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cam54

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Joined
Sep 10, 2015
Messages
7
Reason
Loved one DX
Diagnosis
09/2015
Country
US
State
CA
City
Ojai
My MIL has recently been diagnosed with ALS, symptoms include slurring and difficulty speaking, tongue numbness and fasciculations and twitches in upper arms and one or two fingers, symptoms began in February of this year and have gotten progressively worse. I just wondered if anyone had any idea why PLS has never been an option? She has no lower limb involvement currently and has had MRIs, blood tests and an EMG for testing. I know im just hoping for ANY diagnosis other than PBP but I wondered if it was possible that PLS still be on the table.
 
This is from an older post of mine, but is relevant to your question...

The ALS, PLS, PMA Name Debate

Gowers, a founder of British neurology, stated in 1899 that he had never encountered a single case of PMA in which the pyramidal tracts (UMN) were unaffected. He did not think the introduction of the term “ALS” by Charcot (founder of Neurology) to be very helpful because it implied that the primary lesion was degeneration of the pyramidal tracts and that atrophy of anterior horn cells was secondary. He felt that Charcot's distinction in effect gave a new name to an old disease. He concluded that PMA, PBP, and ALS were "essentially one disease."

Brain in 1933 introduced the term "motor neuron disease" so that apparently different conditions could be brought together in a single general category and used the terms “motor neuron disease” and “ALS” interchangeably.

A series of studies have supported the opinion that PMA, PLS, and PBP were subsets of ALS.

Swank and Putnam in 1943 analyzed 197 patients. They considered PLS and PMA to be ALS that had yet to develop fully.

Lawyer and Netsky in 1953 did an analysis of 53 patients with ALS and concluded that motor neuron diseases form a group that includes PMA, PLS, ALS, and PBP.

Mackay in 1963 reviewed 126 patients with ALS and concluded that regardless of the onset, the spastic forms nearly always become atrophic, the atrophic also spastic, while the spinal forms nearly always become bulbar, and the bulbar forms, if the patients live long enough, become also spinal. The entire group is, therefore, best regarded as a single degenerative disease, ALS, which constitutes a spectrum of atrophic process at one end, spastic at the other, and both in the center. With time, cases at each end of the spectrum move towards the center.

This is where most neurologists appear to be today—with PLS (UMN signs only) on one end of the spectrum, PMA (LMN signs only) on the opposite end of the spectrum and ALS (UMN and LMN signs) in the middle, to which both ends are drawn.

Problems
1) MND, since Brain introduced the term has been used interchangeably with ALS which makes the term confusing at best.
2) The billing codes do not agree with current neurologic thinking. Instead
Amyotrophic lateral sclerosis is 335.20
Progressive muscular atrophy is 335.21
Primary lateral sclerosis is 335.24
And Motor Neuron disease is coded 335.2, which is different from ALS’s code, though the man who coined the term didn’t differentiate between the two.

The Real Problem
The real problem is that, while all the above diseases should be called ALS, the bureaucrats don’t understand that. ALS carries major benefits, while sometimes the terms, PLS, PMA and even MND may not. BUT THEY SHOULD. It is up to you to make sure your billing is coded correctly. Talk to your neuro about the coding problem and if he won’t agree with most other neuros that PLS, PMA and ALS are all the same thing (ALS) then keep fighting with him or find another neuro.
 
Sorry to hear about this but it does sound like ALS.

PLS is very rare. About 6400 get ALS every year in the US. About 64 people get PLS a year. PLS is a slow progressing disease. It sounds like the first symptoms were bulbar related (speech). Bulbar PLS stars with speech and slowly descends down the body. Arm and leg involvement will take years. Bulbar ALS progresses quickly. I have Bulbar PLS. They were able to give me a PLS diagnosis after 2 years since I had no arm/leg/lung involvement. My arms/legs started to be affected after 5 years.
 
Cam, I have PLS. I've never had the muscle fasciculations and my EMG came out relatively clean for LMN involvement. Considering my limited knowledge on the subject, these two signs may have swayed their diagnosed toward ALS. At this point, anything is possible so don't rule anything out. Prayers your way. TB
 
Hi Cam-
Your m-i-l's Upper Body is involved. That's not quite the same as Upper Motor Neuron involvement. Upper and Lower motor neurons describe where these motor neurons originate and do not have anything to do with upper body (arms, head) or lower body (legs, feet).
PLS, which can start in any part of the body, is a pure upper motor neuron disease. It is usually slow moving and is primarily spasticity and stiffness as its initial symptoms.
ALS combines Upper Motor Neuron and Lower Motor Neuron issues and can be much faster in its spread.

Your mother in law's doctor has detected Lower Motor issues combined with Upper Motor issues to arrive at his diagnosis.
 
Thanks for the responses, I figured it was a long shot but thought id better check in case there was any hope.
 
Cam I think you are confusing upper motor neurones (between brain and spinal cord) with upper extremities and lower motor neurones (between spinal cord and muscles) with lower extremities ...
 
Cam,

PLS usually (not always, but usually) starts in the legs, and moves up. There are differences in clinical symptoms as well. Comparatively:

ALS: Hyporeflexia; flaccidity; muscle wasting; fasciculations
PLS: Hyperreflexia; spasticity; no muscle wasting; usually no fasciculations

I have PLS, and all of the above are true in my case. Started in bilateral lower legs, has now moved into lower back, neck, and arms.

Mike
 
Mike that is only very generalised though.

My Chris who had ALS experienced a lot of spasticity until a muscle was completely paralysed.

I've found there is a lot of difference between how ALS presents and progresses depending on the degree of upper or lower motor neurone involvement.
 
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