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A 29-year-old male asked:

what is the definition or description of: primary lateral sclerosis?

1 doctor answer1 doctor weighed in
Dr. Bennett Machanic
Neurology 52 years experience
Variation of ALS: Motor neuron disease or ALS presents in several variable forms, and is a disease of motor neuron degeneration in brain and spinal cord. In the primary lateral sclerosis variant (only 1-3% of all cases), the disorder affects the upper motor neuron only, and is focal on one side or the other. By definition, this form would be far less malignant.

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A 37-year-old member asked:

What are the symptoms of primary lateral sclerosis?

1 doctor answer1 doctor weighed in
Dr. Bennett Machanic
Neurology 52 years experience
Variant of ALS: Als affects motor nerve cells, but varies in presentation. Diagnosis of primary lateral sclerosis is challenging, as involves upper motor neuron cells and early on, an EMG test could well be non-diagnostic, as it tests the lower cells. Since this variation involves central corticospinal cells, there is unilateral weakness of arm and/or leg without sensory loss. Later, loss of muscle bulk maybe.
A 33-year-old member asked:

What is primary lateral sclerosis?

1 doctor answer3 doctors weighed in
Dr. Bennett Machanic
Neurology 52 years experience
Variation of ALS: Motor neuron disease or ALS presents in several variable forms, and is a disease of motor neuron degeneration in brain and spinal cord. In the primary lateral sclerosis variant (only 1-3% of all cases), the disorder affects the upper motor neuron only, and is focal on one side or the other. By definition, this form would be far less malignant.
A 44-year-old member asked:

I am suffering from primary lateral sclerosis, is there a treatmnet?

1 doctor answer2 doctors weighed in
Dr. Bennett Machanic
Neurology 52 years experience
Yes there is: Primary lateral sclerosis is a form of lou gehrig's disease, which affects the motor neuron cell, and may involve a slow chronic course. The drug Riluzole is the only product on the market so far, and has shown some potential to prevent tracheostomy. Part of the treatment involves supportive care, and prevention of infections, so work closely with a team of experienced physicians.
A 30-year-old member asked:

Primary lateral sclerosis only 1 side, is this normal?

1 doctor answer2 doctors weighed in
Dr. Bennett Machanic
Neurology 52 years experience
Not unexpected: Not surprising but this may not stay unilateral, or this may be the ultimate outcome. Motor neuron disorders vary in expression, but require attentive followup with a neuromuscular specialist on an ongoing basis. Riluzole may be of value.

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Last updated Jan 12, 2015

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