Questions posted in the Neurology and Neurosurgery Forum have been answered by doctors from The Cleveland Clinic Foundation.

Subject: Re: ALS vs. PLS
Forum: The Neurology and Neurosurgery Forum
Topic Area: AMYOTROPHIC LATERAL SCLEROSIS - ALS
Posted by CCF Neurology MD:NT on August 02, 1998 at 13:39:28:
In Reply to: ALS vs. PLS posted by GF on July 31, 1998 at 16:35:18:



I have had muscling twitching for 2+ years now and have been told that if I have not developed other symptoms, such as weakness or atrophy by now, that I probably do not have ALS. Does this sound accurate? If so, could I possibly have PLS? what are the other symptoms for PLS? Should I be more concerned???


Dear GF:

As you well know, making a diagnosis of ALS (or excluding it) is a very serious matter. If an individual experiences frequent and widespread muscular twitching (which to an examining physician appears to be fasciculation), one of the more common things in the differential diagnosis is ALS. Other disorders also appear on the list, such as the benign fasciculation syndrome, multiple radiculopathies, Kennedy's syndrome, etc. The diagnosis of ALS is supported by the presence of progressive weakness without sensory signs, atrophy of muscles, and physical signs of what is called upper motor neuron involvement. The diagnosis is confirmed by an EMG test which demonstrates a typical set of abnormalities, and by other tests (such as MRI, lumbar puncture, blood tests) that serve to exclude conditions that can mimic ALS.

The fact that you have not had any symptoms other than twitching for over two years makes it less likely that you have ALS, but does not exclude it with certainty.

Primary lateral sclerosis (or PLS) is a rare subtype of ALS in which the disease affects only the upper motor neuron. Since the lower motor neuron is spared, disease manifestations that arise from the lower motor neuron (such as atrophy, fasciculation) are not observed in PLS.

You could get a clear answer to your question by seeing a neuromuscular specialist with a special interest in ALS. In case you live near Cleveland, I can strongly recommend Dr. Hiroshi Mitsumoto, Dr. Kerry Levin, Dr. Robert Shields, and Dr. Erik Pioro, who are experts on the subject. An appointment can be made by calling (800)223-2273, or (216)444-5559 locally.

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