Primary Lateral Sclerosis
I have recently had a bit to do with a disease known as Primary Lateral Sclerosis (PLS). It has been a very re-warding and educational experience. The below is a summary of the rare disease and what physiotherapy input can do to help those suffering from PLS.
What is PLS?
Primary Lateral Schlerosis is a rare degenerative disease of the upper motor neurons. It is a hard disease to diagnose as several other disease can easily present with similar presentations with one of these being Amytrophic Lateral Sclerosis (ALS). There is no diagnostic tests to confirm PLS, it is more a process of eliminating other neurological conditions first.
PLS affects the upper motor neurons of the legs, arms and the the bulbar muscles of the face. Often the legs are affected first. Symptoms include;
- Balance disturbances,
- Stiffness and rigidity of the limbs,
- Weakness (as a secondary symptom)
- Spasticity in the legs and arms.
As the bulbar muscles are affected, speech and swallowing is also impaired. The first symptom to appear is generally progressive lower limb spasticity without a loss of strength (Rollins, Oskarsson & Ringle, 2010).
PLS is not believed to affect cognitive or mental functioning yet one trial showed mild deficits in frontal lobe function and memory in 8/9 patients studied (Rollins, Oskarsson & Pringle, 2010).
The disease is a slow progressing one yet it is not fatal. Disease progression takes an average of 15-20 years and is quite slow. Average age of onset is approximately 50 years (Pringle et al, 1992) and is more common in men than women.
Treatment
Medications
- Muscle relaxants such as Baclofen, Tizanidine or Benzodiazepines are often administered to try reduce spasticity.
- Quinine and Phenytoin can help to alleviate cramp symptoms.
Physiotherapy
There is limited evidence out ther on the effects of exercise for those suffering from PLS as well as other motor neurone diseases. An updated systematic review published in 2012 looked into what evidence is out there for the effects of exercise in the Amytrophic Lateral Sclerosis or motor neurone disease. They found there was minimal evidenceinvestigating the effects of exercise on this population. The review found two trials which met their criteria yet each were quite small in size. From these two trials the following was shown
1. A moderate intesnity and load exercise program three times a week versus regular care (stretching etc) improved function on the Amyotrophic Lateral Sclerosis functional rating scale (ALSFRS) at three months (Dal Bello-Haas & Florence, 2012).
2. There were no reported adverse affects to exercise such as increased spasticity and cramps in the exercise group of the above study (Dal Bello-Haas & Florence, 2012).
- Spasticity: Spasticity is usually treated with a combination of pharmacological treatment and stretching exercises. Sometimes splinting is required. Remember to be careful with giving out stretches in a HEP as spasticity and cramps can often come on when the muscle on stretch. Be sure that if providing stretches the effects are monitored closely and that they are not done at a high speed (Which will can bring on spasticity). Massage can also be used to help alleviate tight muscles.
- Strength work: There are many options for maintaining and improving strength in those with PLS. Hydrotherapy is a great option as the heat of the pool provides great therapeutic effect for the muscles. For some, a home based exercise program is more suitable. Whatever shape or form, it is important to target the stabilising muscles of the trunk and pelvis as well as the muscles of the lower (and upper) limbs as balance can often be affected due to rigidity and spasticity.
Falls risk is high in this small population, thus monitoring balance deficits is important. Balance type exercises can also be given specific to the individuals needs.
- Multidisciplinary Involvement: Getting a person with PLS involved with a multidisciplinary team is crucial for quality care. Often there are clinics where the client can access physiotherapy, speech therapy, medical practitioners, occupational therapists and dieticians.
References
Pringle et al. (1992). PRIMARY LATERAL SCLEROSIS: CLINICAL FEATURES, NEUROPATHOLOGY AND DIAGNOSTIC CRITERIA
Rollins, Oskarsson & Ringle. International Neurology: A clinical approach. Blackwell Publishing. 2010.
Primary Lateral Sclerosis and Physical Therapy
NINDS Primary Lateral Sclerosis Information Page
Primary Lateral Sclerosis Treatment & Management
Dal bello-Haas, V. & Florence, J. (2012). Therapeutic exercise for people with amyotrophic lateral sclerosis or motor neuron disease. Cochrane database of systematic reviews. DOI: 10.1002/14651858
