Managing Locked-In Syndrome: Lessons from a Profile of a Rare Case
Communication by an eyeblink-based Morse code system opens the door to progress for a 32-year-old with quadriplegia but intact cognition.
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A 32-year-old man was transferred to a Cleveland Clinic inpatient rehabilitation hospital from a long-term acute care hospital. Until two months previously, he had been a healthy student, pursuing graduate-level engineering studies in Ohio. At that time, a pontine hemorrhage (see image below) left him with aphonia, dysphagia and quadriplegia. He was on multiple antibiotics due to ventilator-associated pneumonia. He arrived with a tracheostomy tube, a percutaneous gastrostomy tube and a Foley catheter in place.
As he improved, he was decannulated and continued respiratory therapy with breathing exercises. Nutritional support was provided and monitored. He was brought to the rehab hospital’s gym daily for physical therapy and occupational therapy, which consisted of neuromuscular electrical stimulation and passive range-of-motion movements of his arms and legs to retard muscle atrophy and maintain range of motion of his extremities. Transfer techniques were practiced, as was assisted wheelchair mobility.
Locked-in syndrome is a rare condition that most often results from an ischemic or hemorrhagic stroke affecting the corticospinal, corticopontine and corticobulbar tracts in the brainstem. It can also be caused by trauma, tumors, myelinolysis, toxins or heroin abuse.
Patients are conscious and aware but unable to communicate because of paralysis and inability to speak. Often the ability to blink and move the eyes is preserved, offering the only potential avenue of communication. Some patients can change facial expressions or move the head or tongue.
Diagnosing the condition requires very careful observation while asking yes/no questions and instructing the patient to respond by blinking or moving the eyes in prescribed ways. Trying to make contact frequently is warranted, even after a period of months, as it’s common for patients to tire easily and to have a short attention span, especially soon after the injury.
We soon discovered we could communicate with our patient by asking him to blink once for “yes” and twice for “no.” Using this system, we could better assess him by asking sequentially if he had pain, dizziness, nausea, etc. It also enabled our care team and visitors to ask if he wanted to listen to music or an audio book to help provide mental stimulation.
We next moved on to Morse code, using long and short blinks for the code’s system of dashes and dots (see figure below). The rehabilitation team printed up a code sheet for him to refer to during conversations. Morse code communication is tiring for the patient, but the ability to express phrases of even a few words is a big leap from relying on yes/no responses. During daily medical checks, staff routinely ran through the review of symptoms using yes/no blink responses, then asked the patient if he wanted to communicate via Morse code.
More-advanced communication assistive devices are available, and many are eligible for reimbursement under health insurance policies. A device that detects eye movements allows patients to communicate by looking sequentially at individual letters, icons or a slate of common words, while special software types out the results on a computer screen. Patients with intact head or even eyebrow movements can use a “head mouse” system that detects electromyographic activity to move a cursor on a screen for communication and independent computer use.
Family members should be continuously involved as much as possible — remotely by Skype or similar apps if they are not physically present — to communicate, learn day-to-day care and develop long-term plans. The rehabilitation team helped obtain a power wheelchair and taught our patient’s family how to transfer him and use it.
It is especially critical that a patient’s eventual caretakers become comfortable using the communication techniques established in the hospital and are motivated to keep searching for advances as the patient is able and as new technology becomes available.
After six weeks of inpatient rehabilitation, the patient was deemed ready for discharge, and the team assisted with arrangements to send him home.
Rehabilitation of patients with locked-in syndrome is a tremendous challenge that few centers are equipped to undertake. A multidisciplinary rehabilitation team is essential, as is a management strategy that includes the following objectives and components:
The American Congress of Rehabilitation Medicine’s Cognitive Rehabilitation Manual can serve as a guide for treating cognitive aspects of the condition and providing compensatory strategies, but care must be adapted for each patient.
Although many patients regain some function over time, most remain chronically locked in or severely impaired. Clinicians must always be mindful that despite these patients’ grave disability, they are often cognitively intact with normal memory and thought processes. It is a continual challenge for caretakers to help patients feel connected to others and their environment, and to enable them to live out their lives with a level of mental richness approaching what they enjoyed before.
Dr. Aguilera is a physical medicine and rehabilitation physician in Cleveland Clinic’s Department of Physical Medicine and Rehabilitation.