Progression

[bryant123martha]

I have had proximal leg weakness for the past 9+ years. After 8 years I asked the doctor and showed lower motor neuron signs like Absent / demenished deep tendon reflex, with no Upper motor signs, and my EMG showed denervation as well as my muscle biopsy showed denervation. He diagnosed me with SMA but my genetic testing came back negative but the doc said it was still SMA so I went to the international SMA conference and spoke to the researchers. They agreed I should be tested for Tay Sachs, and it can't be SMA if it is not on the SMN 1 or 2 gene. I went to the MDA clinic to see a new doctor and for the first time I am presenting with Upper Motor neuron signs lke Babinski sign and some hyperreflexia in my hands. I have recently noticed some weakness in my arms and hands. My hands are visabily showing atropy and I keep dropping things. He did another EMG and and it showed the same. He said the EMG looks like SMA, polio as a kid, but it must be PMA. It could be turning into traditional ALS.
Since my appointment 3 months ago it is progressing faster in my hands and trunk.

I know ALS is very individualized but does anyone know if it starts in the legs does it usually go to the hands and arms then the bulbar and lungs?

If it starts slower like with Lower motor neuron signs does it usually continue slower?

Has anyone had similar progression?

Thank you for all your help!

 

[lisaohgee]

My mom's started with her hand, then her feet, legs, arms, head, etc. It moved it's way upwards. She's now completely paralyzed save her a little head movement.

 

[bryant123martha]

Thank you very much Lisa. Sorry to hear about your mom. It is good to know how others progress even if I know it can be different.
Have a blessed day!

 

[lisaohgee]

Sure thing.

 

[hjlindley]

Mine started in my right hand, then hip flexors and left ankle,now left hand. Waiting for r. ankle. This devil is a disguise artist.

 

[Susan10]

Mine started in my left (nondominant) hand and has progrssed up that arm and now affects my walking tho dr says my strength is good in legs. A friend at my support group said his started in his right hand (nondominant) and is progressing similarly to my situation. Everyone's different and as much as we'd like a roadmap, I don't think there is one.

 

[bryant123martha]

Yes, a road map would be nice. It is good to know how others are similar and different.
Thank you!

 

[Malissa57]

Question: As the disease advances are extreme muscles spasms in the legs and stomach common or do those extreme spasms tend to subside as the muscle is affected. Since my brother's diagnosis in May of 2011, every twitch, spasm, movement has been stronger. He's even gained weight. Please advise