eMedicine Specialties > Neurology > Neuromuscular Diseases
Primary Lateral Sclerosis: Treatment & Medication
Updated: Jun 8, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
Mechanism-specific treatments directed at the pathological process that underlies primary lateral sclerosis (PLS) have not been identified. Consequently, treatments are directed at alleviating symptoms and may be classified as follows:
- Pharmacologic: Treatments for spasticity include baclofen (Lioresal), tizanidine (Zanaflex), and the benzodiazepines, such as diazepam (Valium) or 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: Stretching exercises, usually used in combination with pharmacologic treatment, may help alleviate spasticity. 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. 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: These may be needed to compensate for specific disabilities. Periodic evaluation for these by physical and occupational therapists may be beneficial.
- Support groups: Due to the rarity of PLS, support groups exclusive to patients with PLS are not likely to be available. Subscription to the national PLS newsletter may enable patients to identify others within a community with whom they might form an informal support group (see Patient Education ).
- Multidisciplinary clinic: Patients with PLS may benefit from evaluation and follow-up at multidisciplinary clinics such as those available for the more common amyotrophic lateral sclerosis (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.
- Other: Patients late in the course of PLS may develop ventilatory failure and may require noninvasive ventilatory support.
Surgical Care
Some patients with spasticity that is not controlled with oral medications may be candidates for intrathecal baclofen. A continuous infusion pump is implanted surgically under the skin with the tip of the infusion catheter in the thecal sac.
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 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 with HSP and patients with unexplained upper motor neuron symptoms who are referred for genetic testing.
Diet
A balanced diet based on the patient's physical activity and other needs is recommended to avoid excessive weight gain or inanition.
Activity
Activity should be maintained as tolerated to maximize existing function and to preclude accelerated dysfunction due to disuse and development of contractures.
Medication
Medications to alleviate spasticity are discussed here. Patients in whom oral medications do not provide adequate relief may wish to consider intrathecal baclofen (ie, via continuous infusion pump).
Skeletal muscle relaxants
These agents are used to treat reversible spasticity associated with MS or spinal cord lesions.
Baclofen (Lioresal)
May induce hyperpolarization of afferent terminals and inhibit both monosynaptic and polysynaptic reflexes at spinal level.
Adult
10-80 mg/d PO in divided doses; titrate dose until beneficial effects observed or adverse effects preclude further increases
Pediatric
Not established
Opiate analgesics, benzodiazepines, alcohol, tricyclic antidepressants, guanabenz, MAOIs, clindamycin, and hypertensive agents may increase effects
Documented hypersensitivity, cognitive impairment, liver dysfunction
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in autonomic dysreflexia and when spasticity is utilized to obtain increased function; autonomic dysreflexia can result from withdrawal of this medication; may result in elevation of liver enzymes, cognitive changes, fatigue, and paradoxical weakness if dose too high
Tizanidine (Zanaflex)
Centrally acting muscle relaxant metabolized in liver and excreted in urine and feces.
Adult
4-32 mg/d PO in divided doses; titrate dose until beneficial effects observed or adverse effects preclude further increases
Pediatric
Not established
Tizanidine-induced somnolence, stupor may be increased by alcohol; clearance decreased by oral contraceptives; can increase hypotensive effects when administered with diuretics
Documented hypersensitivity, cognitive impairment, liver dysfunction
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in renal impairment; may result in elevation of liver enzymes; may result in cognitive changes or fatigue; avoid driving or other activities requiring alertness until cognitive functions no longer affected
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 activity of GABA.
Individualize dosage and increase cautiously to avoid adverse effects.
Adult
10-40 mg/d PO in divided doses PO; use lowest effective dose
Pediatric
Not established
Toxicity of benzodiazepines in CNS increased by phenothiazines, barbiturates, alcohols, and MAOIs
Documented hypersensitivity, narrow-angle glaucoma
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Caution with other CNS depressants, low albumin levels, or hepatic disease (may increase toxicity); avoid driving or other activities requiring alertness until cognitive functions no longer affected; abrupt discontinuation may precipitate seizures
More on Primary Lateral Sclerosis |
| Overview: Primary Lateral Sclerosis |
| Differential Diagnoses & Workup: Primary Lateral Sclerosis |
 Treatment & Medication: Primary Lateral Sclerosis |
| Follow-up: Primary Lateral Sclerosis |
| References |
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Further Reading
Keywords
PLS, motor neuron disease, motoneuron disease, progressive spasticity, stiffness, MNDs, primary lateral sclerosis, upper motor neurons, lower motor neurons, ALS, amyotrophic lateral sclerosis, progressive muscular atrophy, PMA, spinal muscular atrophies, SMAs, degenerative diseases
