By Dr Ananya Mandal, MD
Motor neurone disease is a progressive neurodegenerative disorder that leads to decreased control of muscle movement and eventually paralysis. Symptoms can be mild at first and a tendency to drop things is often one of the first signs of the condition to develop.
Symptoms usually progress over weeks or months after onset and eventually lead to a complete inability to perform even simple tasks that involve muscle activity such as walking, swallowing, breathing or speaking.
In most cases of motor neurone disease, the condition progresses rapidly and the sufferer undergoes an obvious and visible deterioration in a relatively short space of time.
Symptoms usually begin in one region of the body. In some forms of the condition, the hands and feet may be the first regions affected, leading to difficulty picking things up or a tendency to trip, while in others individuals, it may be the muscles involved in talking and chewing that are initially effected.
At first, symptoms may be restricted to one side of the body, but over time the symptoms spread and become more debilitating. As the disease progresses, there is an overall decline in the control of muscle movement that can eventually lead to complete immobility and paralysis. Eventually, the sufferer becomes unable to move and carry out most of the activities of daily living that including taking care of themselves such as maintaining their hygiene, feeding themselves or moving about unassisted.
Motor neurone disease severely shortens life span in most people who develop it. In about 50% of cases, affected individuals will only live for 3 to 4 years after symptom onset, although some people manage to survive for up to 10 years and occasionally even longer. Some estimates suggest that around 1 in 5 patients survive for 5 years after diagnosis and 1 in 10 survive for 10 years after diagnosis. One of the most well known survivors who has lived with this disease for more than four decades is Professor Stephen Hawking.
In the end stages of motor neurone disease, a person usually experiences paralysis and severe breathing difficulty. Breathing can become particularly difficult at night when lying down and as the disease progresses, a breathing mask may be advised for use at night to enhance sleep quality and decrease day time drowsiness. Eventually breathing assistance can no longer make up for the loss of lung function, which often leads to death in these individuals.
Reviewed by Sally Robertson, BSc
Last Updated: Mar 25, 2014