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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