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Amyotrophic Lateral Sclerosis (ALS) – Overview

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that damages motor nerve cells, leading to muscle weakness and paralysis.

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Things worth knowing about "Amyotrophic Lateral Sclerosis (ALS)"

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that damages motor nerve cells, leading to muscle weakness and paralysis.

What is Amyotrophic Lateral Sclerosis (ALS)?

Amyotrophic lateral sclerosis (ALS) is a severe, progressive neurological disease in which the motor nerve cells (motor neurons) in the brain and spinal cord gradually degenerate and die. As a result, muscles lose their nerve supply and can no longer function properly. ALS is also known as Lou Gehrig's disease, named after the famous American baseball player who was diagnosed with the condition. The disease affects both upper and lower motor neurons, leading to progressive muscle wasting and paralysis.

Causes

The exact causes of ALS are not yet fully understood. The following factors are discussed:

Genetic factors: In approximately 5–10% of cases, ALS is inherited (familial ALS). Mutations in genes such as SOD1, C9orf72, FUS, and TARDBP have been identified.
Sporadic form: The majority of cases (approximately 90–95%) occur without a known family history.
Oxidative stress: Damage caused by reactive oxygen species may harm nerve cells.
Protein aggregation: Accumulations of misfolded proteins within nerve cells are considered a possible cause.
Environmental factors: Certain environmental toxins and high-intensity physical activity have been investigated as potential risk factors.

Symptoms

ALS symptoms develop gradually and worsen over time. Common signs include:

Muscle wasting (atrophy), often starting in the hands, arms, or legs
Progressive muscle weakness and paralysis
Muscle twitching (fasciculations) and cramps
Difficulty swallowing (dysphagia) and speech problems (dysarthria)
Breathing difficulties due to weakening of the respiratory muscles
Stiffness and exaggerated reflexes (spasticity)

Cognitive abilities are usually preserved in most patients for a long time, although some individuals may experience cognitive changes as the disease progresses.

Diagnosis

Diagnosing ALS is complex and relies on a combination of examinations:

Neurological examination: Assessment of muscle strength, reflexes, and coordination
Electromyography (EMG): Measurement of the electrical activity in muscles to evaluate motor neuron damage
Nerve conduction study (NCS): Assessment of nerve conductivity
Magnetic resonance imaging (MRI): Imaging used to exclude other conditions
Laboratory tests: Blood and cerebrospinal fluid analysis to rule out other causes
Genetic testing: When familial ALS is suspected

Diagnosis follows the internationally recognized El Escorial criteria.

Treatment

There is currently no cure for ALS. Treatment aims to slow disease progression and improve the quality of life for those affected:

Drug Therapy

Riluzole: The only widely approved medication that modestly slows disease progression by inhibiting glutamate activity.
Edaravone: An antioxidant approved in some countries that is intended to reduce oxidative stress.

Supportive Therapies

Physiotherapy: Maintaining mobility and slowing muscle deterioration
Occupational therapy: Provision of assistive devices and support for daily activities
Speech therapy: Support for swallowing and communication difficulties
Ventilatory support: Non-invasive ventilation (NIV) for respiratory weakness
Nutritional support and PEG tube: For swallowing difficulties and malnutrition
Palliative care: Symptom relief and psychosocial support in later stages of the disease

Prognosis

ALS is a serious disease with a median survival time of two to five years following diagnosis. However, some patients live significantly longer. The course of the disease varies greatly between individuals. Intensive research worldwide is focused on developing new therapeutic approaches.

References

World Health Organization (WHO): Neurological Disorders – Public Health Challenges. Geneva, 2006.
Hardiman O et al.: Amyotrophic lateral sclerosis. Nature Reviews Disease Primers, 2017; 3: 17071.
Miller RG et al.: Practice parameter update: The care of the patient with amyotrophic lateral sclerosis. Neurology, 2009; 73(15): 1218–1226.

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