Motor neuron disease treatment

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Motor neuron diseases are progressive nervous system disorders affecting motor nerve cells in the cerebral cortex and spinal cord. Treatment of MND consists mainly of supportive and symptomatic therapy. Only Riluzole and Edaravone may provide a limited survival effect. The most modern and breakthrough methods are Stem cell therapy and Gene therapy.

Motor neuron disease is not only a disorder but a group of rare neurological conditions in which motor neurons - the nerve cells that control skeletal muscle movement – progressively degenerate. Loss of motor neurons results in muscle weakness gradually worsening over time, impairing the ability to move, eat, speak, or even breathe.

Depending on the severity of the disease, damage can occur to the motor nerve cell in the brain and spinal cord ('1st or upper motor neuron') with the resulting spastic paralysis or to the motor nerve cell that pulls from the spinal cord to the muscles ('2nd or lower motor neuron') with the resultant flaccid paralysis.

The many forms of motor neuron disease are classified according to whether the loss of neuron function is inherited or sporadic and whether the condition affects 1st or 2nd motor neurons:

  • Amyotrophic lateral sclerosis (ALS) is the most common form, which affects both motor neurons. ALS usually strikes midlife, but it can occur in people of any age. Most ALS cases are sporadic. Only 10% are inherited, with at least a dozen genes involved.
  • Spinobulbar muscular atrophy, or Kennedy's disease, is a form of spinal muscular atrophy (SMA) caused by mutations in the androgen receptor gene on the X chromosome. This disease typically strikes in adulthood and primarily affects men (X-linked inheritance). The disease progresses very slowly, with most patients having an average life expectancy.
  • Progressive bulbar palsy (PBP) affects the lower motor neurons that originate from the brainstem, also known as the bulbar region. PBP is sometimes considered a subtype of ALS because most PBP patients eventually also develop widespread muscle weakness.
  • Primary lateral sclerosis (PLS) affects only upper motor neurons. PLS progresses much more slowly than ALS and is not usually fatal. However, because many ALS cases start similar to primary lateral sclerosis, a PLS diagnosis is definitive only after at least four years.
  • Pseudobulbar palsy (involuntary emotional expression disorder) affects the ability to control facial (including the jaw), mouth (such as the tongue) and throat muscles. It involves such vital functions as the ability to speak, eat and swallow. In addition, there may be uncontrolled crying or laughing at inappropriate times (pseudobulbar affect or "emotional incontinence").
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The causes of MND

The reasons for the development of the disease have not been thoroughly studied, but research in recent years indicates its multifactorial nature. There is a possible role of:

  • Genetic factors;
  • Environmental factors;
  • Impaired glutamate metabolism;
  • Free radical damage;
  • Defects in cytoskeleton proteins;
  • Autoimmune and inflammatory mechanisms;
  • Viral infections;
  • Exposure to toxins;
  • Excessive physical exertion;
  • Smoking.

Motor neuron disease affects men more often and usually manifests itself between the ages of 50 and 70. In about 5 -7% of patients, the disease is congenital and is present in family members.

Usually, motor instructions from the brain are transmitted to the muscles via two neurons: an upper and a lower motor neuron. Therefore, lesions of upper and lower motor neurons affect the muscles in different ways:

  • Losing upper motor neuron function often leads to increased muscle tone, muscle stiffness (spasticity), and over-responsive reflexes.
  • Lower motor neuron lesions often result in reduced muscle tone, flaccid paralysis, muscle wasting and twitching.

Each form of the disease, however, has its characteristic symptoms:

  • Amyotrophic lateral sclerosis (ALS) starts with spastic paralysis, overactive reflexes in the lower limbs, flaccid paralysis, reduced responses in the upper limbs, and tongue twitching. In the later stage, symptoms may also include slurred speech and difficulty swallowing and breathing.
  • Early symptoms of Kennedy’s disease include tremors, lower limb weakness, and tongue twitching. Late symptoms include widespread muscle weakness and wasting.
  • Primary lateral sclerosis manifests with stiffness, weakness and muscle spasms (spasticity) in the legs, rarely starting in one leg and eventually progressing to arms, hands, tongue and jaw.
  • Progressive bulbar palsy symptoms include difficulty chewing, swallowing, and speaking.
  • Pseudobulbar palsy manifests by dysarthria (slurred speech), dysphagia (difficulty swallowing), dysphonia (spasms of vocal cord muscles producing voice changes), and emotional lability.

