In this article, I'll tell you about this complicated disorder and how to care for a patient who's locked-in. First, let's look at how it develops.

Conscious but unmoving

* brain tumors that infiltrate the ventral pons

* prolonged hypoglycemia

* hemorrhage that originates within or infiltrates into the pons

* ischemia caused by basilar artery occlusion or hypotensive or hypoxic events

Temporary LIS can be pharmacologically induced or can result from severe cases of chronic inflammatory demyelinating polyneuropathy. In this article, I'll focus on permanent LIS.

The prognosis for patients with LIS is unpredictable. In rare cases, they may regain certain neurologic functions, but the chances for motor recovery are very limited. Research shows that early, aggressive treatment offers the best chance of an improved outcome.

Researchers have classified LIS into three categories:

* classic—quadriplegia and anarthria with preserved consciousness and vertical eye movement

* incomplete—the same features as classic but with remnants of voluntary movement other than vertical eye movement, such as a weak arm or hand movement or sometimes the ability to move facial muscles, cry, or groan

Patients with LIS resulting from a stroke may have had premonitory signs and symptoms, such as headache, dizziness, vertigo, and hemiparesis. These signs and symptoms usually occur within a few days before the onset of the locked-in state, but a few patients experience premonitory signs and symptoms for days to months before LIS onset.

Difficult diagnosis

After beginning to consider a diagnosis of LIS, the neurologist will order magnetic resonance imaging (MRI), the preferred method for identifying ventral pontine lesions. Once a lesion is confirmed by MRI, the neurologist should reexamine the patient for vertical eye movements. He'd ask the patient questions that could be answered yes or no and look for meaningful eye movements to assess mental status.

Computed tomography (CT) isn't considered useful for diagnosing LIS because the resolution quality is compromised by bone artifact and it may be unreliable for imaging pontine infarcts.

Using an electroencephalogram (EEG) to diagnose LIS is controversial at best. The EEG changes caused by brainstem lesions can vary from slight changes to diffuse or bilateral slowing to more striking changes such as paroxysmal discharges and sharp activity, focal abnormalities, or low-voltage fast activity.

Using the most effective assessment tool available, carefully assess the patient and evaluate her level of consciousness. In the past, the Glasgow Coma Scale (GCS) was the gold standard for evaluating a patient with coma. (See Scoring the GCS.)

When you have a patient with LIS, the FOUR Score Coma Scale allows you to accurately assess her eye response, motor response, brainstem reflexes, and respiration. Because a patient with LIS can move only her eyes, the eye response section of the tool is invaluable. Use it to assess whether your patient can track or blink and her response to pain. A score of E4 indicates at least three voluntary ocular excursions. If her eyes are closed, you must open them and examine her vertical tracking. Alternatively, you must document two blinks on command. This finding is key to recognizing that the patient is fully aware and has LIS. If you have any doubts about your findings, consult with another more experienced nurse who can verify your results.

What are the implications when you care for a patient with LIS? To handle this complex role, you'll need to use a multidisciplinary team approach that includes nurses, neurologists, the rehabilitation medicine team, case managers, psychologists, social workers, chaplains, speech therapists, pharmacists, and nutritionists, among others. You'll need to teach the patient and her family (or significant other) about this multifaceted condition. The treatment plan will focus on providing supportive care, medical management, and rehabilitative care while managing complicating factors such as hypertension, diabetes, pneumonia, respiratory failure, and urinary tract infections.

Finding a way to communicate

Patients with LIS are totally dependent on their nurses and other caregivers, and they can't communicate distress, fear, or helplessness verbally. Validate your patient's fear, anxiety, and pain, when appropriate.

Establish a good rapport with your patient with LIS by using a calm, reassuring voice to explain each procedure before you do it. Give your patient control over her care by establishing an eye-blinking pattern for communication. Don't ask your patient open-ended questions; instead frame questions that can be answered yes or no.

Strictly adhere to schedules for care and frequent nursing rounds. Explain any change in routine care to your patient and give her the opportunity to consent to the change whenever possible. Providing consistent sitters for a patient with LIS can reduce her fear of being abandoned.

Involve the patient's family as soon as the patient is diagnosed with LIS. The family and the patient need to work together with the multidisciplinary team to address issues to reduce stress, anxiety, and fear.

Educate hospital staff providing care to patients with LIS. Teach them about the condition and patients' needs and fears. Remind them that the patient can hear and understand everything they say. Increasing staff confidence will reduce everyone's stress and anxiety.

