Myotonia congenita is a genetic disorder that affects the muscles used for movement. Myotonia refers to the delayed relaxation of these skeletal muscles, resulting in a variety of obvious symptoms including temporary paralysis, stiffness or transient weakness.
In humans these symptoms are often manifest as difficulty in swallowing, gagging and frequent falls. Children are affected more than adults. One of the most dramatic manifestations are the falls (‘fainting’) that can occur as a result of a hasty movement.
Although physiologically distinct, ‘fainting’ is a reasonably accurate description of the sudden loss of movement and the transient nature of the disorder. Like fainting, loss of movement is usually quickly resolved. However, unlike fainting, myotonia congenita involves muscular rigidity or stiffness, so more closely resembles catalepsy.
There are two types of myotonia congenita, termed Thomsen disease and Becker disease, both of which are usually associated with mutations in the gene CLCN1 1. This encodes a chloride channel (a ‘hole’ through the cell membrane that allows the transfer of chloride ions) critical for muscle fibre activity.
With loss-of-function mutations in CLNC1 the muscle fibre continues to to be activated. When stimulated, for example if the fibre is triggered to suddenly contract for jumping or running (or to stop a fall), the muscle fibre is hyper-excitable and continues to contract, and shows delayed relaxation.
Around 1 in 100,000 people exhibit myotonia congenita, though it is about ten times more common in northern Scandinavia. Treatment involves use of a number of anticonvulsant drugs.
The same loss-of-function CLCN1 mutation in humans is seen in symptomatically similar horses, dogs … and goats.
In the late 19th century four goats were imported to Marshall County, Tennessee. Their strange behaviour when startled was first described in 1904 and defined as a congenital myotonia by Brown and Harvey in 1939.
These pre-war studies formed the basis of of our understanding of both the physiology and genetics of myotonia congenita, though the specific mutation in the CLCN1 gene was only confirmed several years after it had been identified in humans.
Since then myotonic goats have become an internet staple, with any number of slightly distressing (for me at least, if not for the goats) YouTube videos showing their characteristic fainting when surprised or frightened 2.
Don’t bother watching them.
If you want to see a fainting goat in action watch little ‘Ricky’ jump up onto a swinging seat on the National Geographic website.
It’s a perfect example.
He jumps up, gets a mild fright as the swing moves, goes stiff legged and simply rolls over and falls to the ground. A few moments later he’s back on his feet again, looking slightly shaken perhaps, but none the worse for wear.
All of that preamble was to introduce the topic of fainting queens.
This was a subject I’d heard about, but had no experience of until last week.
Periodically it gets discussed on Beesource or the Beekeepingforum – usually the topic is raised by a relatively small-time amateur beekeeper (like me) and it gets a little airtime before someone like Michael Palmer, Michael Bush, Hivemaker or Into the Lion’s Den 3 shuts down the conversation with a polite “Yes, I see it a few times a year. They recover”, or words to that effect.
Since these commercial guys handle hundreds or perhaps thousands of queens a year I think we can safely assume it’s a relatively rare phenomenon.
Since I don’t handle hundreds or thousands of queens a year – and you probably don’t either – I thought the incident was worth recounting, so you know what to expect should it ever happen.
And to do that I have to first explain the fun I had with the first of the two queens in the hive I was inspecting.
A two queen colony
It was late afternoon and I was inspecting the last of our research colonies in the bee shed.
The hive had two brood boxes and a couple of supers. Nothing particularly surprising in that setup at this time of the season; the colony was quite strong, the spring honey had been extracted and a couple of supers had been returned to the hive for cleaning.
However, it wasn’t quite that straightforward.
The lower brood box had been requeened ~3 weeks earlier with a mature queen cell from one of my queen rearing attempts. I’d seen that the virgin had emerged and restricted her to the lower box at my last visit.
I’d added a queen excluder (QE) over the lower box with the intention of removing all the old frames above the QE once the brood had emerged.
