AMYOTROPHIC LATERAL SCLEROSIS
About ALS
Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease or Lou Gehrig’s disease is a devastating multisystem neurodegenerative disease. ALS is primarily characterized by degeneration of both upper and lower motor neurons.¹² The condition leads to progressive motor decline, and generally people die from failure of respiratory muscles two to four years after disease onset.¹³
Around 50% of patients with ALS will also suffer with extra-motor manifestations, such as changes in behavior, and problems with language and executive function.¹²
ALS can be separated into familial and sporadic ALS.¹⁴ Sporadic is more prevalent, accounting for around 90% of cases and occurs when only one person in a family has ALS.¹⁴ Familial ALS accounts for 10% of cases and in this instance more than one family member has ALS.¹⁴
The estimated global prevalence of ALS is 4.1–8.4 per 100,000 people.¹⁶ and the average age at onset is 58–63 years for sporadic ALS and 40–60 years for familial ALS, with a peak incidence in those aged 70–79 years.¹⁷
Research shows variations in 40+ genes associated with ALS.¹⁵ Globally, the most common include expansions of chromosome 9 open reading frame 72 (C9orf72) and variants in superoxide dismutase 1 (SOD1), TANK1-binding kinase 1, fused in sarcoma (FUS), and TAR DNA-binding protein 43 (TDP-43).¹⁵
There is currently no cure for ALS, and patient care is focused on symptom management and palliative care.¹⁸ Some disease modifying treatments are available, however these offer limited or unconfirmed benefit.¹⁹
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