LOCKED-IN SYNDROME

The signs and symptoms of locked-in syndrome may include:

• Quadriplegia and paralysis that is global except for vertical eye movements and blinking
• A coma-like condition (pseudocoma) where the patient only can respond or communicate with others by eye movements
• Unresponsiveness to painful stimuli (inability to withdraw an extremity from painful stimuli)
• No horizontal eye movements
• Cannot consciously chew food, swallow, breathe, speak, or move voluntary muscles
• Must rely on caregivers for most basic functions (body movements and hygiene, for example)
• Fully alert and aware of the environment
• Ability to see, hear, and have normal sleep-wake cycles
• Ability to think and reason normally

Diagnosis

It may be difficult to initially diagnose locked-in syndrome in some patients because some patients may be comatose for a while and then develop locked-in syndrome or some patients with a new onset stroke may resemble individuals with locked-in syndrome.

The diagnosis can be missed if eye movement (vertical and blinking) is not assessed in seemingly unresponsive patients. Evidence for locked-in syndrome can be seen with MRI imaging of the specific brain area that shows damage. In addition, PET and SPECT brain scans can further assess the patient’s abnormality.

Treatment

Supportive care is the main treatment for locked-in syndrome since there is really no specific treatment for this condition. Supportive care such as:

• Breathing Support
• Good Nutrition
• Preventing Complications of Immobilization (such as lung infections, urinary tract infections, and blood clot formation)
• Preventing Pressure Ulcers
• Physical Therapy to Prevent Contractures
• Speech Therapy (to help in developing communication via eye blinks and/or eye vertical movements)
• Computer terminal control linked to the patient’s eye movements if possible.

Infrequently, treatment of the underlying cause such as shrinking a tumor or rapidly treating a medical overdose may improve the patient’s condition.