Happy National Recovering From Brain Surgery Day!

It's a tricky in-between sort of day today, sandwiched between Pancake Day (yay!) and Valentines Day (yay!). Technically it's Ash Wednesday, the first day of Lent, but that just means you can't do anything fun. And I can't do anything fun anyway (that is actually a lie, I have watched a lot of TV and read a lot of awesome books while recovering). So, I decided: I have co-opted February 13th to be National Recovering From Brain Surgery Day! Hurrah!

Are you recovering from brain surgery? No? Then you should find someone who is and shower them in affection and expensive gifts. Then you should probably leave them for a couple of hours so they can have a nap. But then you can come back and give them more gifts! Because that is the meaning of National Recovering From Brain Surgery Day. Hurrah!

This adorable hedgehog is wearing a hat to celebrate February 13th, as science has proven that people recovering from brain surgery enjoy pictures of cute animals wearing hats.

If you are recovering from brain surgery, then celebrate National Recovering From Brain Surgery Day by demanding attention and love from all those around you. But don't strain yourself. Hey, maybe you should take a nap. Hurrah!

Now for the awareness part: Brain tumours are more common than you might think, especially amongst younger people. Brain and central nervous system tumours are the second most common group of cancers in children in the UK, and they kill more children  and people under the age of 40 than any other type of cancer. Yet brain tumour research receives comparatively little funding; just 1.4% of government spend on cancer research related to brain tumour research in 2011, although the proportion spent on brain tumour research has been rising since 2002.

So! Today be nice to someone who's had brain surgery (whether it was for a tumour or epilepsy or head trauma, or just out of sheer curiosity). If you're feeling generous you could even make a donation to a charity like The Brain Tumour Charity, or get involved with fundraising. But one of the most important things you can do for yourself and your family is just to be aware of the possible symptoms of brain tumours.

Have a lovely February 13th!

Sick Note

It turns out that the procedures for getting signed off sick from work in the UK have changed since the last time I had pituitary surgery. I didn't know this, and nor did most of the doctors, nurses, surgeons and assorted healthcare workers at the hospital, but apparently that these days the hospital can only sign you off sick for the period you were actually in the hospital. Your GP has to certify you for the rest of the time. Seems a bit random given that the consultants at the hospital probably have a much better idea of how much time off you need, but nevermind.

Anyway, this has resulted in me receiving from my surgeon a little certificate entitling me to a grand total of three days off work. Reason given: brain surgery.

Growth Hormone and Creutzfeldt-Jakob Disease

It's National Pituitary Awareness Month, and I thought I should look for an interesting pituitary-related story to tell you all. As it turned out, I didn't have to look too far.

 Most people living in Britain today will remember the 1996 scare about "mad cow disease" or BSE (in cows the disease is called Bovine Spongiform Encephalopathy; when it's passed to humans it's variant Creutzfeldt-Jakob Disease or vCJD). It’s an extremely nasty degenerative brain disease, invariably fatal, and there was huge concern that beef contaminated with the disease had been in the food chain for some time. The illness can have a latency period of up to ten years before symptoms appear (or much, much longer according to some researchers), making it very difficult to trace the cause of the illness - and meaning that no-one has any definite idea how many people could have been infected. As of October 2009, there had been 166 identified cases of the illness in the UK.

Beware! This could be a mad cow.

It was a huge scandal in the UK, and I remember as a child being disappointed that I wasn't allowed to eat roast beef for what felt like a very long time - although admittedly this was less because I loved roast beef and more because I loved the accompanying Yorkshire pudding my mum served with it. But until recently I was not aware of a similar, albeit smaller-scale scandal that had occurred several years earlier.

Between 1963 and 1985, the US Government funded a programme which provided human growth hormone to children across the US who had failed to grow as expected. Failure to grow in children is sometimes due to a deficiency in growth hormone (surprise!) and this is still a treatment for children today; the difference is that these days it's made in a lab, while at that time it was extracted directly from the pituitary glands of human cadavers.

In 1985, it came to light that three of the people treated with human growth hormone (hGH) had gone on to die of Creutzfeldt-Jakob Disease. This is not the same illness as vCJD but it is similar - and it's very, very rare. The programme was stopped immediately and an investigation launched.

To date, 29 of the people treated with hGH in the US before 1977 have been diagnosed with CJD. That's about one in 95. The rates were much higher in some other countries; in the UK, which produced its own hGH, 64 of the 1849 people treated developed CJD; and in France, which also produced its own hGH, 119 out of 1700 patients went on to develop the disease. There have been cases reported in numerous other countries; the variation in incidence is likely due to the variation in the way the hormone was extracted and processed.

