Locked-in syndrome: When you're paralysed, but still aware

The 2007 movie The Diving Bell And The Butterfly is based on the memoir of the late French Elle editor-in-chief Jean-Dominique Bauby (played by Mathieu Almalric, left) who developed locked-in syndrome as a result of a stroke in 1995. — Handout

I recently read about something called pseudo-coma. Is there such a thing? Doesn’t “pseudo” suggest that it is not really a coma, but masquerading as one? But what can possibly masquerade as a coma?

To understand pseudo-coma, let’s talk about coma first.

If someone is in a coma, it means that you are in a state of prolonged loss of consciousness.

Most comas last for several weeks.

If you are in a coma for a longer time, you may transition into a vegetative state, which may lead to brain death.

A coma will manifest as such:

• Closed eyes with the pupils not responding to light, suggesting that the patient’s brainstem reflexes are depressed.
• Limbs (i.e. arms and legs) not responding – not even to painful stimulation – except for reflex movements.
• Possible irregular breathing.

A coma can be caused by multiple conditions, such as a head injury, stroke, bleeding in the brain, brain tumour, infections spreading to the brain or involving the brain, diabetes, drugs, or even alcohol intoxication.

A coma is a medical emergency.

You have to rush the patient to the hospital immediately so that doctors can preserve their body function.

So what is a pseudo-coma?

A pseudo-coma is better known as locked-in syndrome.

People who have locked-in syndrome are conscious and alert.

They can think, reason and hear everything that is going around them.

But they cannot speak, move or show facial expressions.

Some may be able to move their eyes up and down – these are called vertical eye movements.

One can even have normal sleep-wake cycles in this syndrome.

Locked-in syndrome is not common.

But if it happens, it tends to occur more in people aged 30 to 50 years old.

Oh dear, it sounds terrible.

It can be torturous.

There are several types of locked-in syndrome:

• Classical form

  You are immobile, meaning you are unable to move your arms, legs and body.

  But you can move your eyes up and down. You can also blink.

  You can hear everyone talking around you.

  You can think and reason.

  In this type of locked-in syndrome, other people can communicate with you, as you can establish a communication code using your eye movements.

  You also cannot feel pain.

• Incomplete form

  This is akin to the classical form with a twist.

  You do maintain some sensation and movement in some parts of your body.

• Total immobility form

  This is also like the classical form, except that you cannot even move your eyes.

  Therefore, you have no means of communicating with others.

  However, you can think and reason, and still hear everyone around you.

  If put on an electroencephalogram (EEG), your brain waves can be measured.

What causes this locked-in syndrome?

Locked-in syndrome is caused by damage to a certain part of the brainstem called the pons.

It looks like a horseshoe.

The pons is a mass of nerve fibres that connects your medulla oblongata (the lowermost part of your brainstem) to your cerebellum, which is the part of your brain that governs coordination and physical movement, including the movements of your limbs and facial muscles.

The pons is an important linkway to your spinal cord, which allows neural messages to be transmitted in order to tell your body to move, and also the other way – for your body to tell your brain about sensations such as pain.

The word “pons” means “bridge” in Latin.

In addition to being an important conduit and movement coordinator, the pons regulates your sleep-wake cycle, breathing and micronutrition.

That is why, when your pons is damaged, all these functions may completely or partially cease.

What causes the pons to be damaged?

Anything that can cause damage to your brain can involve the pons.

The commonest cause is a stroke that affects that particular area.

It can be either a blood clot type of stroke (ischaemic) or a bleed in the brain (haemorrhagic stroke).

Other causes may include infections that are only limited to that part of your brain, a tumour, loss of the protective myelin sheaths of the nerve cells in that area, head injury and abuse of certain drugs.

Is there any recovery from locked-in syndrome?

The doctors will have to find the cause of the locked-in syndrome and treat it.

If it is an ischaemic stroke, for example, the doctors must try to find the clot and dissolve it with thrombolytic agents.

For the syndrome itself, only supportive therapy can be given.

You may need an aid for breathing, a tube for feeding, and management to prevent bedsores due to prolonged immobilisation.

Speech therapists can help patients communicate with eye movements and blinks for those who can move their eyes.

There are even communication devices that allow affected people to access the Internet.

Patients with locked-in syndrome can recover.

However, it is unlikely that they can recover completely, and some residual paralysis and loss of sensation may remain.

Some get around on a motorised wheelchair and have 10 to 20 years of an adjusted life.

ALSO READ: A bond that doesn't paralyse

Dr YLM graduated as a medical doctor, and has been writing for many years on various subjects such as medicine, health, computers and entertainment. For further information, email [e-mail]. The information provided is for educational and communication purposes only, and it should not be construed as personal medical advice. Neither The Star nor the author gives any warranty on accuracy, completeness, functionality, usefulness or other assurances as to such information. The Star and the author disclaim all responsibility for any losses, damage to property or personal injury suffered directly or indirectly from reliance on such information.