Hereditary Spastic Paraplegia Treatment & Management

  • Author: Nam-Jong Paik, MD, PhD; more...
 
Updated: Jan 6, 2012
 

Approach Considerations

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

Regular physical therapy (PT) is important for maintaining and improving range of motion (ROM) and muscle strength, as well as for maintaining aerobic conditioning of the cardiovascular system.

Consultations in HSP can include a physical medicine and rehabilitation specialist and a neurologist.

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Physical Therapy

As stated previously, regular PT is important for maintaining and improving 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.

Types of exercises

The types of exercise incorporated into PT programs for patients with HSP may include strengthening, stretching, and aerobic exercises.

Strengthening exercises 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 help to maintain or increase ROM and to reduce such problems as tendinitis, bursitis, and muscle cramps.

Aerobic exercises 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.

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Contributor Information and Disclosures
Author

Nam-Jong Paik, MD, PhD  Chair, Department of Rehabilitation Medicine, Seoul National University Bundang Hospital; Professor, Department of Rehabilitation Medicine, Seoul National University College of Medicine

Nam-Jong Paik, MD, PhD is a member of the following medical societies: American Association of Neuromuscular and Electrodiagnostic Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Jae-Young Lim, MD, PhD  Associate Professor, Seoul National University College of Medicine, Seoul National University Bundang Hospital, South Korea

Jae-Young Lim, MD, PhD is a member of the following medical societies: American Association of Neuromuscular and Electrodiagnostic Medicine

Disclosure: Nothing to disclose.

Additional Contributors

Kat Kolaski, MD Assistant Professor, Departments of Orthopedic Surgery and Pediatrics, Wake Forest University School of Medicine

Kat Kolaski, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine and American Academy of Physical Medicine and Rehabilitation

Disclosure: Nothing to disclose.

Teresa L Massagli, MD Professor of Rehabilitation Medicine and Pediatrics, University of Washington School of Medicine

Teresa L Massagli, MD is a member of the following medical societies: American Academy of Pediatrics, American Academy of Physical Medicine and Rehabilitation, and Association of Academic Physiatrists

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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Photograph of a 16-year-old girl with complicated hereditary spastic paraplegia. She has a spastic gait disturbance, mental retardation, and extrapyramidal symptoms. Note the dysmorphic features.
Dysmorphic appearance of a 16-year-old girl with complicated hereditary spastic paraplegia. This patient displays a short stature (145 cm) and hair loss. Anterior (left), lateral (middle), and posterior (right) views are shown.
General appearance of sisters with complicated hereditary spastic paraplegia. They are aged 16 and 17 years. Physical examination revealed increased deep tendon reflexes in all 4 extremities, with an extensor plantar reflex. Sensory losses in the patients have affected mainly their joint positions and vibration sensations.
 
 
 
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