ALS Diagnosis changed to possible PMA

[littlered]

Hi All,

While this is my first post, I've been reading this forum for many months. My close friend was diagnosed with ALS in June 2012 with first symptoms starting May 2011. Today, she received a call from her ALS doctor who told her that they are not sure she has ALS and that her progression could indicate PMA. So far her left arm and leg have experienced the most muscle loss. The right side is just beginning. Her breathing at her appointment last week was 98%. Could this be why they are considering a change in diagnosis?

Thank you for any thoughts you may have. There is a bundle of information on ALS, but I'm finding that isn't the case on PMA.

 

[SMP51]

this is what I found. and it doesn't seem to be much different than ALS other than it is or could be much slower in progression, sort of like a sister disease or variant of the same disease AND your friend could have trouble getting the assistance she needs if not technically diagnosed with ALS! see #4!
do not let them change her diagnosis on paper!


Diagnosis[edit]

PMA is a diagnosis of exclusion, there is no specific test which can conclusively establish whether a patient has the condition. Instead, a number of other possibilities have to be ruled out, such as multifocal motor neuropathy or spinal muscular atrophy. Tests used in the diagnostic process include MRI, clinical examination, and EMG. EMG tests in patients who do have PMA usually show denervation (neurone death) in most affected body parts, and in some unaffected parts too.[3]

It typically takes longer to be diagnosed with PMA than ALS, an average of 20 months for PMA vs 15 months in ALS/MND.

Prognosis[edit]

The importance of correctly recognizing progressive muscular atrophy as opposed to ALS is important for several reasons.
1) the prognosis is a little better. A recent study found the 5-year survival rate in PMA to be 33% (vs 20% in ALS) and the 10-year survival rate to be 12% (vs 6% in ALS).[1]
2) Patients with PMA do not suffer from the cognitive change identified in certain groups of patients with MND.[4]
3) Because PMA patients do not have UMN signs, they usually do not meet the "World Federation of Neurology El Escorial Research Criteria" for “Definite” or “Probable” ALS and so are ineligible to participate in the majority of clinical research trials such as drugs trials or brain scans.[1]
4) Because of its rarity (even compared to ALS) and confusion about the condition, some insurance policies or local healthcare policies may not recognize PMA as being the life-changing illness that it is. In cases where being classified as being PMA rather than ALS is likely to restrict access to services, it may be preferable to be diagnosed as "slowly progressive ALS" or "lower motor neuron predominant" ALS.

An initial diagnosis of PMA could turn out to be slowly progressive ALS many years later, sometimes even decades after the initial diagnosis. The occurrence of upper motor neurone symptoms such as brisk reflexes, spasticity, or a Babinski sign would indicate a progression to ALS; the correct diagnosis is also occasionally made on autopsy.[5][6]

 

[littlered]

Thank you SMP51 for reponding. Very interesting on the insurance issue. I will definetly pass that along. She has registered here on the forum (ShereeB), but isn't on here very oftern.