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Amyotrophic Lateral Sclerosis (ALS) – Symptoms and Treatment

Amyotrophic lateral sclerosis (ALS) is a severe progressive neurological disease in which motor nerve cells gradually degenerate and die, leading to increasing muscle weakness and paralysis.

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Things worth knowing about "Amyotrophic Lateral Sclerosis"

Amyotrophic lateral sclerosis (ALS) is a severe progressive neurological disease in which motor nerve cells gradually degenerate and die, leading to increasing muscle weakness and paralysis.

What is Amyotrophic Lateral Sclerosis?

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive and ultimately fatal neurological disease. It affects the motor neurons -- the nerve cells in the brain and spinal cord responsible for controlling voluntary muscles. As these neurons degenerate and die, the brain loses its ability to initiate and control muscle movement, resulting in progressive paralysis, muscle wasting, and eventually respiratory failure. ALS currently has no cure and most cases follow a rapidly progressive course.

Causes

The exact causes of ALS remain incompletely understood. Two main forms are recognized:

Sporadic ALS: Accounts for approximately 90-95% of all cases with no clear family history. Potential risk factors include intense physical activity, exposure to environmental toxins, and prior head injuries.
Familial ALS: Approximately 5-10% of cases are inherited, with mutations in genes such as SOD1, C9orf72, FUS, and TARDBP being most commonly implicated.

Proposed disease mechanisms include oxidative stress, excitotoxicity caused by excess glutamate, mitochondrial dysfunction, and impaired RNA and protein processing within motor neurons.

Symptoms

Symptoms of ALS develop gradually and depend on which motor neurons are first affected:

Muscle weakness and atrophy: Often begins in the hands, arms, or legs with progressive loss of strength.
Fasciculations: Involuntary, visible muscle twitching beneath the skin.
Spasticity: Increased muscle tone and stiffness due to damage to upper motor neurons.
Dysarthria and dysphagia: Difficulties with speech and swallowing when bulbar muscles are affected.
Respiratory failure: Progressive weakness of the breathing muscles, which becomes life-threatening over time.

In most patients, cognitive function, sensory perception, and bladder and bowel control are preserved until the later stages of the disease.

Diagnosis

ALS is a clinical diagnosis requiring the exclusion of other conditions. Key diagnostic investigations include:

Neurological examination: Detection of combined upper and lower motor neuron signs.
Electromyography (EMG): Identifies characteristic patterns of denervation in affected muscles.
Nerve conduction studies (NCS): Used to rule out other neuromuscular conditions.
MRI of the brain and spinal cord: Excludes structural causes of symptoms.
Blood tests and lumbar puncture: Rule out inflammatory or metabolic disorders.
Genetic testing: Recommended when family history or clinical findings suggest a hereditary form.

The diagnostic reference framework is defined by the El Escorial criteria and the updated Gold Coast criteria of the World Federation of Neurology.

Treatment

There is currently no cure for ALS. Treatment focuses on slowing disease progression and maintaining quality of life through a multidisciplinary approach.

Drug Therapy

Riluzole: The long-established disease-modifying drug for ALS. It reduces glutamate release and extends survival by an average of several months.
Edaravone: An antioxidant approved in several countries (including the USA and Japan) that may slow functional decline in selected patient groups.
Tofersen: An antisense oligonucleotide targeting the SOD1 gene mutation, approved for patients with SOD1-related familial ALS.

Symptomatic and Supportive Care

Physiotherapy: Maintains mobility and muscle function for as long as possible.
Occupational therapy: Adapts daily activities and provides assistive devices.
Speech and language therapy: Addresses difficulties with speaking and swallowing.
Nutritional support: When swallowing becomes severely impaired, a percutaneous endoscopic gastrostomy (PEG) tube may be required.
Respiratory support: Non-invasive ventilation (NIV) is used to assist breathing as respiratory muscles weaken.
Palliative care: Pain management, psychosocial support, and end-of-life planning are integral components of ALS care.

Prognosis

The median survival after diagnosis is approximately 2 to 5 years, though around 10% of patients survive significantly longer. Prognosis is influenced by age at onset, disease subtype, and whether the bulbar muscles are involved early. Care at specialized multidisciplinary ALS clinics has been shown to improve both quality of life and survival.

References

Hardiman O, Al-Chalabi A, Chio A et al. - Amyotrophic lateral sclerosis. Nature Reviews Disease Primers. 2017;3:17071. DOI: 10.1038/nrdp.2017.71
Miller RG, Mitchell JD, Moore DH - Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). Cochrane Database of Systematic Reviews. 2012;3:CD001447.
van den Berg LH, Sorenson E, Gronseth G et al. - Revised Airlie House consensus guidelines for design and implementation of ALS clinical trials. Neurology. 2019;92(14):e1610-e1623.

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