Amyotrophic Lateral Sclerosis: A serious neurological disease

What is amyotrophic lateral sclerosis?

Amyotrophic lateral sclerosis (ALS) is a terminal disorder characterised by the death of the motor nerve cells which control the voluntary muscles. 

Motor nerve cells are responsible for the movement of legs, arms and the face. Degeneration and death of such nerve cells lead to loss of movement, speech and finally affects the ability to breathe. ALS is also known as Lou Gehrig’s disease or motor neurone disease.

ALS

Causes and risk factors

Causes

No single factor has been identified to cause ALS.  Genetic mutations have been reported in some familial cases of ALS. Toxic exposures, oxidative stress and immune system abnormalities are thought to cause ALS.

Risk Factors

Around 5-10% cases of ALS are inherited or of familial nature and 90-95% of ALS cases arise sporadically i.e. they occur without any family history.

Symptoms and signs

The symptoms of ALS vary from individual to individual. Usually, the symptoms are very subtle in nature, beginning in the muscles responsible for swallowing and speech or in the arms, and legs. The symptoms are progressing weakness and paralysis of the voluntary muscles.

When ALS affects the respiratory system, permanent ventilator support may be required for sustaining breathing.

The sense of sight, hearing, touch, smell and taste are not impacted as only voluntary nerve cells are affected by ALS.

Diagnosis

ALS is difficult to diagnose. A series of diagnostic tests and clinical examinations are required to rule out other diseases. Some of the tests used for the diagnosis of ALS include:

  • A comprehensive neurological examination
  • Nerve conduction velocity (NCV)
  • Electromyography (EMG)
  • Urine and blood tests
  • Parathyroid and thyroid hormone levels
  • Myelogram
  • Biopsy of muscles/nerve
  • Spinal tap
  • Magnetic resonance imaging (MRI) and X-rays

Treatment and prevention

Treatment

ALS can’t be reversed. The treatment is aimed at slowing the progression and preventing unwanted complications. An integrated approach with a team of doctors and health professionals is usually involved in the management of ALS.

Medication like riluzole is reported to slow down the progression of ALS in some individuals. Medications for managing spasms, muscle cramps, spasticity, depression and drooling may be prescribed.

Breathing: ALS makes the respiratory muscles weak and gives rise to breathing difficulties. Devices may be provided to aid breathing in the night. Non-invasive or mechanical ventilators may be recommended depending upon the impact of ALS.

Physical and occupational therapy may be employed to promote mobility, adaptive equipment may aid in maintaining independence and performing daily activities.

Communication issues arising out of ALS may be addressed by a speech therapist. In later stages, computers and tablets may be used.

Individuals with ALS also require psychological and nutritional support by their caregivers and family members.

Prevention

There are no preventive measures for ALS as the causes are not clear.

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Complications

Following complications can arise with the progression of ALS:

  • Breathing difficulties: Gradually the respiratory muscles get paralysed and hinder breathing. Individuals with ALS often suffer from respiratory failure that causes death.
  • Dementia
  • Communication issues
  • Difficulties in eating and swallowing food

Next steps

On noticing any signs and symptoms of ALS, visit your physician. Muscle weakness and fatigue need not always mean ALS.

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References

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  1. ALS: Amyotrophic Lateral Sclerosis. The Muscular Dystrophy Association. Accessed 4 Mar. 16. https://www.mda.org/disease/amyotrophic-lateral-sclerosis
  2. Amyotrophic lateral sclerosis factsheet. National Institute of Neurological Disorders and Stroke. Accessed 4 Mar. 16. http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/ALS.htm
  3. What is ALS? Amyotrophic Lateral Sclerosis Society of Canada. Accessed 4 Mar. 16. https://www.als.ca/en/node/63
  4. What is ALS? The ALS Association. Accessed 4 Mar. 16. http://www.alsa.org/about-als/what-is-als.html?

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