However, at that last visit I’d ended up with a good looking 4 ‘spare’ virgin queen. Although I had no need for her at the time, and no time to make up a nuc 5, I decided to put her in a fondant-plugged introduction cage in this upper box.
This ‘upper’ queen couldn’t fly and mate in the week I was away, but I reasoned that I could merge the colony with the bottom box if the ‘lower’ queen failed to mate 6.
So, after adding the virgin queen to the top box I added a second QE and the two supers.
She can fly …
Having removed the supers and the upper QE I carefully inspected the upper box looking for the virgin queen who had been released from the cage
No sign of her 🙁
I went through the box again.
Time to try some of the ‘queen finding tricks’.
I moved three frames out of the way having examined them very carefully. The remaining 8 frames were then spaced out as four, well separated, pairs. I let the colony settle for a few minutes and then looked at the inner face of each pair of frames.
No sign of her 🙁
I looked again … nada, rien, niets, nunda, dim byd and sod it 7.
The obvious conclusion was that the colony had killed the queen after releasing her from cage.
I reassembled the upper brood box and lifted it off the lower QE, in preparation to leave it outside the shed door while I went through the lower box.
As I carried the brood box to the door I briefly looked up and saw a 8 virgin queen climbing up the inner pane of one of the shed windows, flapping frantically and fast approaching the opening that would allow her escape.
For obvious reasons I have no photographs of the next few minutes.
For those unfamiliar with the bee shed windows, these have overlapping outer and inner panes, so are always open. They provide a very effective ‘no moving parts’ solution to clearing the shed of bees very quickly.
Which was the very last thing I wanted at that moment 😉
… rather well
I had a brood box and hive tool in my hands, the shed door was wide open, there was all sorts of stuff littering the floor and the virgin queen was inches away from making a clean getaway.
It’s worth noting that when virgin queens are disturbed and fly they almost always return to the hive. However, the hives in the shed have a single entrance and all the hives were already occupied with queens. I couldn’t let her fly and hope for the best … it probably wouldn’t end well.
By balancing half the brood box on an unoccupied corner of an adjacent hive roof I made a largely ineffective swipe for the queen, but disturbed her enough she flew away from the window in spirals around my head.
I s t r e t c h e d to reach the shed door and pulled it close, so reducing the possible exits from eight to seven. A small victory.
I put the brood box safely on the floor, leaning at an angle against the hive stand 9, and abandoned the hive tool.
The next 5 minutes were spent ineptly trying to catch the queen. When she wasn’t flying around the shed (where the lighting isn’t the best) she usually made for the same window.
The one behind the hive with four supers stacked on top 🙁
After a few more laps of the shed, dancing around the precariously balanced brood box and reaching around the hive tower for the window, I finally caught her.
And caged her 10.
I’m looking for publisher for my latest book, ‘Slapstick beekeeping’. If any readers know of a publisher please ask them to contact me.
After all that I should have had a little rest. I’d had enough excitement for the afternoon 11.
But there was still the queen in the bottom box to find and mark.
The queen in the bottom box was mated and laying well.
I made a near-textbook example of finding her 12.
After moving aside a few frames I should have announced (to the non-existent audience), “She’s on the other side of the next frame … ” (the big reveal) ” … ah ha! There you are my beauty!”.
Holding the frame in one hand I checked my pockets for my marking cage 13.
All present and correct.
I then calmly picked her up by her wings. She was walking towards me, bending slightly as she crossed over another bee, so her wings were pushed up and away from her abdomen.
A perfect ‘handle’.
I didn’t touch her abdomen, thorax or head.
And, as soon as I lifted her from the frame, she fell into a swoon and ‘dropped dead’.
Her wings were extended to the sides, her abdomen was curled round in a foetal position and she appeared completely motionless.
I dropped her into the marking cage and took the photo further up the page.
It was 6:49 pm.
For several minutes there was no obvious movement at all. Her legs and antennae were immobile. She showed no sign of breathing.
I gently shook her out onto a small piece of Correx on a nuc roof to watch and photograph her. I picked her up by the wing and held her in my palm … perhaps she needed some warmth to ‘come round’.