If she has active lateral head or limb movement but can't use a regular call light, provide her with one that's pressure-sensitive and teach her how to use it. This device is activated by slight pressure from any body surface. Patients with classic and total LIS won't be able to use this device.

Although the yes/no eye blink limits the content of communication, it lets the patient respond quickly to yes/no questions. But this system has several drawbacks: The patient has no way of initiating requests, and she can't communicate complex thoughts.

Using Morse code, she can communicate more fully. However, this method requires training the patient, staff, and family members, which may not be practical in the acute hospital setting.

The point board system may be the best solution for communicating. The patient learns a sequence of blinks to initiate use of the board, which holds numbers, letters, and common words placed in columns. This system uses less of the patient's energy because she can blink for columns and rows. With the point board, she blinks the number of times that corresponds with the columns and then the rows for words, letters, or numbers. The assistant can write these down.

Once the patient learns to use the point board, explain the system to her family members. Don't be surprised if they're dubious at first. Help them understand that the patient can communicate quite well with this tool. Everyone involved with this task needs to be understanding and patient.

Now let's explore the nursing care a typical patient needs.

Family education is one of the most important issues that you'll undertake. Because of the complexity of LIS, teach the family to have realistic expectations about what their loved one can achieve. Have them express their frustrations where the patient can't hear them, and then address their concerns. They'll need to accept that the only way to communicate with their loved one will be through a communication or point board, Morse code, or blink yes/no method. Support and encourage the patient and her family members.

Think about ways you could stimulate the mind of someone with LIS, such as by playing favorite music, books on tape, or uplifting TV shows or movies. Encourage family and friends to read to her, touch and massage her, and hold her hand.

Involve the social worker and psychologist to help deal with complex issues that arise. Acknowledge family members' feelings of fear, anxiety, and isolation, and offer them your support. These simple steps may help to allay their fears.

Meeting the patient

Julia McCall, 50, is transferred from the neurologic ICU to your medical unit following admission for a stroke. While Ms. McCall was in the neurologic ICU, the nursing staff observed her keenly and noted signs and symptoms suggesting LIS. Ms. McCall was unresponsive and had preserved eyelid and vertical eye movement, anarthria, quadriplegia with intact sensation, and bowel and bladder incontinence. She was hemodynamically unstable and required a tracheostomy and mechanical ventilation. The diagnosis of classic LIS was confirmed by neurologic exam and testing. Her EEG shows diffuse slowing and the MRI showed a pontine lesion. On admission to the neurologic ICU, Ms. McCall's GCS score is as follows:

* Eye response: eye opening to pain, 2

* Verbal response: no verbal response, 1

* Motor response: no motor response, 1

The FOUR Score Coma Scale is as follows:

* Eye response: eyelids closed, open to pain, not tracking, E1

* Motor response: no response to pain or generalized myoclonus status epilepticus, M0

* Brainstem reflexes: pupil and corneal reflexes absent, B1

* Respiration: breathes at ventilator rate or apnea, R0

After several weeks of intense neurologic and nursing intervention, Ms. McCall's GCS and FOUR Score Coma Scale scores improve, and she's transferred to the intermediate medical unit. At this time, her GCS scores are as follows:

* Eye response: open spontaneously, 4

* Verbal response: no verbal response, 1

* Motor response: no motor response, 1

The FOUR Score Coma Scale score is as follows:

* Eye response: eyelids open or opened, tracking, or blinking to command, E4

* Motor response: no response to pain or generalized myoclonus status epilepticus, M0

* Brainstem reflexes: pupil and corneal reflexes present, B4

* Respiration: not intubated, regular breathing pattern, R4

Over the past weeks of neurologic ICU care, Ms. McCall's condition improved significantly. She opens her eyes spontaneously, enabling her to track and blink on command.

You note that Ms. McCall is fully alert but mute without facial expression or movement, is quadriplegic with intact sensation, and has bowel and bladder incontinence. Her eyelid and vertical eye movement are intact. She has a tracheostomy tube but has been successfully weaned off the ventilator, and has a nasogastric tube for nutrition and an indwelling urinary catheter.

After 3 weeks in the intermediate medical unit, Ms. McCall is transferred to a long-term-care facility with a rehabilitation unit.

Research holds hope

The patient diagnosed with LIS will need intense nursing care and management. Your role is to lead the multidisciplinary team in providing comprehensive medical care to the patient and emotional support to the patient and her family or significant other.

