Life With ALS.com
+ Diagnosed Aug 2005
+ Bipap March 2007
+ PEG July 2007
+ Trache and Vent July 2008
Still Living, Loving & Laughing
What is Primary Lateral Sclerosis?
Primary lateral Sclerosis is a disorder of the upper
motor neurons. The degeneration of these upper
motor neurons in the brain and spinal cord, which
control voluntary movements, cause disabling
spasticity and weakness. As the muscles are not
directly affected, there is no wasting or
fasciculation’s (rippling effect under the skin), with
this condition. There are also lower motor neurons
in the body, but these are unaffected by primary
lateral sclerosis.
The red lines indicate upper motor neurons, which
are affected by primary lateral sclerosis.
Green lines are the lower motor neurons, where no
changes occur in primary lateral sclerosis.
How is PLS diagnosed?
Usually tests need to be carried out to exclude
other conditions as the likely cause of symptoms.
Other conditions may include multiple sclerosis,
brain/spinal tumours, spinal injury or a virus.
Amyotrophic lateral sclerosis, which is the commonest form of motor neuron
disease, and affects both upper and lower motor neurons, would also have to be
Excluded.
What are the tests for PLS?
• Magnetic resonance imaging (MRI) scan: used to detect any abnormality of
the brain and spinal cord.
• Nerve conduction test: uses small electrical shocks to check the speed of
nerve conduction to the muscles; in MND this would appear normal.
• Electromyogram (EMG): measures the activity of muscles to detect any
dysfunction.
• Blood tests to check on general health.
PLS Symptoms
• Lower limb weakness, a decreased sense of balance, and stiffness in the limbs.
• Clumsy hands, slurred speech.
• Emotional lability (inappropriate or uncontrolled laughing or crying)
• Speech and swallowing difficulties.
• Bladder urgency in some cases.
Who gets Primary Lateral Sclerosis?
Generally it affects people over 50 years old, whether male or female. It is not
passed on to other members in a family.
PLS Prognosis
Life span could essentially be normal, although it may be life limiting, depending on
whether the progression remains as primary lateral sclerosis, or develops into
amyotrophic lateral sclerosis.
How might I be affected by PLS?
You may have a greater tendency to fall, as balance is affected. There may also be
some pain in the neck, back and legs.
Tasks such as washing, dressing, writing, and cooking may be difficult, because of
decreased mobility.
Speech problems may occur due to the muscles in the throat and neck becoming
sluggish. This may result in impaired control of the tongue, lip and palate, causing a
loss of clear speech.
Swallowing difficulties may result in problems with eating and drinking.
There may also be loss of emotional control, with inappropriate laughing or crying.
Management of Primary Lateral Sclerosis
Management of the disease is mostly by symptom control. This includes medication
to reduce cramped and tightened muscles and physical therapy/exercise, to
maintain flexibility and range of motion. Assistive devices and communication aids
may also be appropriate.
Exercise
Exercise such as walking, or water aerobics, may help to strengthen muscles to
compensate for those that are weakening. Stretching exercises may also lessen
muscle cramps, and maintain motion. Always consult a doctor before undertaking
any exercise.
Your doctor may prescribe medication to relieve cramps and stiffness.
Speech
Speech problems may become such, that speech is impaired over time. There are
many aids that could be useful to continue communication, such as a light writer or
computer adaptations.
Feeding
You may become dependent on others for your daily intake of nutrition and fluids. If
feeding becomes difficult and tiring, due to the swallowing muscles becoming
sluggish, it may be appropriate to consider PEG feeding. This would help you to get
better nourishment, and a feeling of well being.
In all cases, assessment by a professional is advised, in order to benefit from the
appropriate services.
Added: Nov 6, 2010