Was that a twitch?
Or was that me shaking slightly because I’d inadvertently killed her?
Several more minutes of complete catatonia 14 passed … and then a gentle abdominal pulsing started.
This was now 10-11 minutes after I’d first picked her up.
Which got a bit stronger and was accompanied by a feeble waggle of the antennae.
And was followed a minute or so later by a bit of uncoordinated leg flexing.
And after 15 minutes she took her first steps.
It looked like she’d been on an ‘all nighter’ and was still rather the worse for wear.
I slipped her into a JzBz queen cage, sealed it with a plastic cap, and left it hanging between a couple of brood frames.
From picking her up to placing the caged queen into the brood box had taken 24 minutes.
I reasoned that if …
- she fully recovered they’d feed her through the cage and I could release her the following morning
- I’d released her immediately and she’d acted abnormally the colony might have killed her off
- she did not recover I would at least be able to find the corpse easily ( 🙁 ) and so could confidently requeen the colony (with the virgin I’d tucked away safely in my pocket)
The following morning the cage was covered in bees and she looked just fine, so I released her.
She walked straight down between the frames as though nothing untoward had happened.
I didn’t have the heart to mark and clip her … I didn’t want to risk her ‘fainting’ again and, if she had, didn’t have the time to hang around while she recovered 15.
So was this ‘fainting’ myotonia congenita?
I suspect not.
Another name for the Tennessee fainting goat is the ‘stiff-legged’ goat. This reflects the characteristic rigidity in the limbs when the muscles fail to relax. The queen’s legs were curled under her, rather than being splayed out rigidly.
However, this interpretation may simply reflect my near complete ignorance of the musculature of honey bees 😉
However, I do know that the basics of muscle contraction and relaxation are essentially the same in invertebrate and vertebrate skeletal muscle. There are differences in the innervation of muscle fibres, but the fundamental role of chloride channels in allowing muscle relaxation is similar.
Therefore, for this fainting queen to be affected by myotonia congenita she should have a mutation in the CLCN1 gene encoding the chloride channel.
Although the honey bee genome has been sequenced a direct homolog for CLCN1 appears not to have been identified, though there are plenty of other chloride channels present 16.
The majority of the 60 or so mapped mutations associated with myotonia congenita (in humans) are recessive. Two copies of the mutated gene (in diploids, like humans or female honey bees) are needed for the phenotype to occur.
Of course, drones are haploid so it should be easier to detect the phenotype.
I’ve never heard of drones ‘fainting’ when beekeepers practise their queen marking skills on them. Have you?
I’ll try to mark and clip this queen again.
It will be interesting to see if she behaves in the same way 17.
A quick scour of the literature (or what passes for the ‘literature’ on weird beekeeping phenomena i.e. the discussion fora) failed to turn up examples of the same queen repeatedly fainting.
Or any mention of daughter queens showing the same behaviour.
All of which circumstantially argues against this being myotonia congenita.
However, there are many other causes of sudden fainting (from the NHS website):
- standing up too quickly – (low blood pressure)
- not eating or drinking enough
- being too hot
- being very upset, angry, or in severe pain
- heart problems
- taking drugs or drinking too much alcohol
… though I can exclude the last one as my bees are teetotal 😉
So, there you have it, a brief account of a cataleptic queen … and her recovery.
A fortnight after the events described above I clipped and marked the queen. I did everything the same – picked her up by the wings in the shed (so again not exposed to bright sunlight – which may be relevant, see the comment by Ann Chilcott).
She (the queen) didn’t faint. She behaved just like the remaining 4 queens I marked on the same afternoon.
So no repeat of the ‘amateur dramatics’ 🙂
- There are rarer forms associated with the mutation of a voltage gated sodium channel (also involved in muscle relaxation), though symptomatically these are similar.
- These usually seem to involve giggling youths chasing the goats around and deliberately scaring them … not my idea of entertainment.
- All large (in the ‘number of hives’ sense) commercial beekeepers.