 The longest latency period recorded between someone receiving human Growth Hormone and going on to develop CJD is 38 years. The shortest period before developing symptoms with these kind of diseases is usually around 2 - 3 years. The symptoms progress very quickly, within just a few months, from dizziness, difficulty balancing and clumsiness to memory loss, seizures and death.

Most disturbingly of all, however, it later came to light that far more of the patients who had been treated with hGH went on to die of adrenal crisis - an entirely treatable problem - than of CJD. This problem isn't caused by the hGH treatment, it's simply the case that people with a growth hormone deficiency are more likely to also be deficient in other pituitary hormones, such as ACTH. Without sufficient ACTH, you will die - but safe and effective hormone replacement is available for people whose bodies don't produce enough ACTH. It's simply that their doctors failed to pick up on the fact that these people were ACTH-deficient until it was too late.

IMFW: Harvey Cushing

This week's IMFW is really more like an IMPW - an Interesting Medical Person of the Week. Harvey Williams Cushing is often regarded as the "father of modern neurosurgery".

An American descended from a long line of doctors, he was born in Ohio in 1869. After studying medicine, he went on to study surgery at the famous Johns Hopkins Hospital just after it had opened, then spent time in Europe before returning to Johns Hopkins as an associate professor in surgery. As well as writing about medicine, he was a talented medical illustrator and several of his drawings were published in textbooks - and as if that wasn't enough, he received the Pulitzer Prize for Biography for his biography of his mentor, William Osler.

Cushing's first monograph was on the subject of the pituitary, and on this blog, you'll have heard Cushing's name from one of his most famous discoveries, the disease named after him - Cushing's Disease; a tumour of the pituitary gland which secretes ACTH (adrenocorticotrophic hormone) and causes a range of symptoms including weight gain, bruising, sweating, high blood pressure and diabetes. Being a modest chap, he had originally named it "polyglandular syndrome", but his name stuck. However, this was far from his only contribution to medicine:

- He introduced blood pressure measurement to America

- He used x-rays to diagnose brain tumours

- He developed medical instruments which are still in use today, including the Cushing Forcep which is used during cranial surgery, the Cushing clip - a small clip for blood vessels to stop bleeding during surgery, which dramatically decreased mortality rates - and the use of electrocautery which he developed with W.T. Bovie, a physicist

- Along with a colleague, Ernest Codman, Cushing devised the first anaesthetic chart to help surgeons and anaesthetists monitor pulse, temperature and breathing - an innovation which was widely adoped

- Cushing pioneered many new surgical techniques including the use of saline for irrigation during surgery

- He developed a surgical cure for trigeminal neuralgia

- His mortality rate was around 10% - which doesn't sound hugely impressive until you realise that before he came along, mortality rates from neurosurgery were 50 - 90% depending on which source you believe

- He developed the transsphenoidal approach for surgery of the pituitary gland

- He invented the macarena (just checking you're paying attention...)

- He identified and named the phenomena of hypopituitarism and hyperpituitarism


Yes... yes, that is a skull.

 So Mr Harvey Williams Cushing deserves our gratitude and respect. He has thoroughly earned the bewildering array of unpleasant medical things named after him, which include: Cushing's Syndrome, Cushing's Disease, Cushing's symphalangism (a.k.a. proximal symphalangism), Cushing's Reflex resulting in Cushing's Triad (a nervous system response to increase intracranial pressure), Cushing ulcer a.k.a. Rokitansky-Cushing Syndrome, Bailey-Cushing Syndrome (he had to share that one), the Cushing forcep and Cushing clip.

Film of transphennoidal endoscopic surgery to remove a TSHoma

So while I don't wish to make everyone feel as nauseated as these injections sometimes make me, I found a film of transphennoidal endoscopic surgery to remove a TSHoma! How exciting! This is the type of surgery I will be having soon, probably around November time. The person whose head this was filmed inside is one of my fellow TSH-producing pituitary tumour brethren.

Don't be fooled, it doesn't actually just take six minutes. There's a whole lot of drilling down your nose required first.

Looks pretty simple to me. Well, I mean, it's not rocket science, is it?

The above is a) more suitable for an audience of a delicate disposition and b) one of my favourite Mitchell & Webb moments!

IMFW: The (Un)Lucky Yasser Lopez

Incredible news recently of an American teenager who was shot straight through the head by a speargun and survived. After a three hour operation to remove the three foot long harpoon from his brain, he has been up and talking. The 16-year old was shot when his friend was loading a spear gun and accidentally fired it in the process. In an incredible stroke of luck, it missed almost all the major blood vessels in his head and pierced only the right side of his brain, meaning that his speech was unaffected.

