Could someone tell me about Lewis Sumner Syndrome?

[Alastor M]

I tried Googling it, but came up with nothing.
I'm being tested for it (dermatomyositis has been confirmed, being treated for it soon), because of both sensory and motor loss, but no UMN signs at all (which was odd because I swore I had some...). In fact, my reflexes seem decreased.
Is it related to or does it mimic ALS?
Thanks a bunch.

 

[rose]

Its a form of CIDP, you might try sending Laurel a message with some of your questions, I believe she knows something about it. good luck

 

[laurel]

Hi Alastor. It sure seems to mimic ALS in some ways. Hubby has CIDP-MADSAM (Lewis-Sumner) and even being diagnosed with that he still has symptoms that don't totally fit the diagnosis. Hubby did have a differential diagnosis of ALS at one point. His symptoms are atrophy of rt. hand and forearm, foot drop rt. foot, diminished reflexes, fasciculations, cramps at times in that hand and both legs, fatigue, brain fog, excessive salivation. He was initially diagnosed with Carpal Tunnel and had several EMG's and nerve conduction tests with a neurologist over a period of 2-3 yrs, was referred by that neurologist to a surgeon for the supposed nerve impingements, had surgery, got worse, went to a speciality referral clinic, and then was referred to a neuromuscular disorder neurologist. There he had EMG, nerve conduction tests, MRI, blood work, specialty blood work sent to Athena Labs for sensory motor testing. He was given a trial loading dose of IVIG of 2 G. per kilo of his weight to see if he responded (he had so many motor symptoms that were like ALS and I guess some question marks with his EMG that he didn't fit anywhere completely). He had good response to the IVIG and has been on it since 2007 every 3 weeks. The IVIG has kept him from getting too much worse --he sort of maintains with a very slow decline. We are very lucky that it didn't turn out to be ALS, but it is still a insidious stinky disease. Fire away with any questions that you may have. I likely have omitted things that you may want to know.
Laurel

 

[Alastor M]

I have to ask, laurel, how fast did the atrophy progress? Was it sudden or subtle?
Also, does he experience a lack of sensation on his atrophied hand as I do?
I've definitely got some thenar atrophy in my right hand and a foot drop of some sort on the left side.

 

[laurel]

Alastor the atrophy and loss of use was slow and insidious. Hubby is not the most self aware kind of guy. He first noticed it when riding our horses and holding the reins. The right rein kept slipping out of his hand--he sort of thought the horse was snatching the reins instead of him not having good grip. Then he noticed that he was having trouble moving toggle switches (he was an airline pilot) i.e. losing his pincher grip. He went to our GP and GP referred him to a neurologist. He had an EMG and nerve conduction tests. Neuro. said no problems, but return if things get worse. Hubby lost more strength in his hand and returned maybe six months later. Same response from the neurologist after repeating the tests. Then perhaps a year later when my husband was trimming our horses feet and had his sleeve rolled up, I gave a gasp as his forearm was shrunken and atrophied, and he couldn't hold the hoof knife with any grip. Back to the neuro, repeated the tests, and now said he had Carpal Tunnel. He had the surgery (we went with the second best surgeon as the first had a one year wait). Atrophy got worse after surgery. We paid and went to a speciality referral clinic and the doctor who assessed him was the surgeon who had the long wait list. Within 10 minutes of examining him he knew it was some sort of progressive muscle atrophy and said "you never had any symptoms of Carpal Tunnel". Anyway it destroys ones faith in the professionals i.e. first neurologist and first surgeon. When he went to the neuromuscular disorder neurologist, she gave a guess of the diagnosis after the EMG and nerve conduction testing at the first appt. Her interpretation and finding on the EMG and nerve conduction tests totally differed from those of the neurologist that hubby saw over a two year period. Hubby has few sensory issues--a little tingling on occasion. He doesn't not complain of numbness or loss of sensation other than with a couple of toes with the foot drop. Anyway I rambled <g>. Slow and insidious--creeping up on him over 2-3 years. He has no thenar muscle and a very shrunken forearm. It got so bad that he couldn't turn a key, use utensils to eat, hold a coffee cup. In the midst of being diagnosed, he developed foot drop. IVIG maintains current function, but he never re-gained all his losses. He feels that he developed his autoimmune faulty response after an episode of diverticulitis and kidney stones with bladder infection.
Laurel