Calprotectin as an immune-dysregulation biomarker in amyotrophic lateral sclerosis: Insights for diagnosis and therapy

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease primarily affecting the motor system, although extra-motor symptoms are also becoming more widely acknowledged. The progressive loss of upper and lower motor neurons at the spinal or bulbar level is a hallmark of ALS, a fatal motor neuron disease [1]. ALS is also referred to as motor neuron disease (MND) and Charcot disease, after Jean-Martin Charcot, the first neurologist to describe the illness.

There are two types of ALS. The most prevalent type, sporadic (90–95%), lacks a clear genetic component. Familial ALS (FALS) accounts for 5–10% of cases and is caused by a genetically dominant inheritance factor [2], [3], [4], [5]. Symptoms typically appear between 50 and 65 years of age [6], [7], [8], [9]. Muscle weakness, twitching, and cramping are the most prevalent symptoms of both forms of ALS, and they can ultimately result in muscle impairment [10], [11]. Dyspnea and dysphagia are symptoms that patients experience in the most advanced stages [12], [13]. ALS frequently begins in a focal area of the brain and then spreads to other parts of the body, where respiratory muscle failure usually limits survival to two to five years after the disease first manifests.

Up to 10% of patients with ALS survive for ten years or longer after the onset of symptoms, although survival rates can vary. The prevalence of ALS is 5.4 per 100,000, while the incidence is 2 per 100,000. In the US, it is thought to affect approximately 18,000 people. Men are 20% more likely than women to have this ailment, and individuals with certain genotypes, bulbar site of origin, cognitive impairment, and older age at onset experience a faster rate of disease progression [14].

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