced endurance. Stress or depression also can contribute to fatigue.
• Back or knee pain
  • Back and/or knee pain is common in people with HSP. The pain is not directly due to HSP itself but is instead often caused by muscle weakness and gait abnormalities resulting from HSP.
  • As certain muscles become weaker, other muscles need to compensate for that weakness. Compensatory measures create an awkward gait that causes strain on many muscles and joints.
  • Patients may thrust their shoulders back or swing their legs outward as they walk. Use of certain mobility devices also may put a strain on the arms or back.
• Stress and depression
  • Stress, depression, and denial are not unusual in patients with HSP or any other chronic illness.
  • Denial is not necessarily a problem, as long as the person in denial is not depriving himself or herself of proper treatment and care. Denial allows some people to cope and set their worries aside.
  • Some people with HSP face denial by family members who refuse to admit that a problem exists. This can create a frustrating and stressful situation.

Consultations

• Physical medicine and rehabilitation specialist
• Neurologist

Medication

The goals of pharmacotherapy are to reduce morbidity and prevent complications.⁶

Antispasmodics

Bladder spasticity has been improved with oxybutynin (Ditropan).

Oxybutynin (Ditropan)

Inhibits the action of acetylcholine on smooth muscle and has a direct antispasmodic effect on smooth muscle. This in turn causes an increase in bladder capacity and a decrease in uninhibited contractions.

Skeletal muscle relaxants

Antispasticity medications can be useful. However, one of the drawbacks of using these agents is that some patients find that the stiffness of spasticity helps them to overcome the muscle weakness that also occurs in HSP. When patients are medicated to reduce stiffness, walking may become more difficult. Adverse effects can also be a problem. If the patient does well with the medications, however, discomfort associated with spasticity can generally be reduced, mobility can be improved, and the effectiveness of PT can be enhanced. Patients in relatively early stages of the illness have achieved symptomatic improvement with oral dantrolene, as well as with oral and intrathecal baclofen.

Baclofen (Lioresal)

May induce the hyperpolarization of afferent terminals and inhibit monosynaptic and polysynaptic reflexes at the spinal level.

Tizanidine (Zanaflex)

Centrally acting muscle relaxant metabolized in the liver and excreted in urine and feces. A single oral dose of 8 mg reduces muscle tone in patients with spasticity for several hours. Blood levels and the spasmolytic effect are linearly correlated.

Dantrolene sodium (Dantrium, Dantrium IV)

Stimulates muscle relaxation by modulating skeletal muscle contractions at the site beyond the myoneural junction and acting directly on muscle itself. Most patients respond to 400 mg/d or less.

Botulinum toxin (BOTOX®)

Binds to receptor sites on motor nerve terminals and inhibits the release of acetylcholine, which in turn inhibits the transmission of impulses in neuromuscular tissue. This agent is most useful for treating spasticity in the gastrocnemius and soleus muscles; it is less effective in larger muscles (eg, quadriceps). Reexamine patients 7-14 d after the initial dose to assess for their response. May be repeated q3-4 mo.

Benzodiazepines

These agents may act in the spinal cord to induce muscle relaxation.

Diazepam (Valium)

Depresses all levels of CNS (eg, limbic and reticular formation), possibly by increasing the activity of GABA.
Individualize the dosage and increase it cautiously to avoid adverse effects.

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