About motoneuron disease

What is motoneuron disease?

Motor neuron disease comprises a group of severe disorders of the nervous system characterized by progressive degeneration of motor neurons (neurons are the basic nerve cells that combine to form nerves). Motor neurons control the behavior of muscles. Motor neuron diseases may affect the upper motor neurons, nerves that lead from the brain to the medulla (a part of the brain stem) or to the spinal cord, or the lower motor neurons, nerves that lead from the spinal cord to the muscles of the body, or both. Spasms and exaggerated reflexes indicate damage to the upper motor neurons. A progressive wasting (atrophy) and weakness of muscles that have lost their nerve supply indicate damage to the lower motor neurons.



What are the symptoms for motoneuron disease?

Weakness in your ankle or leg – you might trip symptom was found in the motoneuron disease condition

Symptoms of Motor Neuron Disease are as follows:

1. Amyotrophic Lateral Sclerosis: ALS influences your upper as well as your lower motor neurons. Gradually, your control over the muscles that aid you in walking, speaking, chewing, swallowing and breathing is lost. They grow weaker and waste away as time passes. You could experience stiffness and twitches in the muscles.

2. Primary Lateral Sclerosis: PLS only impacts the upper motor neurons. Here, speech is slurred and slowed down, along with increasing Weakness and stiffness in your arms and legs, deficient coordination and balance.

3. Progressive Bulbar Palsy: PBP is a former stage of Amyotrophic Lateral Sclerosis. Several patients with this disorder develop ALS at some point. Difficulty controlling your emotions, Trouble speaking straight, chewing and swallowing.

4. Pseudobulbar Palsy: Symptoms exactly similar to Progressive Bulbar Palsy

5. Progressive Muscular Atrophy: This is rarer than ALS. It’s known to start in your hands, and later the Weakness spreads to other body parts. Muscles weaken and might cramp. It can modify into Amyotrophic Lateral Sclerosis.

Spinal Muscular Atrophy
This results in weak arms, limbs and the trunk. There are three types based on symptoms:
1. Type-1: Children cannot involuntarily sit or hold their heads high. Weak muscle tone, slow reflexes, trouble in swallowing and breathing.

2. Type-2: While these kids can sit on their own, they are unable to stand and walk. They have trouble breathing.

3. Type-2: Kids with this may have a curved spine and shorter muscles or tendons around their joints.

Kennedy’s Disease: Shaky hands, muscle cramps and twitching, Weakness in the face, arms and legs, enlarged breasts and a low sperm count is prevalent here.

Symptoms
Weakness in your ankle or leg – you might trip, or find it harder to climb stairs,Slurred speech, which may develop into Difficulty swallowing some foods,A weak grip – you might drop things, or find it hard to open jars or do up buttons,Muscle cramps and twitches,Weight loss – your arms or leg muscles may have become thinner over time,Difficulty stopping yourself from crying or laughing in inappropriate situations
Conditions
Amyotrophic Lateral Sclerosis,Primary Lateral Sclerosis,Progressive Bulbar Palsy
Drugs
NA



What are the causes for motoneuron disease?

Motor Neuron Disease is a branch of serious conditions of the nervous system. It causes anomalies in the nerve. It is a continuous wasting away and weakness of muscles losing their nerve supply. It damages the upper and lower motor neutrons.

Causes of Motor Neuron Disease
1. Glutamate
2. Immune System Issues
3. Mitochondria Issues
4. Oxidative Strain

Here are causes based on the nature and subtype of Motor Neuron Disease

1. Amyotrophic Lateral Sclerosis (ASL): It is medically defined as “sporadic”, which is to say that absolutely anyone can be affected. It is more common in people under the age group of 40-60 years old. It is a fatal disease in nature.

2. Primary Lateral Sclerosis (PLS): Primary Lateral Sclerosis targets individuals in the same age group as ALS. The muscles are prone to get stiffer and weaker over time. However, people don’t die from this like it is possible in ALS.

3. Progressive Muscular Atrophy: This can be hereditary or random. It is much less common than both Amyotrophic Lateral Sclerosis and Primary Lateral Sclerosis.

4. Spinal Muscular Atrophy: Spinal Muscular Atrophy is a hereditary condition caused by the gene SMN1. It damages the lower motor neurons.

5. Kennedy’s Disease: Restricted to only the male population, it is an inherited disease. Females can carry this defect in their genes, but they don’t fall ill from it. However, there is a 50 percent chance that the woman passes it on to her offspring.