- Well, to me at least …
- I had a ferry to catch and it was getting late.
- At the very least I’d learn that this wasn’t a good idea if it all went Pete Tong.
- Spanish, French, Dutch, Corsican, Welsh and my feelings about the situation.
- And, in a stroke of Holmesian logic, I presumed the …
- Doing it this way reduces the chances of crushing bees on the bottom of the frames or the lower edges of the box.
- And the following morning made up a nuc with her.
- As an aside, virgin queens can fly very well … they have been documented flying at least 5 miles to drone congregation areas. A few laps of a 16 x 8 shed is no challenge at all.
- Though I say so myself!
- Scissors and Posca pen were nearby, but long and painful experience has taught me not to keep scissors in my beesuit pocket.
- Actually, not typical catatonia, which is characterised by abnormal movements, immobility, behaviours, and withdrawal, though the term catatonic is often used to mean unresponsiveness
- And I’d used the ‘spare’ virgin queen so had no plan B to revert to had things gone wrong.
- It’s also worth noting that determining that the mutation is present would require sequencing the relevant part of the queen’s genome … itself a rather destructive process that induces an irreversible immobility. Or I could wait for a few weeks and sequence some drones from her.
- Interesting for me … I’m not sure she’ll be quite so engrossed with the whole process.
I too have had queens faint and recover during marking. They do not seem prone to fainting again, but then we don’t usually herd them into the marking cup twice either!
I am not a big fan of BeeSource. Too much snark and disinformation. I vastly prefer your blog, David!
You’ll see one comment (below I think) where Malcolm reports ‘repeat offending’. I’ll certainly be on the lookout for it when I catch up with this queen again. It seemed a bit cruel to clip and mark her when she was comatose so I will get a second chance!
Thanks for the vote of confidence in the posts here … the discussion forums go through good and bad patches. It very much depends how well they’re moderated I think. Moderating is a thankless task, but necessary to keep some of the more excessive responses away from the sensitive or inexperienced. There are certain posters who dominate them – very experienced no doubt and probably well meaning – but whose primary goal appears to demonstrate how much more they know that the person posing the question. This, coupled with poor search facilities and even poorer choice of post titles, means the ‘back catalogue’ is often largely ignored and of little use to anyone.
Even Bee-L struggles sometimes and that’s very low volume and generally rather specialised.
The posts here are also moderated … the very few (thankfully) that I receive that are unsuitable for posting never make the cut.
Excellent post I think I saw that once and thought I killed her
Yes … it’s an unnerving experience the first time. Next time I’ll be better prepared.
Thanks as ever David. I must remember to commend you to the BBC news script writing team as a scribe for their very tenuous links between stories… 😉
Thank Neil … and for the coffee 🙂
My goodness if you think this was tenuous you should see some of the things I’ve got lurking in the ‘drafts’ folder 😉
The same experience here about 4 years ago! I marked a queen and then thought I had killed her as she had all the attributes of a dead bee! Rather than disposing of her I laid her on a top bar because I could not think what to do and closed the hive up feeling quite dejected. A week later I opened up to see if they had raised emergency cells, and was amazed to find her as large as life strutting round the hive. Another week went by and I decided to clip her, which I was going to do originally. You can guess what happened, she ‘played dead’ again!
Your article is interesting as I had concluded that it was some sort of defensive behaviour as she could not then be seen as a threat. I think it could be genetic as her daughter displayed the same behaviour, although I have not seen it since.
Very interesting. Chanelopathies like myotonia congenita are almost always recessive traits which would make it unlikely to also be present in the daughters of a queen that displayed the behaviour. Not absolute, but unlikely. If and when I find the queen again I’ll see if she ‘swoons’ again and – if I rear stock from her next year – whether her daughters also behave similarly.
