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Hereditary Spastic Paraplegia Medication

  • Author: Nam-Jong Paik, MD, PhD; Chief Editor: Stephen Kishner, MD, MHA  more...
 
Updated: Apr 15, 2016
 

Medication Summary

Many symptoms that are common in people with HSP are not caused directly by HSP but are instead caused indirectly by muscle spasticity, weakness, or hyperactive reflexes. As previously mentioned, antispastic 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.[13] However, when patients are medicated to reduce stiffness, walking may become more difficult. Moreover, adverse effects from skeletal muscle relaxants can be a problem.

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Skeletal muscle relaxants

Class Summary

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 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 physical therapy (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, Gablofen)

 

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

Tizanidine (Zanaflex)

 

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

Dantrolene sodium (Dantrium, Revonto)

 

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

OnabotulinumtoxinA (BOTOX®)

 

Botulinum toxin 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 days after the initial dose to assess for their response. Treatment may be repeated every 3-4 months.

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Anxiolytics, Benzodiazepines

Class Summary

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

Diazepam (Valium)

 

Diazepam depresses all levels of the central nervous system (eg, limbic and reticular formation), possibly by increasing the activity of gamma-aminobutyric acid (GABA). Individualize the dosage, and increase it cautiously to avoid adverse effects.

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Contributor Information and Disclosures
Author

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

Chief Editor

Stephen Kishner, MD, MHA Professor of Clinical Medicine, Physical Medicine and Rehabilitation Residency Program Director, Louisiana State University School of Medicine in New Orleans

Stephen Kishner, MD, MHA is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine

Disclosure: Nothing to disclose.

Acknowledgements

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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