Tuesday 1 February 2011: 4 years 5 months on …

There are a number of neuro-degenerative diseases. Most people have never heard of them or have needed to pay much attention to them.

At the end of 1999, shortly after returning from a two-week study tour to the University of Disney World in Orlando, Florida, I had undergone surgery to rectify a herniated disc in my neck. A piece of my right hip bone was used to fuse two vertebrae in my neck!

In September 2006, just before my 50th birthday, I became aware that my left hand and arm were not functioning properly. As these were similar symptoms to those that had appeared previously, I thought that it was merely a recurrence of the problems of 1999. However, that was not to be, and it was after six months of medical tests that I was advised, on Thursday 8 February 2007 that I, most probably, had, what was referred to as an extra-pyramidal Parkinsonian disorder called corticalbasal degeneration. I was informed that I would become severely incapacitated within three years, BUT I was also told that there was a possibility that after five years I would possibly be able to return to sue the specialist for having made an incorrect diagnosis.

I have subsequently searched the internet and read many articles on CBD and various other neurological (and other) disorders. Most people have heard of Parkinson ’s disease (PD), but even then, more often than not, joke about the condition as the one where people rattle and roll and shake tea-cups and drop their cutlery. In fact, there are many differing types of PD, and no two people may have exactly the same symptoms. Some people with PD do not even have the shakes!

There are numerous neurological illnesses, diseases or syndromes or whatever you want to call the affliction. In my reading, I have encountered many, such as corticalbasal degeneration (CBD) – sometimes referred to as corticobasal degeneration, or corticalbasal ganglionic degeneration (CBGD). Then there is Amyotrophic Lateral Sclerosis (ALS) – in the USA often referred to as Lou Gehrig Disease, Motor-neurone disease (MND), Progressive Supranuclear Palsy (PSP), etc etc. (Often Muscular Dystrophy [MD] is also thought of as a neurological illness, but is, in fact, a hereditary muscle disease that leads to paralysis.)

It would appear that even two people diagnosed with the same illness do not necessarily experience all the same symptoms.

Two well known people with the same diagnosis: Morrie Swartz – of  the book / movie / stageshow “Tuesday’s with Morrie” fame had ALS, diagnosed in August 1994 and given two years – he died slightly more than one year later. Professor Stephen Hawking was diagnosed with ALS at the age of 21 in 1963 and given 2 – 3 years to live. He is still alive today at the age of 69 – completely paralyzed and speechless.

There are a whole lot of boxes each with a different label depicting a different condition. The problem is that it is extremely difficult to put a specific condition into a specific box. The reason being that these conditions all seem to overlap and symptoms from one condition or one patient may or may not appear in another condition or in another patient. And all they have to go on are the symptoms that they are able to observe.

To complicate matters more, it is almost impossible to conclusively put a condition into a specific box (or give it a specific label) unless one delves into the deep recesses of the human brain. And that, of course, is impossible until the person has died and the medical fundi’s are then able to scratch around in the brain and decipher just what went wrong! In more than 50% of cases of neurological disorders, it has been shown in death that the diagnosis in life has been incorrect! 

I say it’s like taking your car to the garage – telling them that there’s a problem in the engine and asking them to please tell you what it is, and to fix it, but not allowing them to open the bonnet of the car to check on anything. The most they can do is to listen to the sounds and watch the problems! Now that’s quite a tall order!

 There also appears to be quite a bit of confusion between the various disorders and their causes, so recently on the radio I attempted to explain it as best I could in the following manner:

Most people are familiar with an electrical circuit: there is the battery that provides the current that runs through the electrical wire and that illuminates the electric bulb in the circuit. In order for the bulb to light up, all the parts in the circuit must be in working order.

If any one part does not work, the bulb does not light up. If the battery is flat, or the bulb is fused or the wire is severed, short-circuited or not connected properly, then the bulb will not work!

If one compares this simple illustration to the human body, then the battery is the brain, the wire flex, the nerves and the bulb the muscles. In order for the muscles to work, the whole circuit must be in working order.

In the case of my CBD, the problem lies not with the muscles (the light bulb) or with the nerves (the flex), but with the battery itself (the brain). The brain cells that manufacture dopamine (a chemical required to convey the messages (the current) from the brain to the muscles have malfunctioned and no-one knows why. So the dopamine goes into short supply, the battery goes flat, it can’t be replaced or recharged, and, therefore,  the messages from the brain do not get to the muscles and they stop working! In fact, the muscles start contracting and that’s why my limbs, fingers, toes and shoulders have started curling inwards.

With other neurological illnesses, the problems lie with other brain cells, the muscles themselves or with the nerves, also resulting in paralysis, but resulting from a different cause.

Unfortunately, my battery this week appears to have gone into an ESKOM load-shedding mode, and I have not been able to finish off this blog. Instead, I have spent time with the medical professionals and popped some more pills!



Sunday 6 February 2011 …

 Circuit Breaker (continued) …

In MS (Multiple Sclerosis), for example, the problem lies with the flex – the nerves themselves. Nerve cells communicate by sending electrical signals down the flex – long fibres called axons which are wrapped in an insulating substance called myelin. In MS, the body’s own immune system attacks and damages the myelin. When myelin is lost, the axons can no longer effectively conduct signals, in the same way as when the plastic covering the elctrical wire in the flex is damaged and a short circuit is created. The earth leakage blows and the bulb does not glow. In the human body, various neurological symptoms appear, including physical and cognitive disabilities.

In the case of muscular dystrophy (MD), the paralysis is caused by hereditary muscle diseases that weaken the muscles themselves. MD is charcterised by progressive skeletal muscle weakness, defects in muscle proteins and the death of muscle cells and tissue.  

Motor neurone diseases is the term sometimes used to cover, almost as a group, all neurological illnesses that SELECTIVELY affect motor neurones, the brain cells that control voluntary muscle activity including speaking, walking, breathing, swallowing and general movement of the body. I have highlighted the word SELECTIVELY because it is obvious (from examples above and from case studies that I have read) that it is highly unlikely and unusual for any two people, even those diagnosed with the same disease, to have the same symptons.

What is frightening though, is the number of people who have these diseases and yet how little is still known about cause, treatment and prognosis. Unfortunately, I have not been able to source figures for South Africa, but the following are estimates for the USA (which has a population of some 450 million people – 10 times more than RSA):

  • Total number of people with a form of degenerative neurological condition – more than 6 million
  • Parkinson Disease – 1,5 million
  • Alzheimer’s Disease – 4,5 million
  • PSP – 20 000
  • MS – 400 000
  • ALS – 30 000
  • CBD – 10 000

 It would be interesting to research just how much money is spent globally on researching these illnesses. When I attempted to raise money for research work into CBD in South Africa, I was told that no such research work was being done in this country. Much research is done on the brains of those people who live and die with neurological illnesses and donate their brains to medical science.

Last week, I saw the medical people.

This week I have to see the legal people.

I won’t be leaving much material wealth behind. Anyone want my brain?

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