Many thanks for your comment … the first I’ve seen confirming ‘repeat offending’ 🙂
Hi David – I have had 2 queens faint on me after marking on the comb in a press in cage – so no direct handling. The first time I put down to perhaps pressing down too hard to immobilise her. Convinced I had probably killed her – I left her with bees attending her on top of the frame, closed up and was surprised to see her busy laying a week later. The following year – her daughter behaved exactly the same way after being marked. This time I watched her for a while and she eventually slowly began to recover. A friend had a similar experience this year and Tony Harris too recently reported his experience on Twitter. I’m a very small time beekeeper but only breed my own queens – but I haven’t seen it since. Not sure about the channelopathy theory – I had wondered if it was more of a stress response – it happens in humans too – under stress – some people fall to the ground and lie motionless until the perceived threat has past.
The channelopathy comments are simply drawing parallels with goats … I’ve no idea whether that’s got anything to do with it really, though it suggests ways to test this.
And certainly there are many other reasons for ‘fainting’, including significant stress.
I’ll look back through Tony’s Twitter comments – I’d missed them.
Finally, interesting that a daughter queen showed the same response. That would argue against a channelopathy if my understanding of honey bee genetics and the – almost exclusively – recessive nature of myotonia congenita is correct.
I’m with Ann and the idea of predatory avoidance behaviour although I appreciate your comments about queens mainly living in the dark – but maybe they have a particularly good reason to be cautious when they are not. It seems other insects ‘play dead’ when under threat too. It could be an inherited trait too. Definitely worth another cup of coffee!
Thanks for the coffee … I’ll post something there later (but not tonight, it’s been a long day with the bees).
I’d have thought the only time(s) a queen was ever visible was when she was on an orientation or mating flight. Surely she spends the rest of the time in between the frames? ‘Playing possum’ when out on a flight would be nonsensical … and if the hive is disturbed enough that she’s exposed, then the entire colony have got some pretty major problems.
However, behaviour now may reflect evolutionary history … so perhaps it was useful then.
It would be interesting to know what proportion of queens do this. My guesstimate is less than 0.5% (based on numbers I’ve marked over the last decade).
I’m now going to have to look into this a bit more as this wikipedia article has introduced me to the terms tonic immobility (temporal paralysis) and thanatosis (feigning death). Something for the long, dark winter perhaps …
Great videos though!
The wonders of smartphones 🙂
David I experienced this once as a fairly new beekeeper of 2 yrs. I marked the Queen and she ‘ played dead ‘! I was so upset that I had managed to kill her that I just closed the hive up and left them for a few days. When I went back in to finally sort the problem out there she was swanning about as if nothing had happened!! I looked it up on Google but wasn’t able to get much info so thank you for this interesting blog!
It must have been quite distressing for a relative newcomer to the hobby. My comments above are just suggestions … I’ve no greater insight into why they (rarely) do this.
Interesting account of “fainting” queen. I too have seen it a few times. I’ve always thought it happens as part of the evolutionary response of animals playing dead to avoid being eaten by predators that eat only live prey. Professor Jamie Ellis mentioned the same thing in one of his “Bees in a Podcast” presentations.
I guess that’s a possibility but it seems a strange adaptation for an animal that lives in complete darkness (so small predators wouldn’t be able to see it) and an adaptation that’s likely to go singularly unnoticed by large predators that target hives – bears and honey badgers for example.
Jamie might be right – he often is – but, if so, I’d expect it to be much more regularly seen by beekeepers. I don’t keep count of the queens I mark, but it probably averages out at ~20 a year over the last decade, and I’ve only seen it once. That seems rare for an evolutionary evasion strategy.
Interesting comments from others here about seeing it more than once in the same queen or her daughters. If I can find her today (or when I finally do) I’ll add a note to the post.
Hello David, I’ve seen it about 4 times myself. Well yes, she is in the dark most of her life and something happens in her brain to do with eyesight, (I’ve forgotten the scientific name that I read in a journal recently) but basically she is photophobic except when in mating or swarming mode. So taking her out into the light might trigger something along those adaptive lines, though why it would only happen to certain queens and not all, who knows. An interesting post of yours. Thank you.