Yasser Lopez was conscious and speaking when he arrived at the ER -a good sign, although he later had to be sedated; doctors also had to cut off part of the protruding spear before he could fit into the hospital's CT scanner (the scan images are incredible, by the way - I highly recommend following one of the links to take a look).

The story is reminiscent of the famous tale of Phineas Gage, much beloved of neurologists - and it should be remembered that even when people survive such accidents, they are highly likely to be left with some form of impairment, and follow-ups are critical.

IMFW: Autism Research Defrosted

In a previous post about brain injuries in sport, I wrote about the Centre for Traumatic Encephalopathy, which collects and studies brain tissue from athletes involved in high-contact sports. Recently another "brain bank" has been in the news; the Harvard Brain Tissue Research Center at McLean Hospital, where a freezer malfunction damaged one-third of the world's largest collection of brain tissue donated for autism research.

150 brains were prematurely thawed as a result of the broken freezer, with alarm systems and daily inspections failing to alert staff to the malfunction until it was too late. Some of the tissue may still be useable for genetic research, but it's considered to be a huge setback in the field of autism research generally. Postmortem research has previously led to breakthroughs in the study of Huntingdon's disease and Parkinsons.

The loss of the potential research opportunities afforded by the collection is devastating not only to the medical community, but to the families of those whose brains were donated for research. The hospital is conducting two investigations into the fault, with a third carried out by a charity, Autism Speaks.

IMFW: Brain Injuries in Sport

An interesting story that has been popping up now and again for the last few months concerns brain injuries in the US NFL (National [American] Football League), so that's what we're focusing on in this week's Interesting Medical Fact of the Week.

American Football is obviously a high-contact sport, hence the crazy amounts of protective gear the players wear - including helmets. However, evidence has been mounting for some time that all this protective gear may not be enough.

Several high-profile former players have killed themselves in the last few years. Dave Duerson committed suicide in February 2011, after texting his family to request that his brain be donated to the NFL's "brain bank" - namely, the Centre for the Study of Traumatic Encephalopathy, which was set up by former athletes to investigate the long-term effects of the concussions suffered by sportspeople and military personnel.

The Centre focuses on studying chronic traumatic encephalopathy (CTE), which is a progressive, degenerative disease of the brain. As early as the 1920s, there was recognition that this condition particularly affected boxers, but it now appears that it is also associated with repeated brain trauma (i.e. concussions or other head injury). Professional American football players, ice hockey players and those involved in sports such as wrestling, are particularly likely to sustain these kinds of injuries.

Chronic traumatic encephalopathy is a nasty illness. Much like Alzheimer's Disease, it involves the build-up of tau proteins in the brain, and the initial symptoms of memory loss, confusion, depression, and self-control problems eventually lead to full-blown progressive dementia with some symptoms of Parkinson's Disease.

When Duerson's brain was studied, researchers found that the repeated trauma to his head during his professional career appeared to have caused severe damage. One of the difficulties with chronic traumatic encephalopathy is that there can be a significant latency period between the original injuries and the appearance of recognisable symptoms; however, along with Duerson, many other former NFL players - and those involved in other high-contact sports - have been diagnosed with chronic traumatic encephalopathy after their deaths. Chris Henry, another American football player who died after falling from a moving vehicle, was the first player to be posthumously diagnosed with CTE while still active as a professional sportsman; but the earliest signs of the disease have even been found in the brain of an 18-year-old student athlete.

As well as Duerson, former professional footballers Junior Seau and Ray Easterling also recently committed suicide. Along with several other American football players, Easterling was involved in a class action lawsuit against the NFL over its handling of concussion-related injuries, which alleges that the NFL continuously denied any knowledge of a connection between NFL players sustaining repeated head injuries and the later development of dementia or CTE. In addition to this, another lawsuit was recently launched on behalf of over 100 former professional players, claiming that the NFL repeatedly denied the existence of any connection.

Chronic traumatic encephalopathy is a very rare disease, and one of the difficulties in researching it is that it can only be definitively diagnosed post mortem. But the work of the Centre for the Study of Traumatic Encephalopathy is already causing waves in the world of American football, and there have already been calls for changes to the game's regulations, effectively banning head contact from the game. The Centre's research is still ongoing, so whether that will actually happen remains to be seen.

But are those helmets enough?

If you're interested in this topic and would like to find out more, I recommend the Centre's very informative website, which also has a selection of case studies you can read about.