![]() Anatomical isolation, a preponderance of upper motor neuron involvement and a younger age of onset confer a better prognosis. ![]() While the classical ALS phenotype constitutes the majority of cases (∼85%), progressive muscular atrophy (lower motor neuron involvement in isolation for > 4 years, ∼10% of cases), primary lateral sclerosis (upper motor neuron involvement in isolation for > 4 years, 1–3% of cases) and progressive bulbar atrophy (bulbar isolation, ∼2–4% of cases) make up the remainder ( Al-Chalabi and Hardiman, 2013, Gordon, 2013). Patients demonstrate variable degrees of upper and lower motor neuron involvement and the site of symptom onset can be classified as spinal or bulbar. Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disease of the upper and lower motor neurons, leading to inexorable motor decline and a median survival of three years from symptom onset ( Al-Chalabi and Hardiman, 2013, Baumer et al., 2014).
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