Motor Neurone Disease

What is Motor Neurone Disease

Motor neurone disease (MND) is the name given to a group of related diseases in which there is progressive degeneration of the motor neurones in the brain and spinal cord. Motor neurones are the nerve cells that control muscles, and their degeneration therefore leads to weakness and wasting of the muscles.

This wasting generally occurs in the arms and legs initially, with some groups of muscles being affected more than others. Some patients with a particular type of MND may develop weakness and wasting in the muscles supplying the face and throat.

This can lead to problems with speech, chewing and swallowing.

What are the Symptoms of Motor Neurone Disease

MND can present itself in a number of ways, depending on the particular group of muscles which are initially affected. Initial symptoms can include wasting and weakness of muscles of the hands or weakness and stiffness in the legs with a dragging of one leg.

What are the Causes of Motor Neurone Disease

About 90% of cases of MND are "sporadic", meaning that the patient has no family history of ALS and the case appears to have occurred with no known cause. Genetic factors are suspected to be important in determining an individual's susceptibility to disease, and there is some weak evidence to suggest that onset can be "triggered" by as yet unknown environmental factors.

Approximately 10% of cases are "familial MND", defined either by a family history of MND or by testing positive for a known genetic mutation associated with the disease. The following genes are known to be linked to ALS: Cu/Zn superoxide dismutase SOD1, ALS2, NEFH (a small number of cases), senataxin (SETX) and vesicle associated protein B (VAPB).

Traditional Medical Treatments for Motor Neurone Disease

Currently there is no cure for MND. The only drug that affects the course of the disease is riluzole. The drug functions by blocking the effects of the neurotransmitter glutamate, and is thought to extend the lifespan of an MND patient by only a few months.

The lack of effective medications to slow the progression of MND does not mean that patients with ALS cannot be medically cared for. Instead, treatment of patients with MND focuses on the relief of symptoms associated with the disease. This involves a variety of health professionals including neurologists, speech-language pathologists, physical therapists, occupational therapists, dieticians, respiratory therapists, social workers, palliative care specialists, specialist nurses and psychologists. A list of neurology clinics that specialize in the care of patients with ALS can be found on the World Federation of Neurology website www.wfnals.org .