Amyotrophic Lateral Sclerosis (Lou Gehrig`s Disease)
Amyotrophic Lateral Sclerosis
(Lou Gehrig's Disease)
What is ALS?
ALS is a form of motor neuron disease
caused by the degeneration of neurons
located in the ventral horn of the spinal
cord and the cortical neurons that
provide their afferent input.
The disorder is characterized by rapidly progressive
weakness, muscle atrophy and fasciculations,
spasticity, dysarthria, dysphagia, and respiratory
compromise. Sensory function generally is spared, as
is autonomic, and oculomotor activity. ALS is a
progressive, fatal, neurodegenerative disease with
most affected patients dying of respiratory
compromise and pneumonia after 2 to 3 years;
although occasional individuals have a more indolent
course and survive for many years.
The earliest symptoms of ALS are typically obvious
weakness and/or muscle atrophy. Other presenting
symptoms include muscle twitching, cramping, or
stiffness of affected muscles; muscle weakness
affecting an arm or a leg; and/or slurred and nasal
To be diagnosed with ALS, patients must have signs
and symptoms of both upper and lower motor neuron
damage that cannot be attributed to other causes.
The very high presence of the disease among Italian
soccer players, more than five times higher than
normally expected, has raised the concern of a
possible link between the disease and the use of
pesticides on the soccer fields.
For patients without a family history of the disease,
which includes 95% of cases, there is no known cause
No test can provide a definite diagnosis of ALS,
although the presence of upper and lower motor
neuron signs in a single limb is strongly suggestive.
Instead, the diagnosis of ALS is primarily based on the
symptoms and signs the physician observes in the
patient and a series of tests to rule out other diseases.