Manfredini and colleagues looked at gene expression associated with queen mating. There were a bunch of genes involved in vision downregulated after mating, including things like Rhodopsin 2. They state “Changes in the expression of these visual perception genes mirrors the transition from photophilic behaviour observed in virgin queens that engage in mating flights, to more photophobic behaviour in mated queens confined within the nest.” They predict some of these genes will be upregulated as the colony prepared to swarm.
One of the things that’s interesting to me is that – like fainting – not all mated queens appear equally photophobic. It’s not uncommon to find a queen that continues looking for empty cells and laying eggs after a frame has been removed from the hive. Others appear unperturbed, but stop laying, and a few – even mated queens – rush about like virgins.
I’m hoping to find and mark the fainting queen early next week … I’ll try and remember to also see whether she’s ‘naturally nervous’ before I grab her 😉
With Best Wishes
Interesting, David — this is one new beekeeper with Myasthenia Gravis, a muscle wasting disease not closely related to myotonia and I don’t faint!
I’m struggling to find the recently-marked queen in 1 hive, marked with enamel paint [!] as the posca pen had run out….maybe the enamel killed her off ??
I’ve heard that the workers sometimes take exception to ‘scent’ of enamel paints (no personal experience) but it should have been OK if the paint was dry when you re-introduced her. Are there eggs? That’s a sure sign that she’s there but hiding (assuming you marked her over 3 days ago of course).
Good luck finding her.
Hi David, I’ve seen this once, 2 seasons ago. Most distressing (for me as well as the queen). I marked her in a cage and she came round after a few minutes but not fully. As others have also said, I left her on the top bars and she was behaving normally and laying well by the next inspection. I’ve since marked queens only on the comb with a baldock cage, rather than directly handling or taking away from the comb. I’ve also found there is more risk of balling, if using a cage away from the comb.
Interesting … going by the number who are commenting “it happened to me”, perhaps it’s a bit more common than I thought.
I’ve got one of those Baldock cages and periodically stab my fingers on it when rummaging around in the bee bag for something else. I used it when I started marking queens, but soon changed to a cage with a foam-topped plunger.
I don’t think I’ve ever had problems with queens being balled when returning them. I only use Posca pens and, once it’s dry, I just remove the plunger and lay the ‘barrel’ with the queen in it along a seam of bees over the brood nest. She often just calmly walks out.
When she doesn’t, she’s usually joined by a load of workers who finally encourage her to return to the hive. I’ve certainly had less problems doing this than returning a queen by hand (for example, after moving her off a frame I want for something else … like grafting) which I’m always a bit wary about.
Hi David, This is really interesting. Just 3 weeks ago we were delighted to find a laying queen in a well tempered hive. We transferred her to a marking cage and gently administered a blob of paint. She immediately keeled over – subsequently recovering exactly as you described – with twitching, leg stretching and abdominal pulsing. After about 10 minutes we returned the groggy looking queen to the hive and she slowly wobbled her way down between 2 frames.
We inspected after 7 days to find her looking completely well and walking purposefully over the comb. BUT, on the next inspection we observed her curled up and immobile on the floor. We gently placed her back on a frame and she walked uncertainly around. This time there were queen cells present. We wait to see what we’ll find on the next inspection …….
Hello Bernard & Jane
Sounds like she was on the way out anyway. If the time you saw her after marking she was ‘performing’ and, presumably a week later, she was – shall we say – ‘underperforming’, then perhaps the workforce realised and decided to replace her.
I’ve had a higher proportion of queens disappear this season than I remember in previous years. In our research colonies it’s probably because they’re being opened quite frequently, sometimes by relatively inexperienced beekeepers, but even my production colonies appear to be replacing queens quite frequently.
It’s always disappointing waiting for the queen to start laying, finding her once she is, marking/clipping her and then find you have to go through the whole rigmarole again a fortnight later (with the consequent weakening of the hive due to the brood break).
Fortunately, swarming looks to be just about finishing here, so at least they can just knuckle down and get on with collecting nectar from the lime (please?!).
the joy and mystery of bees…😊
It was good fun to read this, especially as it ended well.
Thanks Paul … always good when things end well.