Symptoms
Weakness in your ankle or leg – you might trip, or find it harder to climb stairs,Slurred speech, which may develop into difficulty swallowing some foods,A weak grip – you might drop things, or find it hard to open jars or do up buttons,Muscle cramps and twitches,Weight loss – your arms or leg muscles may have become thinner over time,Difficulty stopping yourself from crying or laughing in inappropriate situations
Conditions
Amyotrophic Lateral Sclerosis,Primary Lateral Sclerosis,Progressive Bulbar Palsy
Drugs
NA



What are the treatments for motoneuron disease?

Motor Neuron Disease is a rare disorder that causes a modification in the nerves, directly detrimental to the nervous system. The weakness it results in only gets worse in time. It is possible that some people affected by this condition still have several years ahead of them.

Treatments for Motor Neuron Disease
1. While there is no standard cure, as such, for Motor Neuron Disease, there are treatment alternatives to lessen the dominance of the impact of the disease on the patient’s daily lifestyle.

2. Riluzole is an ALS treatment approved by the Food and Dug Administration. It slows down the pace of this disease. As glutamate is an excitotoxin linked to neuronal destruction, this treatment focuses on bringing down the glutamate levels in a body.

3. Radicava is another treatment approved by the FDA to slow down the degeneration of physical function to one-third of it.
Symptoms
Weakness in your ankle or leg – you might trip, or find it harder to climb stairs,Slurred speech, which may develop into difficulty swallowing some foods,A weak grip – you might drop things, or find it hard to open jars or do up buttons,Muscle cramps and twitches,Weight loss – your arms or leg muscles may have become thinner over time,Difficulty stopping yourself from crying or laughing in inappropriate situations
Conditions
Amyotrophic Lateral Sclerosis,Primary Lateral Sclerosis,Progressive Bulbar Palsy
Drugs
NA



What are the risk factors for motoneuron disease?

Risk factors for Motoneuron Disease
1. Affects more males than females
2. Veterans are more likely to develop this disease
3. Patients with Alzeihmer’s disease
4. Neurodegenerative conditions
5. Individuals in their 60s and 70s
6. Having family members with this disease
7. Immunity system too exposed to different viruses
8. Vulnerable to toxins and various chemicals.
Symptoms
Weakness in your ankle or leg – you might trip, or find it harder to climb stairs,Slurred speech, which may develop into difficulty swallowing some foods,A weak grip – you might drop things, or find it hard to open jars or do up buttons,Muscle cramps and twitches,Weight loss – your arms or leg muscles may have become thinner over time,Difficulty stopping yourself from crying or laughing in inappropriate situations
Conditions
Amyotrophic Lateral Sclerosis,Primary Lateral Sclerosis,Progressive Bulbar Palsy
Drugs
NA



Is there a cure/medications for motoneuron disease?

Definition of Motor Neuron Disease:
Motor neurons are responsible for your primary body movements like, speaking, chewing, swallowing and breathing. Upper motor neurons are situated in the brain to send messages from the brain to your spinal cord while lower motor neurons are transmitters in the spinal cord, carrying messages from the brain to your muscles.

Cure/medications for Motor Neuron Disease
Although there is no identified cure, there are a number of medications available to get the symptoms under control.
1. Edaravone (Radicova)-Slows the progression of the disease.

2. Nusinersen (Spinraza)-Declines the rate of progression.

3. Onasemnogene abeparvovec (Zolgensma)-Slows the pace of the symptoms.

4. Botulinum toxin (Botox)-Helps with muscle stiffness.

5. Baclofen (Lioresal)-A muscle relaxer.

6. Ibuprofen (Advil, Motrin)-For muscle cramps, spasms, severe pain in the joints and muscles.

7. Destromethopan hydrobromide (Neudexta)-Helps to relieve one from abrupt crying and laughter-- a symptom of Motor Neuron Disease.
Symptoms
Weakness in your ankle or leg – you might trip, or find it harder to climb stairs,Slurred speech, which may develop into difficulty swallowing some foods,A weak grip – you might drop things, or find it hard to open jars or do up buttons,Muscle cramps and twitches,Weight loss – your arms or leg muscles may have become thinner over time,Difficulty stopping yourself from crying or laughing in inappropriate situations
Conditions
Amyotrophic Lateral Sclerosis,Primary Lateral Sclerosis,Progressive Bulbar Palsy
Drugs
NA



Video related to motoneuron disease