motor neron diseases

[tjq]

are there many types and subsequent different effects

[Char1938]

I am a 70 yr old female with diabetes and neuropathy , the pain has gotten worse in my legs and hips so I had a EMG and neuroligest dx plexopathy , I now have pain in my hands with swelling redness and the thumbs have seemed to have locked and i cant bend them , they are painful and so is the index fingers . Also have pain in my shoulders and arms and sometimes I cant even raise my arm it is so painful . Went to a specialist and do not have arthritis . My question is do I have plexopathy in my upper body also ? I have looked up plexopathy but what little is written on it is to deep for me . I do have other medical problems such as CAD ( 2 heart stents, aortic abdom stent ,aortic
bifemoral bipass ) PAD ( leg stents)
My father was dx with Atipical ALS varient but when he outlived the dx they changed it to diabetic amotrophy and several other dxs . depending on what Dr. you talked to . His symptoms were typical als with muscle wasting , weakness , trouble swallowing , ect , except at slower rate about 20 years .
My Dr. knows very little about it , In fact when I got the plexopathy dx I had to spell it for him and tell him what I was told from the specialist .
I have been told it is rare .
thank you in adavace for any imformation on this .
Char

[Esteban Cheng-Ching, MD]

Thanks for using the forum. I am happy to address your questions, and my answer will be based on the information you provided here. Please make sure you recognize that this forum is for educational purposes only, and it does not substitute for a formal office visit with your doctor.
The motor neuron diseases are a group of conditions in which the neurons located in the spinal cord related to motor control of the muscles, are affected. There are multiple conditions, and they are divided in acquired and inherited.
The inherited ones are rare. Usually present in children or young adolescents. There are many types of inheritance and they could be either autosomal dominant or autosomal recessive depending on which type of disease it is. To mention some, Spinal Muscular atrophy is a group of these conditions, and there is an infantile and intermediate and a juvenile form. ALS (Lou Gehrig's Disease) could be inherited in 5%, and there is a juvenile for for ALS as well.
Regarding the acquired forms, usually present in adults. ALS is the most common one, and in this condition there is degeneration of the motor neurons in the spinal cord, as well as the pathways between the brain and these neurons. As I wrote, about 5% could be an inherited form. The cause is not clear at this moment. The clinical presentation could fall into difference categories. Bulbar symptoms when the patient has difficulty swallowing, chewing, coughing, breathing and speaking. There is weakness in the upper and lower extremities, with muscle wasting, and fasciculations or “twitching of the muscles”. The ocular muscles are usually spared. Patients may eventually have respiratory problems.
Other forms of acquired motor neuron disease to be mentioned are: Primary lateral sclerosis, progressive bulbar palsy, poliomyelitis, or other conditions that eventually damage the motor neurons.
Each of the conditions have the common manifestation of weakness, with what neurologist call “lower motor neuron signs”, however the manifestations for each of them could be different.
I think that if you have any concern about a motor neuron disease, you should have an evaluation by a neurologist, who based on a detailed history and physical exam, could tell you what kind of neurologic condition it is. If it is a motor neuron disorder or not, and specifically which one, it is.
I hope that you find this information useful. Good luck.