Clinical aspects of motor neurone disease

Motor neurone disease (MND), or amyotrophic lateral sclerosis (ALS), is a progressive, fatal neurodegenerative disease affecting upper and lower motor neurones. It eventually causes widespread weakness and wasting, spasticity, dysarthria, dysphonia, dysphagia and respiratory failure. The diagnosis is clinical, and it is important for management to establish this early. It presents with upper and lower motor neurone signs affecting the bulbar, cervicothoracic and lumbosacral regions (ALS), only lower motor neurone signs affecting one or more limbs (progressive muscular atrophy), purely bulbar region symptoms and signs (bulbar onset), or only upper motor neurone signs in the (usually lower) limbs (primary lateral sclerosis).