Motor Neuron(e) Disease

Thursday 30 June 2011: 4 years 9 months on …

Since it was reported that ex-Springbok rugby player, Joost van der Westhuizen, has been diagnosed with “probably” motor neurone disease, I have received numerous queries regarding the illness.

Here are some interesting points gleaned from WIKIPEDIA: 

“The motor neurone diseases (or motor neuron diseases) (MND) are a group of neurological disorders that selectively affect motor neurones, the cells that control voluntary muscle activity including speaking, walking, breathing, swallowing and general movement of the body.

Terminology

MND refers to a group of diseases that affect motor neurones. In the United States, MND is more commonly called amyotrophic lateral sclerosis (ALS), or Lou Gehrig‘s disease, after the baseball player.

Signs and symptoms

Symptoms usually present themselves between the ages of 50-70, and include progressive weakness, muscle wasting, and muscle fasciculations, spasticity or stiffness in the arms and legs, and overactive tendon reflexes. Patients may present with symptoms as diverse as a dragging foot, unilateral muscle wasting in the hands, or slurred speech.

Diagnosis

The diagnosis of MND is a clinical one, established by a neurologist on the basis of history and neurological examination. There is no diagnostic test for MND. Although an individual’s progression may sometimes “plateau”, it will not improve.

Treatment

Currently there is no cure for ALS.

The lack of effective medications to slow the progression of ALS does not mean that patients with ALS cannot be medically cared for. Instead, treatment of patients with ALS 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.

Prognosis

Most cases of MND progress quite quickly, with noticeable decline occurring over the course of months.

MND is typically fatal within 2–5 years. Around 50% die within 14 months of diagnosis. The remaining 50% will not necessarily die within the next 14 months as the distribution is significantly skewed.

Professor Stephen Hawking is a well-known example of a person with MND, and has lived for nearly 50 years with the disease. (Morris Schwartz, of “Tuesdays with Morrie” fame,  also had MND (ALS).)

Mortality normally results when control of the diaphragm is impaired and the ability to breathe is lost.”