Motor Neuron Disease

Motor neuron disease is uncommon degenerative disease of the central nervous system that at present has no definite treatment.

Symptoms:

Symptoms usually start after age of 50 years but can occur at young age too. Patient usually notices decrease in hand grip of either hand. Patient can have difficulty in talking or swallowing. Sometimes patient has difficulty in getting up from the sitting position or may notice weakness of either leg or foot. Patient may notice frequent muscle twitching and wasting of muscles of hands or any other body part. The disease progresses over time and eventually patient may get wheel chair or become bed bound.

Investigations:

Doctor may run battery of tests including some baseline tests and advanced blood tests. Doctor may order MRI of your brain and spine. Nerve conduction studies and electromyography (NCS/EMG) are always ordered.

Treatment:

Treatment is usually supportive. No definite treatment is available at the moment. Those who have difficulty swallowing may get help with NG tube or feeding gastrostomy.

Physiotherapy:

Physiotherapy is very important in treatment of patients of motor neuron disease especially in those patients who have limb weakness. Those patients who are on wheel chair or bedridden, physiotherapy helps preventing the contracture formation.

Frequently Asked Questions:

If the patient has difficulty swallowing, what are the options?

Patient can get benefit from NG tube or feeding gastrostomy.

Does motor neuron disease cause respiratory paralysis?

Yes in advanced cases, patient may develop weakness of muscles of respiratory tract and may need oxygen supplementation or CPAP or BiPAP.

Is tracheostomy necessary for a patient who has problems with breathing?

It is totally the patient’s choice. Difficulty with breathing can be helped with oxygen supplementation or CPAP or BiPAP. Eventually the respiratory difficulty gets worse and then patient may opt for tracheostomy.

What is the benefit of drug Riluzole?

It can add just a couple of months to patient’s life or tracheostomy free period in patients with amyotrophic lateral sclerosis. It is NOT the definite treatment.

What is the benefit of drug edaravone (Radicava)?

This drug may slow the functional decline but again it is NOT the definite treatment.

Whether the patients of motor neuron disease can develop dementia?

Yes some patients of motor neuron disease can develop dementia later in the course of disease.

Is motor neuron disease a group of diseases?

Yes it is a group of diseases that has four types. Commonest are amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA). Severity and prognosis will depend upon the type of the disease.

Are all types of motor neuron disease fatal?

No, only amyotrophic lateral sclerosis (ALS) and some types of spinal muscular atrophy  (SMA) are usually fatal.

Is motor neuron disease a common disease?

No, this is an uncommon disease.

What is the life expectancy in motor neuron disease?

Life expectancy in motor neuron disease is less than normal individuals. Please ask your doctor for details.

Is motor neuron disease painful?

No, it is a painless condition.

How to take care of bedridden or wheelchair bound patient?

Please see section on “taking care of paralyzed patients

Is there any definite cure for motor neuron disease?

At present there is no definite cure for motor neuron disease but many trials are under way.

Disclaimer:
The information given here is intended for use of general public . It should not be taken as standard medical reference.

About the Author:

One of the most well known neurologists in Lahore, he is serving as Assistant Professor of Neurology at Lahore General Hospital. He is a graduate of King Edward Medical College and did residency in general medicine & neurology at Mayo Hospital Lahore. He did FCPS in neurology and moved to Lahore General Hospital, and has been working there since.

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