17 February 2017

TOPIC NAME:- LOCKED IN SYNDROME

COLLEGE NAME:- SUPERIOR COLLEGE
SESSION:- MARCH 2016
DISCIPLINE:- ICU-CCU
PRESENTED TO:- MAM ZUNAIRA
PRESENTED BY:- IFFAT YASMIN

17 February 2017
Locked In
Syndrome
Locked-in syndrome is a
rare neurological
disorder in which there
is complete paralysis of
all voluntary muscles
except for the ones that
control the movements
of the eyes. Individuals
with locked-in
syndrome are conscious
and awake, but have no
ability to produce
movements (outside of
eye movement) or to
speak (aphonia).
17 February 2017
 Cognitive function is
usually unaffected.
Communication is
possible through
eye movements or
blinking. Locked-in
syndrome is caused
by damaged to the
pons, a part of the
brainstem that
contains nerve
fibers that relay
information to other
areas of the brain.

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Signs & Symptoms
Individuals with locked-in syndrome classically
cannot consciously or voluntarily chew,
swallow, breathe, speak, or produce any
movements other than those involving the eyes
or eyelids. In some cases, affected individuals
can move their eyes up and down (vertically),
but not side-to-side (horizontally). Affected
individuals are bedridden and completely reliant
on caregivers. Despite physical paralysis,
cognitive function is unaffected.

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Signs & Symptoms
Individuals with locked-in syndrome are fully
alert and aware of their environment. They
can hear, see and have preserved sleep-wake
cycles.
Affected individuals can communicate through
purposeful movements of their eyes or
blinking or both. They can comprehend people
talking or reading to them.

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Signs & Symptoms
Individuals with locked-in syndrome often
initially are comatose before gradually
regaining consciousness, but remain
paralyzed and unable to speak.

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 Causes
Locked-in syndrome is most
often caused by damage to a
specific part of the brainstem
known as the pons. The pons
contains important neuronal
pathways between the
cerebrum, spinal cord and
cerebellum. In locked-in
syndrome there is an
interruption of all the motor
fibers running from grey matter
in the brain via the spinal cord to
the bodys muscles and also
damage to the centers in the
brainstem important for facial
control and speaking.

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Causes
Damage to the pons most often results from
tissue loss due to lack of blood flow (infarct) or
bleeding (hemorrhage) less frequently it can
be caused by trauma.
An infarct can be caused by several different
conditions such as a blood clot (thrombosis) or
stroke

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Causes
. Additional conditions that can cause locked-
in syndrome include infection in certain
portions of the brain, tumors, loss of the
protective insulation (myelin) that surrounds
nerve cells (myelinolysis), inflammation of the
nerves (polymyositis), and certain disorders
such as amyotrophic lateral sclerosis (ALS).

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 cerebromedullospinal disconnection
de-efferented state
pseudocoma

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Affected Populations
Locked-in syndrome is a rare neurological
disorder that affects males and females in equal
numbers. Locked-in syndrome can affect
individuals of all ages including children, but
most often is seen in adults more at risk for
brain stroke and bleeding. Because cases of
locked-in syndrome may go unrecognized or
misdiagnosed, it is difficult to determine the
actual number of individuals who have had the
disorder in the general population.

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Related Disorders
Symptoms of the following disorders can be
similar to those of locked-in syndrome.
Comparisons may be useful for a differential
diagnosis.
Locked-in syndrome is also called pseudo-coma
because affected individuals are conscious but
make little body movement like unconscious
eyes-closed coma patients or unconscious
eye-open vegetative state patients.

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Related Disorders
Akinetic mutism is a rare
neurological condition in
which an affected
individual does not move
(akinetic) or talk (mute)
despite being awake.
Individuals with akinetic
mutism have normal
sleep/wake cycles, but
(when awake) lie still and
unresponsive, neither
moving nor talking.
Akinetic mutism is a form
of minimally conscious
state often due to vascular
or traumatic damage in the
midline frontal grey matter.

