Anterior horn cell disorder

[manivish]

Hello everyone,

My mother in law met with an bus accident in which she fell on her back inside a bus. After a month or two she started feeling immobility (gradually) in her right arm and shoulder. Thinking this could be due to the accident, she did the normal massaging, heat warming etc and slowly her shoulders started narrowing down. After a few months this spread to her left arm .. the motion was restricted more from shoulders to elbows. We started accupressure, aqua pressure and other means of mobility treatments. However nothing helped. Now after 2 years the matter is worse .. both hands are almost immobile and the right leg has started showing similar signs. All her reports - blood report, MRI etc were all normal i.e. showing no sign of any clot or pain area ... also she never experienced any pain in her body. Her hands just started getting cold as if the there is no blood supply.
Few months ago, we visited a neurologist who diagnosed her with AHCD - anterior horn cell disorder. She is now following the medicines prescribed (mainly Riluoter) and exercises that have been recommended. Please share us with your knowledge and suggest any treatments that will help her recover sooner. She is now quite depressed.

 

[lgelb]

Re: Mother in law diagonized with AHCD - anterior horn cell disorder - need help

There is more than one anterior horn disorder, so that is a vague label. Is that the most specific thing you heard? Where was she seen?

From your mention of cold, I might have thought a syrinx following the accident was suspected (which also is the only anterior horn dz that people really "recover" from because it is a lesion that can be drained) but then you also mention a normal MRI. She needs a re-evaluation and second opinion at a neuromuscular disease center, such as UNC.

 

[manivish]

Thank you Igelb for your response. As you correctly mentioned that Anterior Horn Cell Disorder is a vague and broad label and we are still unable to understand what exactly the issue is under the broad label of AHCD. Doctors are of the opinion that it is not ALS or MND (considering the fact that she does not have any pain and also that the disease is currently restricted to the limbs - hands and legs). We did get an EMG test conducted and the report mentioned the below which we are not clearly able to decipher
1. Upper Limb NCVs revealed a normal background, with bilateral C8/T1 motor axon loss
2. Needle EMG of bilateral C5-T1 muscles suggested diffuse AHCD
3. Needle EMG of the left L4/L5 segmental muscles also revealed similar features.

I am attaching a few reports and would be highly grateful if somebody can provide an insight on whether this is anywhere related to ALS or MND or any thing else. This will help us move to the next step.

Also here are some of the differences compared to the symptoms of ALS that were observed in her
1. The problem of nerve weakening started only after the bus accident (falling on her back inside the bus)
2. She never experienced any pain in her limbs or back
3. Her hands turned blue after a couple of weeks after the injury
4. Her hands remained cold and she had to externally apply heat and wear warm clothes to keep them warm
5. She experience numbness in her fingers but never twitching or pain


Any help and guidance will be greatly appreciated

 

[lgelb]

I don't see any reports?
Lack of bulbar dysfunction doesn't mean anything in limb onset disease. Bulbar sx can occur years later. Pain isn't a criterion for MND. It comes with prolonged immobility, awkward positions and such.
As you say, numbness isn't a criterion either. So the two together point elsewhere. Cold/blue hands could mean Raynauds. Was that explored?
Without more EMG detail, I don't know what evidence they saw for "diffuse AHCD," or even what they think that means. But I don't see any relationship with ALS from what you have presented.

 

[manivish]

Thanks Igelb.

Attaching the reports here ... http://tinyurl.com/prnjskb

Could you please throw more light on what you mean by "Lack of bulbar dysfunction doesnt mean anything in limb onset disease".

 

[lgelb]

Mani, many cases of limb onset ALS don't present with bulbar features until, often, considerably later. And in some cases, not at all. So that is why I said that the fact that there have been no bulbar sx doesn't mean that it is not ALS. Certainly, she has something.

Was a Doppler ultrasound on her arm(s) done? Cold, numbness, paralysis, post-trauma -- emboli/compression mechanisms do seem a distinct possibility.

What I got most of all from your test reports/notes, which the next clinic assuredly is going to repeat/expand, is that your MIL needs to see a new doc (at the Carolinas ALS center or whatever someone else on the forums wants to recommend). Have you contacted someone there about an appointment?

Phone: 704-446-1900

As to the PT this guy recommended, I don't know what she's been doing, but I wouldn't be doing anything difficult, strenuous or doubtful. Since his rx is not really on [any] point, I'd just do whatever gentle stretching, walking, swimming and the like she can handle. Passive range of motion exercises for her arms, massage and gentle stretches/movement by you of her wrist, fingers. Even immobile parts need that.

There is a lag time so I would call straightaway and tell them you need a second opinion for ALS. I know, that's not what the note says, but it doesn't say anything coherent, so say what's going to get you an appt.

We would always recommend a 2nd opinion at an ALS center anyway, so there is no point in retracing the steps you took. I know it's been hard. You will have better answers soon.

Best,
Laurie

 

[Dusty7]

I agree with Laurie's recommendations.

Understand that the anterior horn cells are also known as lower motor neurons, and lower motor neuron disease is a form of ALS. Motor neuron disease spreads from solely upper motor neuron symptoms (PLS-primary lateral sclerosis) at one end of the spectrum to solely lower motor neuron symptoms (PMA-progressive muscular atrophy) at the other. In the middle is what is generally called ALS, with both upper and lower motor neuron symptoms--but everything on the spectrum is also called ALS.

Nothing in your MIL's story says ALS to me. What is does say is that your MIL needs to go to an ALS specialist and get checked out thoroughly.