Questions posted in the Neurology and Neurosurgery Forum have been answered by doctors from The Cleveland Clinic Foundation.

Question Title: immune-mediated motor neuropathy

Forum: Neurology Forum
Topic: AMYOTROPHIC LATERAL SCLEROSIS - ALS

I am 31 years old and have been experiencing muscle twitches, muscle cramps and hyper reflexia for the past 4 weeks. Just this past week I have noticed that there is weakness in both my arms and calves (since can't lift things etc.) and I keep getting recurring slurred speech. Can ALS progress this fast so that in 4 weeks I can go from feeling perfectly normal to having trouble using my arms and legs and having slurred speech?
I also have several members in my family who have autoimmune diseases (two sisters included) and was wondering if ALS is thought to be an autoimmune condition (in that the body attacks the motor neurons or calcium channels etc.).
Does anyone know anything about the disease called immune-mediated motor neuropathy. I read in medline that it is similar to Motor Neuron Disease but that it is definitely mediated by the immune system and that it has been shown to respond to immunosuppressants.
Lastly, are there other motor neuron diseases similar to ALS but that have a slightly different pathogenesis and prognosis?
Any input would be greatly appreciated for I can't get any information on these inquiries as of yet.

________


Certainly the symptoms that you describe - cramps, muscle twitches, and hyperactive reflexes - are all symptoms seen in Amyotrophic Lateral Sclerosis ( ALS or Lou Gherig's Disease), but at times these are very nonspecific symptoms and you have a lot of atypical features too. First, ALS generally presents in older individuals - Avg Age 55-60 years old. Secondly, the progression in ALS is not typically as fast as you describe - progression is over months to a few years - you describe a Four week course. Now certainly there have been unusual cases that appear in younger people or progress this quickly, but this is very far from the norm and should prompt a search for something else. It sound though that you have been quite affected in the past four weeks so if you've not been evaluated by a neurologistr yet - this is a priority. A history and physical should be able to define your symptoms better (for example what are these twitching) and tell just what level of the nervous system the problem is at. For example, a disorder purely in the Central Nervous Systme (brain and spine) can cause weakness, brisk reflexes and even types of muscle twitches (for example spasms or jerking movements). When something is progressing this quickly though, you should be evaluated. If your exam suggests involvement of the central nervous system, imaging like an MRI would help. If the peripheral nervous system is involved, you would need an EMG (electrical test of the nerves and muscles).

Now to your other questions - The cause of ALS is unknown - it involves a degeneration of the motor nerve cells, but it is not an autoimmune disease that we know of, and is not be related to most autoimmune diseases. There is a proportion of ALS though, that does run in families,but if you're asking if it's realated to lupus, Graves disease or multiple sclerosis, the answer is no.

I responded to your question on Multifocal Motor Neuropathy in your other post.

Finally, there are other diseases of Motor Neurons. The motorneuron is a part of the nervous system just like say the celerbellum. And just like tumors, strokes, viruses, etc. can cause cerebellar problems, many disorders can affect motor neurons. But most of these disorders have specific clinical patterns and thus would not be confused with ALS. For example, Polio is a disease of motor neurons, but, if you could find a new case in the U.S. today, you would never mistake it for ALS. A disorder which affects infants or children (generally) called Spinal Muscular Atrophy is a disorder of the lower motor neurons (from the roots in the spine to the muscle). Again though, it would not be confused with ALS. ALS has a pattern with both the Upper Motor Neuron's (from the brain travelling to the spine) and the Lower Motor Neurons being involved - this causes a particular pattern of symptoms and exam findings. The difficulty is when only a few of these findings are present. When atypical features are present (for example, in the young, when urinary or visual complaints are seen, when it progresses very slowly or very rapidly), other less common disorders are considered.

A lot to take in, but I hope this was of help. See a neurologist promptly for evaluation of your problems.


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