Locked in syndrome is a rare neurological disorder in which there is complete paralysis of all voluntary muscles except for the ones that control movement of the eyes. Individuals with locked-in syndrome are conscious and awake, but have no ability to speak or move.
Patients are often initially comatose before gradually regaining consciousness, but remain paralyzed. They typically cannot voluntarily eat, breathe, speak, or produce many movements other than those involving the eyes or eyelids. Affected people are bedridden and completely reliant on caregivers. Despite this, cognitive function is unaffected.
The disorder resembles a coma, but people with locked-in syndrome are alert and aware of their environment. They can hear, see and can comprehend people talking or reading to them.
Locked-in syndrome can result from any condition that destroys the middle part of the brain stem, most commonly a stroke. Other causes include:
• Guillain-Barré syndrome
• Infections (in or surrounding the brain stem)
• Traumatic brain injury
• Prescription error
• Diseases of the circulatory system
Locked in syndrome is a disorder that can affect individuals of all ages including children. It is, however, most often seen in adults more at risk for stroke and bleeding.
A diagnosis of the brain disorder is usually made clinically. A variety of tests may be performed to rule out other conditions. Imaging tests of the brain, such as MRI and CT are done to determine the cause, and locate any treatable disorders that may be contributing to the problem.
Because this syndrome can be mistaken for stupor or coma, doctors test people who do not move and appear unresponsive by asking them to open and close their eyes.
Treatment options focus on the underlying cause of the disorder. Tumors, for example, may be treated with steroids or radiation. Rehabilitation and various supportive therapies are very beneficial and should be started as early as possible.
Recovery depends on the cause and its severity. In rare cases, some individuals recover limited motor abilities, however, recovery is seldom complete. Most people need permanent full-time nursing care.
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