It ain't over till the fat lady sings.....
This patient is going to die, and I know how he’s going to die: respiratory failure, probably accelerated by pneumonia. I am looking at a living skeleton. No pain, just weakness.
He has next to no muscles to talk about, because he probably has Progressive Muscular Atrophy (PMA), also known as ‘Duchenne-Aran Disease’. PMA is a sub-classification of Amyotrophic lateral Sclerosis (ALS) (1), and is a form of Motor Neurone Disease (2). He says that he feels slow when he goes up stairs. His quads are weak when I test them .There is no exaggeration of his deep tendon reflexes, no Babinski response, and no muscular spasticity so he probably does not yet have ALS (3). It is calculated that a GP working full-time for 30yrs would probably only encounter one or two cases in their entire career (4), so early diagnoses are often missed (5). No pain, just weakness.
ALS normally comes on in mid to late life, and most patients only last a few years. My patient is different though, he has been like this since his youth, which makes PMA more likely. PMA is only slowly progressive , and patients look like living skeletons. Barnum’s ‘American Museum Freak Show’ had a series of ‘Living Skeletons’, including Isaac W Sprague (6). No pain, just weakness.
There was also a ‘Fat Lady’ in Barnum’s ‘American Museum’ or freak show. Apparently it became common for the ‘Fat Lady’ to be married to the ‘Living Skeleton’. Sprague was happily married to Tamar Moore, with whom he had three sons, none of whom developed PMA. I don’t know if she was one of Barnum’s ‘Fat Ladys’, or not. No pain, just weakness.
Sprague died of asphyxia, aged 45yrs. The fat lady had evidently sung….. No pain, just weakness.
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I am a chiropractor and sonographer. I also mentor colleagues to help them become the clinicians that they want to become. Find out more here.
