Whatever you do, don't breathe too hard
Before reading, you can try the DYT11 simulator to get a sense of what it feels like.
When you've been living with myoclonic dystonia long enough, you develop a repertoire. Not a musical repertoire, although the body does play its own score sometimes. A repertoire of tricks, workarounds, micro-strategies accumulated over the years to limit the damage. Some are logical. Others are absurd. All are necessary.
This post isn't about the medical journey, the surgery, or the adjustments. It's about the other side: raw daily life, the body's infernal loops, and the methods you invent to survive them.
Why not to breathe
Let's start with the most counterintuitive piece of advice you'll hear today: to avoid a myoclonus, don't breathe.
No, seriously. Deep breathing can trigger myoclonus. Taking a deep breath means engaging the intercostal muscles, the diaphragm, the entire thorax. And in my case, that engagement is an open invitation for the nervous system to fire off a jolt. The body interprets that large respiratory movement as an opportunity to express itself. And express itself it does. Badly.
So you learn to breathe small. To never inhale fully. To avoid complete yawns (try interrupting a yawn halfway through, it's an Olympic sport). To keep a low profile, respiratory speaking.
But it gets better. Or worse, depending on your perspective. One of the most effective strategies I've found is to hold my breath. Not for long, just for the critical moment. The principle is as logically bulletproof as it is stupid: a muscle that's already contracted can't be re-contracted involuntarily. If I voluntarily contract my thoracic muscles by holding my breath, the myoclonus has nothing to grab. It arrives, finds the door shut, and moves on.
It's stupid. But it works. A bit.
The problem, obviously, is that you eventually need to breathe. And when you let go, the window reopens. So you learn to dose it, to release gradually, to breathe in stealth mode. It's exhausting, it's ridiculous, and nobody notices.
Myoclonic storms
An isolated myoclonus, you get used to it. A jolt, a twitch, you put the glass down, you try again. It's annoying, it's tiring, but it's manageable. The problem is when the myoclonus events decide to work as a team.
A myoclonic storm is a barrage. A series of jolts that chain together and self-feed in a positive feedback loop that nothing seems able to break. It works like this:
- A myoclonus occurs. Nothing new so far.
- That myoclonus causes a muscle contraction, which causes pain. Not sharp pain, more of a spasm, a sudden fatigue, like a muscle that's been overworked.
- The pain triggers an avoidance reflex. The same reflex as when you put your hand on a hot plate: the body pulls away, abruptly. Except here, there's no plate. There's nowhere to pull away to.
- That avoidance reflex generates stress. The nervous system goes on alert.
- The stress triggers another myoclonus.
- Back to step 1.
It's a vicious circle in the most literal sense. Each jolt feeds the next. The pain feeds the reflex. The reflex feeds the stress. The stress feeds the myoclonus. And it spins. It can last minutes, sometimes longer, and throughout, the body is caught in a storm it triggered itself and is fuelling without being able to stop.
Breaking the loop takes considerable conscious effort. You have to manage not to react to the pain. You have to manage not to stress over the lack of control. You have to manage to let go at the exact moment when everything in your body is screaming to contract. It's like asking someone to relax while being slapped.
The premonition
There's a phenomenon I've never read about in any medical article, but that I've experienced daily for years: the premonition.
Some myoclonus events, not all, but some, I can feel coming. About a tenth of a second before the muscle contracts, something happens. A signal. Not pain, not tingling, something more subtle, like an electrical tremor running up a wire, a disturbance in the circuit that my brain has learned to recognise. The rogue signal is on its way, and for a fraction of a second, I know it.
What science says is interesting. Officially, myoclonus is not supposed to be preceded by premonitory sensations. It's even a distinguishing criterion from tics, which are often preceded by a well-documented "premonitory urge". Myoclonus, in theory, arrives without warning.
And yet.
In neurophysiology, we know that in cortical myoclonus, a cortical spike precedes the muscle jerk by 20 to 40 milliseconds. This spike is detectable by jerk-locked back-averaging EEG, a technique that averages brain activity by locking onto the moment of movement and going back in time. Proof that the signal exists in the brain before it reaches the muscle.
In DYT11, the myoclonus is subcortical in origin. The aberrant signal originates in the basal ganglia, passes through the thalamus, rises to the cortex, then descends back to the spinal cord and the muscle. That journey takes time. And my personal hypothesis is that after decades of cohabitation, my cortex has learned to detect that signal as it passes through. Not early enough to stop it, but early enough to feel it coming. A hundred milliseconds is the time it takes the brain to consciously process sensory information. That's exactly what I feel.
It's a bit like the aura that precedes some epileptic seizures: the brain picks up abnormal activity before it spreads. Except in my case, it's not innate. It's learned. Thirty years of myoclonus, and the brain eventually recognised the pattern.
The problem is that feeling it coming doesn't mean being able to stop it. A hundred milliseconds is enough to know, not enough to act. The premonition is a useless luxury. Or nearly: in rare cases, when the timing is right and the target muscle is accessible, I can voluntarily contract before the myoclonus arrives, and block the jolt. But it's rare, it's exhausting, and it demands constant vigilance.
Retrocontrol strategies
Living with myoclonic dystonia means becoming an engineer of your own body. You map the triggers, you test countermeasures, you optimise defences. It's a permanent, empirical, and often absurd process.
Contract to prevent contraction. That's the basic principle. A voluntarily contracted muscle resists involuntary contraction. So you contract. Hands on the table, arms along the body, abdominals locked. You build yourself a muscular suit of armour against your own nervous system. It works, but it's exhausting, because maintaining a permanent voluntary contraction consumes energy, and fatigue is itself a trigger. You push the problem back, you don't solve it.
Avoid triggers. You learn the map. Stress, obviously. Fatigue. Cold. Certain movements. Certain positions. Certain emotions, including positive ones (yes, joy can trigger myoclonus, because the body has a dreadful sense of humour). You mentally compile a list of things to avoid, and that list grows over time, because you regularly discover new triggers. The map is never finished.
Let go. The most counterintuitive and the most difficult. Sometimes, the best strategy is to do nothing. To accept the jolt, not to fight, to let the body make its noise and wait for it to pass. Because fighting means contracting, means stressing, means feeding the loop. Sometimes, the only way to break the vicious circle is to refuse to participate. Except that requires managing not to panic when your own body is going haywire. Which is, you'll agree, easier to write than to live.
The supreme irony. Stress is the primary trigger for myoclonus. The best thing to do is not to stress. Except that living with a condition that can trigger a myoclonic storm at any moment is, by definition, stressful. Myoclonic dystonia is a condition that gets worse when you try not to make it worse. The snake bites its own tail, and seems to find it very funny.
Experience it
Words are not always enough. To give an idea of what it feels like, I coded a simulator. It is not reality — reality is worse, and above all it never stops — but it is a glimpse. Try it, and imagine this twenty-four hours a day, for a lifetime.
You don't cure myoclonic dystonia. You learn to live with it. You develop workarounds, refine strategies, map the traps. And some days, despite everything, the body wins. The storm passes, the muscles do whatever they please, and you wait for it to settle while trying very hard not to breathe too deeply.
It's a permanent negotiation with a bad-faith partner. A peace treaty renegotiated every morning. And some mornings, you even manage to find it a little funny.
(A little.)