From the Guidelines
Locked-in syndrome is a rare and severe neurological condition where a person is fully conscious and aware but cannot move or communicate verbally due to complete paralysis of nearly all voluntary muscles in the body except for the eyes. This condition is categorized under severe cerebral disability, as described in the Cerebral Performance Categories (CPC) guidelines 1, where patients are conscious but dependent on others for daily support due to impaired brain function. The characteristics of locked-in syndrome align with category 3 of the CPC, which includes patients who are paralyzed and can communicate only with their eyes.
Key Features of Locked-in Syndrome
- Complete paralysis of voluntary muscles except for the eyes
- Full consciousness and awareness
- Inability to communicate verbally
- Retention of cognitive abilities
- Ability to communicate through eye movements or blinking
Causes and Treatment
The most common causes of locked-in syndrome include stroke, traumatic brain injury, diseases affecting the myelin sheath, and certain infections or tumors affecting the brainstem. Treatment focuses on supportive care, preventing complications, and establishing communication methods such as eye-tracking devices or brain-computer interfaces, as supported by guidelines for uniform reporting of data from drowning and other cerebral performance categories 1. Physical therapy, speech therapy, and occupational therapy are crucial for preventing muscle atrophy, contractures, and developing communication strategies.
Prognosis
The prognosis for locked-in syndrome varies, with some patients showing minor motor improvements over time, though complete recovery is extremely rare. The focus of care is on improving the patient's quality of life, given the severe nature of the condition and its impact on morbidity and mortality.
From the Research
Definition and Characteristics of Locked-In Syndrome
- Locked-in syndrome is a rare and devastating condition characterized by tetraplegia, lower cranial nerve paralysis, and anarthria with preserved cognition, vertical gaze, and upper eyelid movements 2.
- It is also defined as a condition where a patient is aware and awake but cannot move or speak due to complete paralysis of all voluntary muscles except for the eyes 3, 4.
- The syndrome has three subtypes: classical, partial, and total, with varying degrees of motor function loss and communication abilities 4.
Causes and Diagnosis of Locked-In Syndrome
- The most common cause of locked-in syndrome is an ischemic event, such as a basilar artery occlusion, which damages the ventral pons and caudal midbrain 4.
- Other causes include hemorrhagic events, tumors, infections, and traumatic brain injuries 5.
- Diagnosis is based on clinical manifestations, including quadriplegia, paralysis of cranial nerves, and preserved vertical eye movements, as well as differential diagnosis to rule out other conditions such as persistent vegetative state and brain death 5.
Treatment and Management of Locked-In Syndrome
- Rehabilitation and management of locked-in syndrome require a multidisciplinary approach, including physical therapy, occupational therapy, and speech therapy to improve communication and mobility 2, 3.
- Treatment options also include the use of intrathecal baclofen to manage spasticity and potentially support the recovery of consciousness 6.
- The goal of rehabilitation is to establish communication, mobility, and connectivity to promote independence, autonomy, and improve quality of life 2, 3.
Prognosis and Quality of Life
- The prognosis of locked-in syndrome is generally poor, with most patients remaining locked in and experiencing poor quality of life, especially in terms of motor functions 4.
- However, with specialized rehabilitative care and access to proper equipment, long-term outcomes and quality of life can be favorable 2.
- Patients with locked-in syndrome often report a high quality of life and well-being, despite the pessimistic views of medical professionals and caregivers 3.