Amyotrophic lateral sclerosis (ALS) is the most common form of motor neurone disease (MND). It is a progressive neurological condition in which motor neurones in the brain and spinal cord stop working properly, leading to increasing muscle weakness.
In the UK, the broader term motor neurone disease is often used, while ALS is common in North America and in medical literature. Oxford University Hospitals explains that nearly 90 percent of people with MND have the mixed ALS form, which is why the terms are often used interchangeably.
Terminology
Motor neurone disease is an umbrella term for several related conditions affecting motor neurones. ALS involves both upper motor neurones, which run from the brain down the spinal cord, and lower motor neurones, which supply muscles.
The term amyotrophic lateral sclerosis describes two features: muscle wasting from lower motor neurone damage and scarring of lateral motor pathways from upper motor neurone damage.
Other forms of MND include progressive bulbar palsy, progressive muscular atrophy, and primary lateral sclerosis. These categories describe patterns of involvement, but individual progression can still vary.
Symptoms
Early symptoms may include:
- Weakness in the hands, arms, legs, or feet.
- Tripping, foot drop, or difficulty climbing stairs.
- Dropping objects or difficulty gripping.
- Muscle twitching, cramps, or stiffness.
- Slurred speech.
- Swallowing difficulty.
As ALS progresses, it may affect walking, arm function, speech, swallowing, breathing, saliva control, and communication. Some people also experience changes in mood, behaviour, or thinking.
Diagnosis
ALS can be difficult to diagnose early because symptoms may resemble other neurological or muscular conditions. Assessment is usually led by a neurologist.
Tests may include neurological examination, blood tests, nerve conduction studies, electromyography, and MRI scans to exclude other causes. Diagnosis is based on the pattern of symptoms, examination findings, progression, and test results rather than one single test.
Causes and Risk Factors
The cause is not fully understood. For most people, ALS appears to arise from a combination of genetic susceptibility and other factors. NHS material states that about 1 in 10 people with MND have a family history of the condition.
Known genetic associations include changes in genes such as C9orf72, SOD1, TARDBP, and FUS, but many cases do not have an identified inherited cause.
Treatment and Care
There is currently no cure for ALS. Treatment aims to slow progression where possible, manage symptoms, maintain communication and independence, support nutrition and breathing, and plan care around the person's priorities.
Care is usually multidisciplinary. It may involve neurologists, specialist nurses, physiotherapists, occupational therapists, speech and language therapists, dietitians, respiratory teams, palliative care, psychologists, social care, and equipment services.
Riluzole may be offered for the ALS form of MND and can help slow progression. Other treatment is directed at symptoms such as cramps, stiffness, saliva, breathlessness, pain, anxiety, swallowing difficulty, communication problems, and respiratory weakness.
Breathing, Nutrition, and Communication
ALS can weaken breathing muscles. Non-invasive ventilation may be considered when respiratory weakness develops. It can improve symptoms and quality of life for some people.
Swallowing difficulty can affect nutrition, hydration, and aspiration risk. Dietetic advice, texture changes, supplements, and feeding tube options may be discussed.
Speech may become weaker or less clear. Speech and language therapists can support voice banking, communication aids, swallowing assessment, and strategies for daily communication.
Prognosis
ALS is usually life-shortening, but the rate of progression varies widely. Some people deteriorate over months, while others live for many years. Prognosis depends on the pattern of disease, respiratory involvement, nutrition, age, general health, and response to supportive care.
Notable People
Public awareness of ALS has been shaped by people such as Lou Gehrig, Stephen Hawking, Rob Burrow, and Doddie Weir. Their experiences are not typical of every case, but they helped bring attention to research, care, and support needs.
References
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