In the absence of an invitation to appear on Desert Island Discs I sometimes find myself playing a rather macabre, inverted form of the programme, along the lines of ‘Eight medical conditions I never want to have on a desert island’. Depending on my clinical workload at the time these monsters tend to vary from week to week but one does seem to remain at the top of my personal list — motor neurone disease.
The death of Ronnie Corbett last week from this condition will not only have caused genuine sadness in those of us brought up with The Two Ronnies hard-wired into our Saturday nights, but for some people will also have raised an eyebrow as they wondered just what the condition actually is. (By contrast, Ronnie Barker died of heart failure, one of the most common health conditions. Corbett died of one of the rarest.)
Although there are various types of motor neurone disease (MND), the most usual type is known as amyotrophic lateral sclerosis, or ALS, accounting for over 80 per cent of MND. Around two people in every 100,000 in the UK develop it each year, and about 5,000 people currently have ALS-MND in this country.
Corbett was slightly unusual in that it usually develops between the ages of 40 and 70, and although it can affect anyone it is twice as common in men as in women. For the vast majority of cases, it is not inherited, does not run in families and — frustratingly — has no obvious cause.
At its most basic, MND causes motor nerves in the body to slowly stop working and in ALS this usually affects the hands and feet first, with stiffening and weakness of muscles being one of the early symptoms.
Unfortunately in its early stages it can mimic many other possible conditions which can cause a delay in diagnosis (the last patient of mine diagnosed with MND initially complained of having difficulty doing up the buttons of their jacket, and nothing else.) Less commonly, initial symptoms affect the throat so that swallowing, chewing and talking can be affected.
There is currently no specific test available to confirm the diagnosis — which is made on clinical grounds, often by a neurology specialist — and no cure. Symptoms progress steadily over time with more and more muscle groups becoming affected so that walking, moving, swallowing and breathing become more difficult but intellect does not usually change. This can be an especially cruel part of the disease as the sufferer remains aware of everything that is happening to them, often right to the end.
The rate at which the disease progresses varies from person to person, but the majority of people with ALS-MND die within three to five years of their symptoms first appearing. About 20 per cent survive five years, and one in 10 survives 10 years or more.
The exception to the rule here is Stephen Hawking, who continues to survive over 50 years after diagnosis despite now only being able to move one eye. Life expectancy turns on two things with MND — the nerves running the breathing muscles, and those affecting the muscles of swallowing — and he appears to be a very rare example of these continuing to function long after MND would normally have shut them down.
Although treatments to help ease symptoms are available, a cure is not and so hope remains focussed on research that is looking to unlock the key as to what causes MND. Find that key, and treatments can develop from there. Until then, it remains an enemy at the gate of modern medicine, and gives no respect to fame or celebrity as Corbett, David Niven and Chairman Mao to name but a few have found out to their cost.