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Related Disorders
A variety of conditions can cause symptoms or a
clinical picture that is similar to locked-in
syndrome. These disorders or conditions include
Guillain-Barre syndrome, myasthenia gravis,
poliomyelitis, polyneuritis or bilateral brainstem
tumors. As said, locked-in syndrome can be
mistaken for a vegetative state that may occur
secondary to trauma or a variety of different
conditions, especially if affected individuals
have visual or hearing loss making the diagnosis
more difficult

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Diagnosis
A diagnosis of locked-in syndrome is usually
made clinically. A variety of tests may be
performed to rule out other conditions. Such
tests include magnetic resonance imaging
(MRI), which shows the damage to the pons,
and magnetic resonance angiography, which
can show the blood clot in the arteries of the
brainstem. These tests can also rule out
damage elsewhere in the brain.

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Diagnosis
An electroencephalogram (EEG), a test that
measures the electrical activity of the brain,
may reveal normal brain activity and sleep-
wake cycles in individuals with locked-in
syndrome.

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Diagnosis
Evoked potentials, tests that average the EEG
signal in response to stimulation (pain or
auditory or visual), permit a look at the
damaged responses in the brainstem and the
preserved responses in the brain

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Diagnosis
Electromyography and nerve conduction
study can be used to rule out damage to the
muscles and nerves.

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Diagnosis
An electromyography is a test that records
electrical activity in the skeletal (voluntary)
muscles at rest and during muscle
contraction. Nerve conduction study
determines the ability of specific nerves to
relay nerve impulses to the muscles.

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Diagnosis
An MRI uses a magnetic field and radio waves
to produce cross-sectional images of
particular organs and bodily tissues such as
the brain. MR angiography uses a magnetic
field and radio waves to produce cross-
sectional images of blood vessels inside the
body.

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Treatment
Treatment should first be aimed at the
underlying cause of the disorder. For
examples, reversal of a basilar artery blood
clot (thrombosis) with intraarterial
thrombolytic therapy may be attempted up to
six hours after symptoms onset. Tumors may
be treated with intravenous steroids or
radiation.

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Standard Therapies
There isno specific therapyof locked-in
syndrome. The only treatment is asupportive
therapy. In some cases is also used
neuromuscular stimulation of affected
muscles, but a result is not very good. The
prognosis of this disease is very bad and it
usually leads to the death within few months
or years.

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Often affected
individuals in the
beginning may need
an artificial aid for
breathing and will
have a tracheotomy
(a tube going in the
airway via a small
hole in the throat).
People usually die
because of some
breath insuficience or
pneumonia.
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 Feeding and drinking will not be possible via
the mouth (it may cause respiratory infection
by running into the lungs rather than
stomach) and hence will need to be assured
via a small tube inserted in the stomach called
gastrostomy

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 It is important to
establish an eye-coded
communication as soon
as possible. Healthcare
givers and family and
friends should try to find
out what is the easiest
code for the affected
individual and
consequently all use the
same code. This can be
'look up' for 'yes' and
'look down' for no or
whatever is the easiest
movement for the
specific case.

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 Communication is then limited to closed yes-
no questions and can next be replaced by
eye-coded letter spellers affected individual
look down to choose her or his letter.

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Next, treatment should be aimed at the early
rehabilitation of the small voluntary movements
that remain or recover (often in a finger or foot
or swallowing and sound production).
Rehabilitation and various supportive therapies
are very beneficial and should be started as
early as possible even if it needs to be stressed
that recovery of near-normal motor control,
speaking, swallowing and walking are extremely
unusual.

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 Nursing care
Family education regarding patients care is
one of the biggest needs

Stimulating the mind of the patient with

music, being read to, etc.