Neurological examinations for motor neuron disease

Diagnosing motor neuron diseases requires differentiating from other conditions with a similar appearance. Therefore, a careful clinical-neurological examination by an expert is critical. Therefore, specialists perform diagnostic procedures such as the following to narrow down the possibilities:

  • Electromyography measures the electrical voltage in the muscles to determine whether the cause of the disorder is in the nerves, the neuromuscular junction, or the muscles.
  • Nerve conduction velocity measurements show the speed at which nerves transmit signals. Only the terminal phase of motor neuron disease has speed impaired, so if signalling is unexpectedly slow, the symptoms are due to another disorder.
  • Magnetic resonance imaging (MRI) of the brain and sometimes the spinal cord can detect abnormalities leading to similar symptoms (stroke, tumours, intervertebral hernia).
  • Laboratory diagnostics, including genetic examinations, show possible changes in the blood and genetic material that lead to the development of motor neuron disease.
  • Lumbar puncture is a diagnostic method for obtaining liquor (the liquid that washes around the brain and spinal cord). It provides information about acute or chronic inflammatory processes in the brain, meninges and nervous system.

Modern treatment of MND

Science-based medicine has not yet cured motor neuron disease. The treatment consists, on the one hand, of drug measures to delay progression and, on the other hand, of symptom-oriented options. The psychosocial support of patients and relatives is also essential.

Nevertheless, doctors and scientists are constantly researching and finding new effective ways to fight the disease. Some of them are already showing brilliant results, and some are still under development:

  • Therapy with Riluzole, which protects the nerves, is approved as a pathophysiologically based approach. In several studies, this substance showed a significant increase in lifespan by 3-4 months.
  • Edaravone, a new drug, may slow the decline in functioning to some extent in patients with amyotrophic lateral sclerosis.
  • Stem cell therapy is more likely to be effective at protecting existing motor neurons than actually replacing or repairing them. Specialists can achieve it by using stem cells to replace supporting cells, deliver nutrients or reduce inflammation.
  • Gene therapy is one of the best options for treating patients with forms of motor neuron disease caused by gene mutations.

Numerous effective symptomatic therapies also alleviate symptoms such as pain, muscle cramps and restricted mobility. Recognising and treating nocturnal breathing and swallowing disorders is also essential. In addition, certain medicines help eliminate symptoms:

  • Treatment with Baclofen relieves muscle spasticity.
  • Medications such as Phenytoin or Quinine may help with muscle cramps.
  • Anticholinergic drugs, such as Amitriptyline (an antidepressant), may be used to reduce salivation.
  • Treatment with Amitriptyline or Fluvoxamine (also an antidepressant) often helps with mood swings or depression.
  • Doctors prescribe drugs with a mild sedative effect, such as Benzodiazepines, if pain occurs during the disorder.

In addition, depending on the symptoms, doctors recommend:

  • The primary therapy for muscular weakness is regular and intensive physiotherapy on a neurophysiological basis;
  • Ergotherapeutic treatment to maintain hand function and fine motor skills;
  • Logopedic therapy to preserve the process of swallowing and speech;
  • Respiratory therapy to support expectoration, secretion mobilisation and cough function.
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Prognosis for MND patients

Motor neuron disease, in most cases, leads to paralysis of the respiratory muscles, which is the primary cause of death: 50% of patients die in the first three years from the onset of the disease, and 20% live for five years, and 10% - 10 years. Survival for more than 30 years is rarely noted.

With dysphagia, the prognosis is poor; respiratory complications resulting from aspiration lead to death in 1–3 years. In the case of primary lateral sclerosis and progressive muscle atrophy, life expectancy increases due to the low risk of aspiration and pneumonia; complete disability develops after more than 20 years.

FAQ

Can you recover completely from MND?

There is no effective therapy for MND, and no treatment will significantly alter its course. The doctor may prescribe symptomatic medications to control involuntary muscle twitching, muscle cramps and excess saliva. However, treatment focuses on retaining function and quality of life and providing comfort.

What are the latest options available for motor neurone disease?

Active clinical research continues as new data open up new treatment options. The priority areas of research are the molecular and genetic mechanisms of motor neuron death, innovative methods of treatment, the possibilities of stem cell therapy, and the detection of biomarkers of the disease, which help detect the condition in the early stages and determine the effectiveness of therapeutic interventions.

Can MND go into remission?

Only a few cases are reported with spontaneous remission of motor neuron disease. Specialists can't explain the exact reason for these cases, and modern treatment for MND does not show the definite possibility of remissions. However, it is better if you always consider the possibility.

Can stem cell treatment cure MND?

Currently, in the context of MND, stem cells are mainly used to decrease symptoms and slow progression. Nevertheless, stem cell transplantation continues to be a future research avenue. Pilot studies suggest the procedure is safe and may even be beneficial. However, this has yet to be confirmed in larger cohorts of people with MND. 

Has any advancement been made in treating ALS?

Research conducted in recent years significantly deepened the understanding of the causes and mechanisms of the disease. In addition, it made it possible to develop drugs that somewhat slow down the progression of the disease, prolong the patient's life, and postpone the transition to mechanical ventilation of the lungs: Riluzole (blocker of glutamate release) and Edaravone (inhibitor of oxidative damage to brain cells).

Where can I get Motor neuron disease treatment?

Germany, Turkey, Spain, Czech Republic, Lithuania are among the best for Motor neuron disease treatment.

5 countries and 15 cities for Motor neuron disease