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Primary Lateral Sclerosis Treatment & Management

  • Author: Carmel Armon, MD, MSc, MHS; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
 
Updated: Dec 30, 2015
 

Approach Considerations

Mechanism-specific treatments directed at the pathologic process that underlies primary lateral sclerosis (PLS) have not been identified. Consequently, treatments are directed at alleviating symptoms.

Go to Amyotrophic Lateral Sclerosis, Amyotrophic Lateral Sclerosis in Physical Medicine and Rehabilitation, and Emergent Treatment of Amyotrophic Lateral Sclerosis for complete information on these topics.

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Treatments for Spasticity

Treatments for spasticity include baclofen (Lioresal), tizanidine (Zanaflex), and the benzodiazepines, such as diazepam (Valium) and clonazepam (Klonopin). Patients in whom oral treatment does not provide adequate relief may wish to consider intrathecal baclofen (ie, infusion of medication directly into the CSF via a surgically placed continuous infusion pump). However, patients must be selected appropriately to ensure that those who receive this treatment are likely to benefit.

Patients who experience pain due to spasticity may benefit from analgesics. Those who become depressed may require antidepressants.

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Physical Therapy, Assistive Devices, and Ventilatory Support

Stretching exercises, usually used in combination with pharmacologic treatment, may help to alleviate spasticity.[13] A program of stretching/strengthening exercises, which may be done at home, may promote full range of joint motion and reduce the risk of contractures. Patients who are weak may require passive range of motion exercises to be administered by their caregivers.[14]

Attempting to overcome severe spasticity with physical therapy alone may result in torn or strained muscles or tendons. Hence, physical therapy that causes pain should be avoided or modified. Other modalities, such as massage or pool therapy, may provide symptomatic relief.

Assistive devices may be needed to compensate for specific disabilities. Periodic evaluation for these by physical and occupational therapists may be beneficial.

Patients late in the course of primary lateral sclerosis (PLS) may develop ventilatory failure and may require noninvasive ventilatory support.

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Multidisciplinary Clinic

Patients with primary lateral sclerosis (PLS) may benefit from evaluation and follow-up at multidisciplinary clinics, such as those available for the more common ALS. These multidisciplinary clinics may provide, in a single location, physical and occupational therapy, speech and swallowing evaluation and therapy, nutritional assessment and counseling, and respiratory assessment.

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Dietary Modification and Activity Considerations

A balanced diet based on the patient's physical activity and other needs is recommended to avoid excessive weight gain or inanition.

Activity should be maintained as tolerated to maximize existing function and to preclude accelerated dysfunction due to disuse and development of contractures.

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Consultations

Depending on the type and degree of dysfunction, the following consultations may be considered:

  • Physical medicine specialist
  • Occupational therapist
  • Psychologist/psychiatrist - Many patients with primary lateral sclerosis (PLS) present with neurocognitive impairments reflecting an executive dysfunction; formal assessment may help in educating patients and families and set the stage for using ameliorative and coping strategies
  • Nutritionist
  • Genetic counselor - Appropriate genetic counseling should be offered to patients who are suspected of having HSP or who are diagnosed with HSP, and to patients with unexplained upper motor neuron symptoms who are referred for genetic testing
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Long-Term Monitoring

Frequency of outpatient follow-up in patients with primary lateral sclerosis (PLS) depends on the patient's need for symptom control. It may range from monthly initially to every 4-6 months once optimal treatment is established, provided that no new symptoms appear.

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Contributor Information and Disclosures
Author

Carmel Armon, MD, MSc, MHS Chair, Department of Neurology, Assaf Harofeh Medical Center, Tel Aviv University Sackler Faculty of Medicine, Israel

Carmel Armon, MD, MSc, MHS is a member of the following medical societies: American Academy of Neurology, Massachusetts Medical Society, American Academy of Sleep Medicine, American Stroke Association, American Association of Neuromuscular and Electrodiagnostic Medicine, American Clinical Neurophysiology Society, American College of Physicians, American Epilepsy Society, American Medical Association, American Neurological Association, Sigma Xi

Disclosure: Received research grant from: Neuronix Ltd, Yoqnea'm, Israel.

Chief Editor

Nicholas Lorenzo, MD, MHA, CPE Founding Editor-in-Chief, eMedicine Neurology; Founder and CEO/CMO, PHLT Consultants; Chief Medical Officer, MeMD Inc

Nicholas Lorenzo, MD, MHA, CPE is a member of the following medical societies: Alpha Omega Alpha, American Association for Physician Leadership, American Academy of Neurology

Disclosure: Nothing to disclose.

Acknowledgements

Paul E Barkhaus, MD Professor, Department of Neurology, Medical College of Wisconsin; Director of Neuromuscular Diseases, Milwaukee Veterans Affairs Medical Center

Paul E Barkhaus, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and American Neurological Association

Disclosure: Nothing to disclose.

Neil A Busis, MD Chief, Division of Neurology, Department of Medicine, Head, Clinical Neurophysiology Laboratory, University of Pittsburgh Medical Center-Shadyside

Neil A Busis, MD is a member of the following medical societies: American Academy of Neurology and American Association of Neuromuscular and Electrodiagnostic Medicine

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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