Coordinating interdisciplinary team regarding

patients care

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Following a plan for patient
care
Respiratory function
Place patient in lateral recumbent position,
keeping neck in neutral position

Elevate head of bed 30 degrees unless

contraindicated

Oxygenate with 100% oxygen before and

after suctioning

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Suction oropharyngeal airway or via
endotracheal/tracheostomy tube every 1-2 hours
to clear drainage. Limit suctioning to 10 seconds
or less, 1 insertion per attempt

Provide tracheostomy care every 4 hours

Frequently monitor rate, depth, pattern of

respirations

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Observe frequently for signs and symptoms of
respiratory distress

Auscultate chest every 2 hours for adventitious

sounds

Monitor ABG values periodically, continue pulse

oximetry

Administer supplemental oxygen as ordered

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 Provide mouth care every 2-4 hours, brush
patients teeth every 8 hours

If patient is mechanically ventilated, provide

sedation vacation with spontaneous
breathing trial as ordered

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 Cardiovascular function
Monitor vital signs frequently

Monitor rate, rhythm, quality of apical and

peripheral pulses

Document any dysrhythmias

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 Dont use foot gatch under patients knees or
place constricting objects behind knees

Position patient so each joint is higher than

previous joint; distal joints will be highest

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 Integumentary system
Use lubricants, protective dressings, proper
lifting techniques to avoid skin injury from
friction/shear when transferring/turning
patient

Use pillows or other devices to keep bony

prominences from direct contact with each
other

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Optimize nutrition and hydration

Conduct pressure ulcer admission assessment,

reassess risk daily; inspect skin daily

Provide pressure-relieving devices but not donut-

type devices

Use protective barriers on fragile or irritated skin

17 February 2017
 Dont massage bony prominences

Perform risk assessment with a reliable,

standardized tool (Braden Scale)

Clean skin at time of soiling; avoid hot water

and irritating cleaning agents; use
moisturizers on dry skin

17 February 2017
 Keep patients heels off bed at all times

Turn and reposition patient at least every 2

hours

Protect skin of incontinent patients from

exposure to moisture

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 Musculoskeletal function
Perform passive range-of-motion exercises at
least 5 times/day

Position patient in proper body alignment,

using trochanter roll, splints, slings, pillows,
etc.

Collaborate with physical therapist

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 Urologic function
Monitor intake and output

Follow strict aseptic technique in care of

patients urinary catheter

Remove urinary catheter as soon as possible

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 Consider intermittent catheterization program

Provide perineal care

Monitor urinalysis and urine culture and

sensitivity results for signs of infection

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 Gastrointestinal function
Monitor and record character and frequency
of bowel movements

Auscultate bowel sounds

Use peptic ulcer prophylaxis as ordered

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 Neurologic function
Provide sensory stimuli by talking to patient;
explain surroundings, treatments

Encourage family to touch, talk to patient

Use orientation instruments (clock, window,

favorite objects, etc.)

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Nutrition and hydration
Request nutritional consultation

Maintain accurate intake/output record; include

daily calorie count

Monitor skin turgor, mucous membranes for

dryness

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 Pain
Assess for nonverbal pain indicators

Assess for distended bladder, fecal impaction

Assess for foreign object on/under skin

Administer analgesics, provide alternatives

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 Monitor urine specific gravity, serum
osmolality values

Provide hydration as ordered

Weigh patient daily

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REFERENCES
TEXTBOOKS
By Rachel L. Palmieri, RN-C, ANP, MS
2009 by Lippincott Williams &
Wilkins. All world rights reserved.
Sahoo S, Pearl PL. Locked-In Syndrome. NORD
Guide to Rare Disorders. Lippincott Williams &
Wilkins. Philadelphia, PA. 2003:554.
Lyon G, Adams RD, Kolodny EH. Eds. Neurology of
Hereditary Metabolic Diseases in Childhood. 2nd
ed. McGraw-Hill Companies. New York, NY;
1996:347-348

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REFERENCES
National Institute of Neurological Disorders and
Stoke. Locked-In Syndrome Information Page.
February 13, 2007.
Orphanet encyclopedia. Locked-In Syndrome.
December 2003. Available at: http://www.orpha.net
Accessed on: April 12, 2010.
Maiese K. Locked-in Syndrome. The Merck Manual
Online Medical Library. January 2008. Available at:
http://www.merck.com/mmpe/sec16/ch212/ch212c.h
tml Accessed on: April 12, 2010.

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 IF YOU HAVE ANY QUESTIONS
THEN
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