Scoring the GCS

The GCS, developed to standardize observations of a patient's level of consciousness, provides a quick, objective, and easy way to assess central nervous system function. The GCS consists of three subscales that are scored separately, then added to give a score from 15 (best) to 3 (worst). A person with a score of 15 is fully alert and oriented and one with a score of 3 is in a deep coma. Usually a score of 8 or less indicates that the patient is unconscious.

Best eye opening response

4 Spontaneous

3 To speech

2 To pain

1 No response

Best verbal response

5 Oriented to person, place, and time

4 Conversation confused

3 Inappropriate or garbled words

2 Incomprehensible sounds

1 No response

Best motor response

6 Follows commands

5 Localizes pain

4 Nonpurposeful movement

3 Abnormal flexion

2 Abnormal extension

1 No response

The FOUR Score Coma Scale includes four subscales to score separately for eye response, motor response, brainstem reflexes, and respiration. Lower scores indicate more severe signs and symptoms.

Eye response (E)

Try to elicit the best level of alertness by using at least three trials, then grading the best response. If the patient's eyes are closed, open them and see if the patient tracks a finger or object. In cases of eyelid edema or facial trauma, tracking with only one open eyelid will suffice. If horizontal tracking is absent, examine the patient for vertical tracking. Alternatively, document two blinks on command, which indicates a locked-in syndrome (patient is fully aware).

E4 Eyelids open or opened, tracking, or blinking to command

E3 Eyelids open but not tracking

E2 Eyelids closed, opens to loud voice, not tracking

E1 Eyelids closed, opens to pain, not tracking

E0 Eyelids remain closed with pain

Motor response (M)

Grade the best possible response of the arms. If the patient demonstrates at least one of three hand positions with either hand, the score is M4. If the patient touches or nearly touches the examiner's hand after a painful stimulus compressing the temporomandibular joint or supraorbital nerve (localization), the score is M3. If the patient has any flexion movement of the upper limbs, including withdrawal or decorticate posturing, the score is M2.

M4 Thumbs up, fist, or peace sign to command

M3 Localizing to pain

M2 Flexion response to pain

M1 Extensor posturing

M0 No response to pain or generalized myoclonus status epilepticus

Brainstem reflexes (B)

Grade the best possible response. Examine pupillary and corneal reflexes. Preferably, test corneal reflexes by instilling a few drops of saline on the cornea from a distance of several inches to minimize corneal trauma from repeated examinations, or use cotton swabs. Test the cough reflex to tracheal suctioning only when both of these reflexes are absent. A score of B1 indicates both pupil and corneal reflexes are absent, but the cough reflex (using tracheal suctioning) is present.

B4 Pupil and corneal reflexes present

B3 One pupil wide and fixed

B2 Pupil or corneal reflexes absent

B1 Pupil and corneal reflexes absent

B0 Absent pupil, corneal, and cough reflex

Respiration (R)

For ventilated patients, use respiratory patterns shown on the ventilator monitor to identify patient-generated breaths. Don't adjust the ventilator while the patient is graded, but try to ensure that the patient has a Paco2 within normal limits. To assess the breathing drive, you may need to disconnect the ventilator for 1 to 2 minutes while providing oxygenation. A standard apnea (oxygen diffusion) test may be needed when the patient is breathing at the ventilator rate.

R4 Not intubated, regular breathing pattern

R3 Not intubated, Cheyne-Stokes breathing pattern

R2 Not intubated, irregular breathing pattern

R1 Breathes above ventilator rate

R0 Breathes at ventilator rate or apnea.

Sources: Eken C, Kartal M, Bacanli A, Eray O. Comparison of the Full Outline of Unresponsiveness Score Coma Scale and the Glasgow Coma Scale in an emergency setting population. Eur J Emerg Med. 2009;16(1):29–36; Mayo Clinic. FOUR Score Instruction Guide. Rochester, MN: Mayo Clinic; 2007.

Following a plan for patient care

Use these guidelines to care for a patient with locked-in syndrome.

Respiratory function

Cardiovascular function

Integumentary system

Musculoskeletal function

Urologic function

Gastrointestinal function

Neurologic function

Pain

Nutrition and hydration

Source: Adapted from Hickey JV. The Clinical Practice of Neurological and Neurosurgical Nursing. 6th ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2009.

REFERENCES

Posner JB, Saper CB, Schiff N, Plum F. Plum and Posner's Diagnosis of Stupor and Coma (Contemporary Neurology71). New York, NY: Oxford University Press; 2007.