. Requirements for ALS Diagnosis

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BLPhill

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Has anyone ever heard from a neurologist that in order to be DX with als that you have to have three of four areas of denervetion. 1. bulbar region 2. arms and hands. 3. torso. 4. legs.
 

ZenArcher

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REQUIREMENTS FOR THE DIAGNOSIS OF ALS
The diagnosis of Amyotrophic Lateral Sclerosis [ALS] requires:
A - the presence of:

(A:1) evidence of lower motor neuron (LMN) degeneration
by clinical, electrophysiological or neuropathologic examination,

(A:2) evidence of upper motor neuron (UMN) degeneration
by clinical examination, and

(A:3) progressive spread of symptoms or signs within a region or to other regions,
as determined by history or examination,


together with
B - the absence of:

(B:1) electrophysiological and pathological evidence of other disease
processes that might explain the signs of LMN and/or UMN degeneration, and

(B:2) neuroimaging evidence of other disease processes that might explain the
observed clinical and electrophysiological signs.


CLINICAL STUDIES IN THE DIAGNOSIS OF ALS

A careful history, physical and neurological examination must search for clinical evidence of UMN and LMN signs in four regions [brainstem, cervical, thoracic, or lumbosacral spinal cord] (see Table 1) of the central nervous system [CNS]. Ancillary tests should be reasonably applied, as clinically indicated, to exclude other disease processes. These should include electrodiagnostic, neurophysiological, neuroimaging and clinical laboratory studies.

Clinical evidence of LMN and UMN degeneration is required for the diagnosis of ALS.

The clinical diagnosis of ALS, without pathological confirmation, may be categorized into various levels of certainty by clinical assessment alone depending on the presence of UMN and LMN signs together in the same topographical anatomic region in either the brainstem [bulbar cranial motor neurons], cervical, thoracic, or lumbosacral spinal cord [anterior horn motor neurons]. The terms Clinical Definite ALS and Clinically Probable ALS are used to describe these categories of clinical diagnostic certainty on clinical criteria alone:

Clinically Definite ALS

is defined on clinical evidence alone by the presence of UMN, as well as LMN signs, in three regions.



Clinically Probable ALS

is defined on clinical evidence alone by UMN and LMN signs in at least two regions with some UMN signs necessarily rostral to (above) the LMN signs.

The terms Clinically Probable ALS - Laboratory-supported and Clinically Possible ALS are used to describe these categories of clinical certainty on clinical and criteria or only clinical criteria:



Clinically Probable - Laboratory-upported ALS

is defined when clinical signs of UMN and LMN dysfunction are in only one region, or when UMN signs alone are present in one region, and LMN signs defined by EMG criteria are present in at least two limbs, with proper application of neuroimaging and clinical laboratory protocols to exclude other causes.



Clinically Possible ALS

is defined when clinical signs of UMN and LMN dysfunction are found together in only one region or UMN signs are found alone in two or more regions; or LMN signs are found rostral to UMN signs and the diagnosis of Clinically Probable - Laboratory-supported ALS cannot be proven by evidence on clinical grounds in conjunction with electrodiagnostic, neurophysiologic, neuroimaging or clinical laboratory studies. Other diagnoses must have been excluded to accept a diagnosis of Clinically possible ALS.



Clinically Suspected ALS

it is a pure LMN syndrome, wherein the diagnosis of ALS could not be regarded as sufficiently certain to include the patient in a research study. Hence, this category is deleted from the revised El Escorial Criteria for the Diagnosis of ALS.
 

rose

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Hi Betty,

Yes, this is true. There is a standard that doctors are required to meet, for a definite diagnosis of ALS. I have some of it quoted below. This is for a "Definite" diagnosis. There are other levels of diagnosis, from suspected, (which is no longer part of the official terminology any longer) to possible, then, probable, (and of course definite)

The clinical diagnosis of ALS, without pathological confirmation, may be categorized into various levels of certainty by clinical assessment alone depending on the presence of UMN and LMN signs together in the same topographical anatomic region in either the brainstem [bulbar cranial motor neurons], cervical, thoracic, or lumbosacral spinal cord [anterior horn motor neurons].

