difference between als and Charcot Marie Tooth

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van1

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PALS
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12/2014
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US
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North Dakota
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Oakes
Can someone tell me the difference between als and Charcot Marie Tooth disease. I know Charcot Marie Tooth is genetic. I would appreciate any info.
 
Hey van. I'm certainly not the expert and not the best at explaining things.

ALS isn't always hereditary where CMT is.

Sensory issues are involved with CMT but no sensory with ALS.

This one I'm probably way wrong, but the way I read it, ALS problems are brainstem and spinal cord related, where problems with CMT are outside these two areas.

If you look up the ninds I think it is, there will be information on both.

I have just been diagnosed with PLS, but I knew early on CMT wasn't an issue to be raised as none of my symptoms fitted.

Hope this helps until someone with more knowledge chimes in.

All the best.
God bless, Janelle x
 
I agree with Janelle. I will add that there are forms of CMT that involve recessive genes so it is possible not to have affected family members. Both your parents would have been carriers

Last we heard Mayo said probable ALS. Is CMT something a second opinion brought up? Why are you asking?
 
A prior neuro asked if I had ALS or CMT in my family. Even though no one in my family has been diagnosed with either, there are some neurological issues. There is so much information about Motor Neuron Disease and I am trying to learn more. I was just reading the symptoms of CMT and I have a number of the symptoms. I should have asked Mayo why CMT is ruled out. For me personally I would need to look at the sensory issue. Thank you for the information. As a side note the Mayo neuro is referring me to a neuromuscular specialist that is closer, and Mayo will receive notes on me in the future.
 
Definitely a question for your next visit. I would not read a lot into that question though. I have been asked all kinds of questions about family history and also about symptoms of other,often rare conditions even though I was a known gene carrier with a very strong family history. They are trained to consider everything and narrow it down most of which happens very quickly until they arrive at a few remaining options that may take time to narrow down
 
CMT or ALS?
CMT is a disease of the peripheral nerves, that is, the motor and/or sensory nerves, affecting the myelin (CMT type 1, 4 and X) or the nerve axon (CMT Type 2). Therefore the problem is between the neurons in the spinal column and the muscles. A nerve conduction study will generally reveal whether the disease is demyelinating or not, and if it is demyelinating the chance of ALS (which is not demyelinating) plus a demyelinating polyneuropathy is rare in the onset stages. Therefore, ALS is generally ruled out if the patient presents with demyelination.

ALS is a disease of the upper motor neurons and/or the lower motor neurons. CMT is a disease of the peripheral nerves and has no upper motor neuron (brain) involvement. Therefore, If the patient shows sign of UMN involvement (hyperreflexia, spasticity, etc.), then CMT can be ruled out.

The lower motor neurons are in the spinal column and are a step rostral (upstream) from the dorsal root ganglion, which is where the motor and sensory nerves coming from the spinal column split into two pairs, one pair going to the limbs and one pair going to the paraspinals.

If an EMG shows denervation in the paraspinals and in the myotome of that paraspinal, there is a strong presumption that the two areas are affected by one disease (that is, a disease affecting the motor neurons of the spinal column, which is called anterior horn cell disease) and it is diagnosed as ALS. If an EMG shows denervation in only the paraspinals or only the limbs, then the problem is distal (downstream) to the dorsal root ganglion (not in the spinal column) so ALS is ruled out.

As a practical matter, the muscles in the limbs frequently show denervation due to radiculopathies and other compression or nerve impingement problems, while the thoracic paraspinals rarely are affected by radiculopathies. So an EMG that shows denervation in a preponderance of thoracic paraspinals is a strong indication of ALS, especially if the myotomes of those paraspinals also show signs of denervation.

Progression
CMT is slower progressing than ALS. CMT is not considered a fatal disease and people with most forms of CMT have a normal life expectancy, though the path can be similar—cane, walker, wheelchair, PWC. There is no cure for either disease.
 
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