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.
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.
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.
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.
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.
Consultations
Depending on the type and degree of dysfunction, the following consultations may be considered:
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Physical medicine specialist
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Occupational therapist
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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
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Nutritionist
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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
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.