The terms Clinical Definite ALS and Clinically Probable ALS are used to describe these categories of clinical diagnostic certainty on clinical criteria alone:

Clinically Definite ALS

is defined on clinical evidence alone by the presence of UMN, as well as LMN signs, in three regions.


The EMG signs of LMN dysfunction required to support a diagnosis of ALS, should be found in at least two of the four CNS regions: brainstem [bulbar/cranial motor neurons], cervical, thoracic, or lumbosacral spinal cord (anterior horn motor neurons).

For the brainstem region it is sufficient to demonstrate EMG changes in one muscle (e.g. tongue, facial muscles, jaw muscles).

For the thoracic spinal cord region it is sufficient to demonstrate EMG changes either in the paraspinal muscles at or below the T6 level or in the abdominal muscles.

For the cervical and lumbosacral spinal cord regions at least two muscles innervated by different roots and peripheral nerves must show EMG changes.


Electrophysiological features compatible with UMN involvement include:

1. Up to 30% increase in central motor conduction time determined by cortical magnetic stimulation
2. Low firing rates of motor unit potentials on maximal effort.




Clinically Probable ALS

is defined on clinical evidence alone by UMN and LMN signs in at least two regions with some UMN signs necessarily rostral to (above) the LMN signs.

The terms Clinically Probable ALS - Laboratory-supported and Clinically Possible ALS are used to describe these categories of clinical certainty on clinical and criteria or only clinical criteria:



Clinically Probable - Laboratory-upported ALS

is defined when clinical signs of UMN and LMN dysfunction are in only one region, or when UMN signs alone are present in one region, and LMN signs defined by EMG criteria are present in at least two limbs, with proper application of neuroimaging and clinical laboratory protocols to exclude other causes.



Clinically Possible ALS

is defined when clinical signs of UMN and LMN dysfunction are found together in only one region or UMN signs are found alone in two or more regions; or LMN signs are found rostral to UMN signs and the diagnosis of Clinically Probable - Laboratory-supported ALS cannot be proven by evidence on clinical grounds in conjunction with electrodiagnostic, neurophysiologic, neuroimaging or clinical laboratory studies. Other diagnoses must have been excluded to accept a diagnosis of Clinically possible ALS.



Clinically Suspected ALS

it is a pure LMN syndrome, wherein the diagnosis of ALS could not be regarded as sufficiently certain to include the patient in a research study. Hence, this category is deleted from the revised El Escorial Criteria for the Diagnosis of ALS.

Importantly:


Electrophysiological features suggesting other disease processes include:

1. Evidence of motor conduction block.
2. Motor conduction velocities lower than 70%, and distal motor latencies over 30%, of the lower and upper limit of normal values respectively.
3. Sensory nerve conduction studies that are abnormal. Entrapment syndromes, peripheral neuropathies and advanced age may render sensory nerve action potentials difficult to elicit in the lower extremities.
4. F-wave or H-wave latencies more than 30% above established normal values.
5. Decrements greater than 20% on repetitive stimulation.
6. Somatosensory evoked response latency greater than 20% above established normal values.
7. Full interference pattern in a clinically weak muscle.
8. Significant abnormalities in autonomic function or electronystagmography.
 

Zaphoon

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Wow! Two very, very good explanations on the diagnostic criteria for ALS. I vote a sticky for this thread!
 

k7ugq

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hello,

I have Myastenia Gravis for 4 years now. I recently started to lose muscle control beginning on right arm then moving to my left with a slight improvement in the right. Just my arms and hands, no other parts of my body appear to be effected. However, some twitching and atrophy with the arms and hands.

I had an EMG last month and the neurologist said I had ALS with 3 to 5 years, come back in 3 months and we'll check the progress. My own neurologist is bewildered regarding the likelihood of having 2 major neuro diseases concurrently. Although I have verified myastenia (blood test and tensilon test positive), I have never responded well to the various myastenia treatments.

When I asked the attending EMG neurologist for documentation attesting to me having ALS (for a VA claim) he never used the words or initials specially saying I had ALS. This caused concern with the VA thinking that my diagnosed of ALS may not be conclusive.

