IVIG Response?

[Bmgilst]

Hello all,

My mother was recently diagnosed with ALS at a medical school hospital after having an initial diagnosis of MMN from her local neurologist. There was some evidence of conduction block in her first EMG, but the opinion from the doctor at the hospital said it didn't seem significant to her.

The weakness started in September when she noticed she could not touch her pinky and the tip of her ring finger, or touch her pinky at all. She then started having a foot drop on the same side. She had her first round of IVIG ( 5 days at .4g / kg per day ) and we thought we saw improvement. She was walking around some without her cane that she had been on for the last month and she regained the ability of touch her pinky with her thumb within 2-3 weeks. However, those effects wore off and she was scheduled for another consult with her neuro and IVIG, but were not able to get it on time because she had a blood clot in her foot and was hospitalized. This of course made walking difficult and she was switched to a walker. She got in to see her neuro and had another dose of IVIG. Things like getting up from a chair seemed to get easier for her from what we could see, but then she had a fall that really scared her and she went from walking well with a walker to wanting to stay in the bed / wheelchair. She has been pretty much bedridden since Dec. 1st and all four limbs now seem to be effected whereas before it was just her right side. We got her into a physical therapy place Dec. 17th, but the therapy has been of very poor quality and wasn't what we promised although the skilled nursing has been great.

However, on her last dose of IVIG ( Feb 3rd, ) she seemed to have a response where she could lift her right arm much better a few days before then she could after the infusion. We also noticed this in January. She was on the minimum dosage of IVIG, .4mg/kg. The neurologist commented on what seemed to be an improvement in January. The doctor who diagnosed her with ALS had not ever seen her before.

She doesn't seem to have any UMN signs - no babinksi, hoffman, increased reflexes or anything like that. The 2nd EMG came back looking like ALS, but the doctor there said there were two things that were inconsistent but I don't know what they were. We have an appt. with her local neurologist Tuesday and are hoping to find out more.

Just wanted to get some input on this, as I was under the impression that ALS would not show any affect from IVIG at all. I've pretty much accepted this is ALS, but I just can't understand this one part.

 

[lgelb]

MMN comes in flavors with only "probable" conduction block, so a minimal block doesn't rule it out. Response to IVIg is a better diagnostic clue in that case, and it sounds like she had it, so I can't think why it would be discontinued. Were an MRI and a blood draw for anti-GM1 antibodies done?

 

[Bmgilst]

She had an MRI of the spine but not of the brachial plexus. Originally they thought it may be cervical spondylosis but when the fascics in the lower body it ruled that out. She also has cervicogenic headaches. Antibodies didn't show anything but I've heard those can be variable I guess.

 

[lgelb]

Well, as you can see from the criteria I linked, she should have a T2-weighted MRI of the brachial plexus and poss a spinal tap. So I'd put those on the discussion list for your appt. Tuesday. The burden of proof would be on the neuro to explain why s/he would not employ established diagnostic criteria for the workup.

 

[Bmgilst]

Thanks. We will discuss that with him Tuesday. I also know that she was on the minimum .4 G / KG and I know a lot of people with MMN routinely get up to 5x that amount. I may ask if there is any way we can do another loading dose for her with a follow up a few days after the regimen so he can document whatever he sees. Can't hurt to ask I guess.

 

[laurel]

When my husband attended the neuromuscular disorder clinic in Vancouver, BC, he was given a loading dose of 2 Grams per kilogram of weight. He was being diagnosed and they were ruling out MMN and ALS. He had a positive response to the IVIG in that he re-gained some strength in his atrophied right hand, and some strength in right leg that had foot drop. He was diagnosed with CIDP MADSAM variant which is a motor variant. Since 2007 he has been treated with IVIG.

Due to the high cost of the this drug (approx. $10,000 every 3 weeks for my husband paid by the gov't), he has had doctors decrease the dose to save money with a rapid lose of muscle strength occurring. Also at one point they began to prescribe according to ideal body weight instead of actual body weight, and he had a rapid decline in strength. He has been stable with 1 gram per kilogram of body weight for several years. He was a diagnostic puzzle, but his positive response led them to his current diagnosis.

Considering what you have written about your mother, pushing for a good hefty loading dose seems indicated. I found I had to be VERY ASSERTIVE in my husband's situation as the cost of the IVIG seems to affect their positions on trying it on patients. We were lucky to find a neurologist who was very supportive in battling the system who set very stringent criteria in prescribing i.e. dosage, calculation of dosage, which conditions are deemed appropriate as a trial etc. We lived a few stressful years of battling the gov't over IVIG. Knock on wood--we are into a routine here with IVIG. Best of luck with your mother. Keep us posted on what happens.

 

[Bmgilst]

We had an appointment with her local neurologist today, which I thought was very good. Unlike the doctor at the university hospital ( who spent maybe 10-15 min ) , her local neurologist spent nearly two hours with us talking about our options and examining her. While he did agree some of the signs are in favor of ALS, he also is confident his earlier EMG showed some conduction block on her median nerve. Her tongue muscle is somewhat effected, but that apparently is possible even though not common with MMN.

He felt that she has no upper motor neuron signs, which would point away from an ALS diagnosis. He also is intrigued by visible effect of IVIG. He is perscribing her a 2.0g / kg dose over the next several days ( in comparison to her bi-weekly .4g dose ) and is going to revisit everything a few days after that round of treatment is over.

 

[lgelb]

Glad to hear she is getting a higher dose and hopefully the response will point away from MND.

 

[Bmgilst]

Did your husband have any sensation issues? My mom has "shocking" sensations in her leg and has had a headache before the motor symptoms started. It's not a normal tension headache though - she said it's sensitive to the touch and feels like lighting shocks. It sounds a lot like what's called trigeminal neuralgia. It subsided when she noticed she was getting weaker to where she didn't feel it, but the IVIG brings it back every time. She had these even before the first round of IVIG; it almost seems like the IVIG helps repair the nerve and it is able to pick up the pain again.

One thing that struck me about her weakness is that when she developed it, it didn't just start out purely distally. She had issues with hip flexion and foot flexion at the same time. Her fasciculations also hit her entire body at the same time. I've heard that CIDP has a higher prevelence in type 2 diabetics, and she became insulin dependent about the same time she started having symptoms. Looking back, she had some falls about the same time the headaches started as well. She also has issues with regulating body heat, tremors, and odd sensations from time to time, even though she's not completely numb.

My question is, how can a doctor say something is ALS without upper motor signs? From my understanding that's required for possible, probable, and definite ALS diagnoses. If anything, she has hypo or areflexia and has no spasticity or increased tone. I guess it could still be PLS. She's definitely showed response to the IVIG in the past and actually did quite well after her original loading dose in October until a blood clot set her back.