Locked-in syndrome

Also called LIS, Pseudocoma, Brainstem stroke and communication

Fully awake and aware with almost no voluntary movement: how a person locked in communicates through the eyes, and why establishing yes and no is the first medical priority.

What it is

Locked-in syndrome is a state in which a person is conscious, aware and thinking normally but has lost almost all voluntary movement, usually after a stroke or injury to the brainstem. Vertical eye movement and blinking are typically preserved; everything else, including speech, swallowing and facial movement, is not. The person hears and understands everything said in the room.

It's rare, and it's the sharpest test this library has of the principle that runs through it: the absence of any response is not evidence of absence. People have spent weeks assumed unconscious before somebody asked them to look up.

Who it can help

The families and clinical teams of anyone with locked-in syndrome, from the first hours, and the person themselves once a channel exists. The same methods serve people with the most severe motor neurone disease, late Huntington's or severe brainstem injury.

Some people recover movement over months, and some do not; either way, the communication system has to be built for now.

How it works

The first channel is nearly always the eyes: look up for yes, down for no, or blink once and twice. Establishing it means asking questions with known answers, many times, and writing the result above the bed so that every person who enters uses it.

From yes and no, the alphabet. Partner-assisted scanning with the letters grouped by frequency (an E-Tran frame or a printed alphabet board in colour-coded blocks) lets the person spell any word, slowly, with a trained partner. It's how Jean-Dominique Bauby dictated a book. Family learn it in an afternoon and get faster for months.

Then technology: eye gaze devices let the person type independently, with word prediction and text-to-speech, and control a computer, a phone and the environment. A person locked in with a working eye gaze system can write, email, study and run their own care. Where the eyes aren't reliable, a single switch on any movement the person can repeat, even an eyebrow or a cheek, drives scanning. Brain-computer interfaces are the research frontier for people with no movement at all.

Getting started

If you're at the bedside and nobody has established yes and no, do it today. Ask "is your name X? Look up for yes." Try questions with known answers. Write the system on a card above the bed and tell every shift.

Print a partner-assisted alphabet board and start spelling. Ask what the person wants to say first; it's often about pain, position or a person.

Ask the team for an urgent referral to the regional specialised AAC service for an eye gaze assessment, and for speech and language therapy to be involved daily. Push: the difference between weeks and months here is a person's entire experience of being alive.

Where to get help

Most locked-in syndrome follows a brainstem stroke, so the Stroke Association is the first charity, with Headway where the cause is injury. Communication Matters explains the AAC services and how to reach them fast. The ACE Centre and Barnsley Assistive Technology Team are specialist NHS centres, and Smartbox and Tobii Dynavox make and trial the eye gaze systems.