Neuromuscular diseases are rare acquired or inherited
(genetic) conditions that affect some part of the neuromuscular
system:
-
the muscles
-
the peripheral motor nerves (in arms,
legs, neck and face)
-
the neuromuscular junction where the nerves
and muscles meet
-
the muscle-controlling nerve cells (motor
neurons) in the spinal cord
More than a million people in the United States are affected
by some form of neuromuscular disease, and about 40 percent of
them are under age 18.
All neuromuscular diseases are progressive in nature, and all
result in muscle weakness and fatigue. Some diseases are present
at birth, some manifest in childhood, and others have an adult
onset. The disease may be passed down through family genetic lines,
and in some cases the student may have an affected sibling, parent
or other relative. At other times there isn’t a family history
and the disease is the result of a spontaneous genetic mutation,
an abnormal immune response or an unknown cause.
Life expectancy varies by disease and severity, from very short
to normal length. Heart and respiratory problems, which are secondary
effects of muscle deterioration, often are the cause of death.
Although muscle wasting isn’t painful, the resultant weakness
can cause cramping, stiffness, joint deformities, chronic aches
and pain, and sometimes the tightening and freezing of joints
(contractures).
In almost everyone with a neuromuscular disease, bladder and bowel
control are normal, although students may need help in the bathroom
as their physical abilities weaken. Sexual functioning and skin
sensation usually are normal.
Strengthening exercises don’t prevent muscle decline in
neuromuscular diseases, and in some cases can further damage fragile
muscle cells if done too vigorously. Moderate and light exercise
or standing exercises, undertaken under the guidance of a physical
therapist (PT) or doctor, may help maintain muscle tone and flexibility,
as well as combat obesity and bone thinning. Be sure a doctor
or PT has approved your student’s exercise plan.
For information about a specific neuromuscular disease, see “Neuromuscular
Disease Descriptions.”
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