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Amyotrophic lateral sclerosis (ALS): Symptoms, diagnosis and treatment

What is amyotrophic lateral sclerosis?

Amyotrophic lateral sclerosis (ALS) is the most common type of the incurable, progressive brain disorder motor neurone disease (MND).

The most famous person with ALS and MND is the scientist Professor Stephen Hawking.

ALS can cause muscle wasting, weakness, twitching, speech and swallowing problems, and muscle spasms.

Motor neurone disease shortens a person's life expectancy with survival times of around 3-4 years after symptoms first develop.

Around 5,000 people in the UK have motor neurone disease.

What causes ALS?

Motor neurone disease occurs as the body's motor neurones lose their function, although it is not known why.

Genetics appear to play a role in around 5% of cases, called familial motor neurone disease.

Where there is no close family history of motor neurone disease, it is called sporadic motor neurone disease.

The overall cause of MND may be a combination of genetic and environmental factors.

What are the symptoms of ALS?

In the early stages, symptoms of ALS include:

  • An increasing weakness in one limb, especially in a hand
  • Difficulty walking
  • Clumsiness with the hands
  • Impaired speech
  • Difficulty swallowing

As the ALS progresses, symptoms may include:

  • A weakening of other limbs, perhaps accompanied by twitching, muscle cramping, and exaggerated, faster reflexes
  • Problems with chewing, swallowing, and breathing; drooling may occur
  • Light twitches under the skin
  • Eventual paralysis

Seek medical advice about ALS if:

You have any of the above symptoms - ALS requires professional medical care.

How is ALS diagnosed?

Diagnosis of ALS is not straightforward and may take a while. A number of tests may be performed including an electromyogram (EMG) to test for nerve damage, and other tests such as MRI and CT scans to help rule out other causes of the symptoms such as muscular dystrophy, multiple sclerosis, spinal cord tumours, and other diseases.

What are the treatments for ALS?

Riluzole is a drug for the treatment of ALS. How it works is not exactly known, but it seems to prevent damage that can result from the nerve cell being overexcited by glutamate. Studies show it may improve functioning and survival. Because of potential side effects involving the liver, close monitoring by your doctor is required.

Physiotherapy can improve circulation and help prolong muscle use in the early stages of ALS. In addition, various medications may be prescribed as the disease progresses to help with symptoms, such as painkillers.

Botulinum toxin and baclofen can be used to help relieve muscle stiffness. Muscle decline and weight loss can be slowed with nutritional supplements called branched-chain amino acids (BCAAs). Phenytoin and carbamazepine may ease cramps. Medication can help control excess saliva production, one of the symptoms of ALS. Antidepressants may also be prescribed to help with depression that often accompanies such a severe disease.

WebMD Medical Reference

Medically Reviewed by Dr Rob Hicks on October 26, 2017

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