As a novice on this issue, I come to the group asking if:

What is the likelihood of have 2 major neurological disease concurrently?
Are there other tests available to differentiate between something that might be mimicking ALS?

In advance, thank you all
 

Marjorie R. Wilcox

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I am sorry you are going through all this. I know with the MG you might eventually be recommended to have your thymus gland removed. I have a friend that had that done many years ago and is still doing fine, but on steroids. He is driving again and doing GREAT! He is in his 80's now.

Yes, there are tests besides the EMGs. Spinal tap, blood tests (CPK) and muscle biopsy,
reflex testing and other neurological testings. I believe it still takes some trial and error before a conclusive diagnosed is given. I do think that by the time you get an ALS diagnosed, they are 90% sure. It's not a good one to be just guessing on.

I would say it is likely that you just have one disease and not both. It was likely thought at first you had the one, and then after testing and concluding, it has been decided you have the other. Some symptoms slide over into each category.

We wish you well as you wait and see. Marjorie and Rick
 

coolhandluke

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Hi my name is michael and im pretty scared, thank you to Al(you are the man) and rose, and all of you, you are so strong. My father has ALS for two years now and im worrying that I also have symptoms... I have twithcing in my legs sometimes, feet, and also an increase in saliva. My left calf muscle is half inch smaller than my right, and ive been measuring both of them for three months, they went from being 14 1/2 inches wide and now are just over 13 1/2 inches wide... when i look at my calve in the mirror it is clearly apparent that my left is significantly smaller than my right,,,also my left ribcage sticks out more than the right side of my rib cage... also my shoulder bone on my left side,(you know its kind of like a pointy ball that sticks up shows predominantly more than the right side, I just feel like I have been more tired lately, and I have flem that i can bring up but have no cold or sore throat... Ive had a history of head and back trauma from sports... do these symptoms sound like any of yours before you were diagnosed, please be as blunt as possible, I think all of you are so courageous, God bless you all, look forward to hearing back...
 

coolhandluke

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also for the babinski s test(my mom is a PA) my big toe goes down kind of extends, but my other toes definitely move they kinda go towards my big toe. also when i stand and look down at my feet, my left foot my toes are splayed, they are spaced apart a little bit and looks diff than my right. thank u all so much, hope to hear back from you.. - michael
 

coolhandluke

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can someone please respond to my post :-(
 

joelc

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I understand that you are anxious for responses to your posts but what you have described is not ALS. The first thing you would notice is weakness not visible signs or twitching, they don't mean a thing. Stop looking at yourself and measuring your limbs.
 

BethU

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k7ugg ... if the physician who diagnosed you with ALS is not willing to put it in writing, and if your own neuro is baffled by this other guy's conclusion, I would be leery of it, too.

I don't know what the odds are of having two major neuro diseases at the same time, but your MG tests are conclusive. It is a serious and tricky disease, as I understand it, and not all the treatments work equally well for everyone. But if you've got those antibodies, you've got MG.

If I were you, I'd ask the office of the ALS-diagnosing doctor for a copy of his written report on his exam and diagnosis ... and hold fire until you get something firmer from him than "wait and see in three months." There are specific criteria that must be met for a diagnosed of ALS, which are explained in other posts on this thread. If you meet them, he should have no problem with putting his diagnosed in writing.

Good luck. Please let us know what happens.
 

coolhandluke

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Thank you so much Joel for your response you really helped put my mind at ease, I just said a prayer for you, your response really
really helped me, God Bless, thank you so much- Michael
 

Twinsmom

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Hi Michael,
I'm sorry you're going through the stress of living with a family member with ALS, and now wondering for yourself. I would encourage you to go to a neurologist and begin the diagnostic process to figure out WHAT is going on. My husband has been going through the diagnostic journey now for 7 months, and we have been given a "probably ALS" diagnosis, that now the doctors are backpedaling on...I hope they are right.

Don't stay in your head and self diagnose...you'll make your self even more stressed.
Peace,
Melody
 

Twinsmom

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PS-I meant, I hope they are right and that it's NOT ALS.

Peace,
Melody
 
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