Locked-In Syndrome

Locked-in syndrome (LIS) is a diagnosis which encapsulates patients who are alerts, cognitively conscious and capable of communication but are unable to move or speak (American Congress of Rehabilitation Medicine, 1995). LIS is most often the result of a ventral pons lesion characterized by motor de-efferentation producing paralysis of all four limbs (American Congress of Rehabilitation Medicine, 1995; Smith and Delargy, 2005). There are difference subcategories, described below, that breakdown the extent of motor and verbal impairment, which can be complete or partial (American Congress of Rehabilitation Medicine, 1995).

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Clinically Relevant Anatomy

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Mechanism of Injury / Pathological Process

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Clinical Presentation

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Diagnostic Procedures

The neurobehavioral criteria for diagnosing LIS consist of:

1. Maintained eye opening
2. Basic cognitive abilities are preserved
3. Severe hyophonia or aphonia
4. Quadriparesis or quadriplegia
5. Communication through vertical or lateral eye-movement or blinking (American Congress of Rehabilitation Medicine, 1995).
   
   

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Differential Diagnosis

LIS can present in a similar clinical manner to various other conditions and therefore should be thought of while assessing a patient, in order to provide a correct diagnosis (Laureys, 2010). Potential alternate disorders or conditions that present in a similar clinical manner to LIS include Guillain-Barre syndrome, myasthenia gravis, poliomyelitis, polyneuritis, or bilateral brainstem tumours (Laureys, 2010).Furthermore, LIS could be mistaken as Akinetic mutism, which is a rare neurological condition where the individual will not move (akinetic) or talk (mute) despite being awake (Laureys, 2010).

Finally, LIS diagnosis can be missed and mistaken as being in a vegetative state (Laureys, 2010). This is even more common in those with vision or hearing problems, which makes the LIS diagnosis even more difficult (Laureys, 2010).

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