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Bmgilst

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Are there any cases of ALS that you guys know of that have been fatal within seven months of onset of symptoms?

My mother was diagnosed with ALS formally on Monday, after initially having a diagnosis of multifocal motor neuropathy in early October. Her serious symptoms started in late August and included twitching and right sided predominant weakness. Her symptoms started not long after starting insulin injections and occurred with preceeding ( and still happening ) headaches and hearing loss.

We thought it was an autoimmune disease because she did have some response to IVIG. After her initial treatment in mid october ( 5 days of IVIG at .4g/kg per day ) she regained thumb opposition and the ability to touch her thumb and pinky on her right hand for the first time in two months. She then had a blood clot which caused her leg mobility to decline and she was put on a walker.

Here is kind of a timeline of her progression:

7/30/15 - Walked up three flights of stairs to help daughter move.
~8/15/15 noted some weakness in hand, not able to touch pinky/thumb on right hand with fasciculations starting and quickly becoming bodywide.

9/15/15 - stopped driving due to right foot weakness and started using cane.
9/28/15 - EMG
10/4/15 - MMN Diagnosis
10/17/15 - IVIG Started
11/4/15 - Blood Clot and started on walker
~11/8/15 - regained thumb opposition
11/18/15 - difficulty rising from chair
11/23/15 - IVIG infusion; rising from chair easier
11/28/15 - Fall while using cane only, ability to walk hindered.
12/7/15 - Fall and knee hurt. Started bedrest. Has not walked since without physical assist.

12/20/15 not able to easily raise right arm to shoulder levle.
12/23/15 IVIG infusion.
12/28/15 Took 38 steps with walker in PT session.
1/2/16 Right arm able to be raised above head easily
1/31/16 not able to raise arm above head.
2/1/16 - Speech difficulty ( weak voice, talking slowly )
2/3/16 - IVIG (one day)
2/7/16 - able to raise arm again.
2/9/16 - Another EMG, no upper motor neuron signs at time, second opinion of ALS.
2/16/16 - local neurologist gives order for loading dose of IVIG again due to previous response. No upper motor signs
2/18/16 - Goes on oxygen.
2/24/16 - Voice seems better, breathing without oxygen while being transferred. Able to solid food ( cube steak with little difficulty )
2/29/16 - Neurologist appointment, voice seems weaker, fatigued, reflexes seem increased compared to last time
3/2-3/4 - Daily decrease in breathing ability, can not drink water without difficulty, can't take pills without applesauce. Air hunger.
3/5 ( Today ) - Hospice nurse comes out. She is unresponsive and is expected to pass within next 24-48 hours.


We do have one other case of ALS in the family. My mom's aunt ( one of nine children ) passed from ALS in 2008 at the age of 78. My mom's generation on that side of the family consists of her and 29 first cousins ranging from age of late 40's to late 70's. She is the first from that generation to have any neurological issue. Her father passed at 76 with due to lung cancer, but he had a mild case of parkinsons that he developed around 68. Both of her fathers parents lived to 82 and 87 without any neurological problems. The neurologists / ALS specialists we've spoke with don't feel there is a strong genetic predisposition based on the pedigree.

Just wondering what you guys think. At this point it is apparent whatever she has is very, very bad but it just seems so quick based on what I've heard about ALS.
 

lgelb

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I am so sorry. Has BiPAP been tried? That would normally precede any consideration of oxygen in ALS. Her history as I understand it does not sound like ALS, nor hereditary, but it still might help.

If/as she passes, you might wish to authorize a post-mortem study to understand the true nature of her disease.

Once again, I am very sorry.

--Laurie
 

Atsugi

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BMgilst, I'm not a doctor, but this just doesn't seem like what I think I know about ALS. It's more like MMN. The EMGs are apparently pointing at LMN problems, and the good response to IVIG also points to MMN, not ALS. The clinical finding of no upper motor neuron signs at time says to me, in my naive and inexpert mind that the second opinion of ALS is unsupported.

Again, I'm no doctor and certainly no expert, and maybe I'm got everything wrong, but it seems to me like this is not ALS.

As to your specific question: Yes, ALS can possibly that fast, but it is rare to be that fast. My own wife was dead just 10 months after her very first symptom. If your mom has ALS, this would be the fastest progression that I've ever heard of in the several years I've been on this site.

If I were you, I would call up a major MND center and see if they want her brain and brain stem donated for research purposes. Then ask them to confirm cause of death was ALS or not. They can tell if it is an inheritable form or not.

Sorry to hear you're going through all this. I know it's difficult and sad and confusing, too. But to ease your mind about a genetic link to ALS, I would ask an MND research center to determine cause of death and specify the gene involved, if it is ALS.
 

Bmgilst

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Thanks for the responses. Mom went home to be with the Lord today about an hour after I wrote this.
 

KimT

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Prayers of comfort for your whole family. She has been set free.
 

lgelb

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My condolences on your loss. Your mom was lucky to have you at her side.

Best,
Laurie
 

pittsburghgal

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I am so sorry for your loss. My prayers are for you and your family.

The significant response to IVIG makes this unlikely to be ALS.
 

Bmgilst

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Thanks for the comments all. We want to respect her wishes and we didn't specifically ask her about donating her brain to research. We may get enough blood for a DNA test so we can have genetic testing done at some point in the future.

It just seems so fast. She went from breathing fine to being unable to breathe in about two weeks. She also went from eating difficult to chew foods ( cube steak, roast beef, etc. ) without any issue to not being able to drink water within about ten days. It just blows my mind.
 
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