Motor Neurone Disease

Page 1 2 3

What is Motor Neurone Disease?

In motor neuron disease (MND) there is relentless and unexplained destruction of lower and upper motor nerve fibres in the brain and spinal cord, leading to progressive weakness of muscles.

Who gets Motor Neurone Disease?

MND affects 4-6 people out of every 100 000. MND usually presents in middle age and is slightly more common in men.

Predisposing Factors

The cause of MND is unknown. In 5-10% of cases there is a family history, but this is often not the case.

Progression

MND has a gradual onset. 70% of patients present with progressive and symmetric limb weakness. This often begins as a foot drop, although deficits in fine hand movements may be noticed first. Localised pain may precede the onset of muscle weakness and wasting by several weeks; this may be severe in late stages of the disease.

The natural history of MND is progressive muscle weakness culminating in death usually from respiratory failure. Patients who are artificially ventilated, fed via gastrostomy and receiving full nursing care may have their life prolonged for a several months albeit in a totally dependent condition.

Page 1 2 3