Locked-in syndrome

What is a Locked-in syndrome?

Locked-in syndrome is a complex medical condition presenting with quadriplegia, bulbar sign, and loss of sensation in the whole body due to damage to the brain stem, mostly the anterior pons.

Locked-in syndrome is also known as pseudocode. in this condition patient is aware but can not move because of totally paralysis
of nearly all muscles in the body except the vertical eye movements.

Locked-in syndrome more commonly affects people who are 30-50 years old.

Types of the locked-in syndrome:

• Classical locked-in syndrome
• Incomplete locked-in syndrome
• Total immobility locked-in syndrome

In the classic locked-in syndrome type Quadriplegia and anarthria with preserved consciousness and vertical eye movement.
The incomplete locked-in syndrome is the same as the classic but with some other types of voluntary movement other than vertical eye movement.
In total immobility, locked-in syndromes are complete immobility and an inability to communicate however the person is fully conscious.

Signs & symptoms of locked-in syndrome:

Patient with locked-in syndrome cannot voluntarily:

• Chew
• Swallow
• Speak
• Make facial expression
• Produce any movements below their eyes

Most people with locked-in syndrome can

• Eyes up and down
• Blink
• Hear
• Have sleep-wake cycles
• Total paralysis of the limbs
• Difficulty in regular breathing pattern
• Poor muscle control
• Dizziness
• Vertigo
• Severe aphonia
• Quadriparesis or quadriplegia
• Expression through eye movement or blinking

Causes of the locked-in syndrome:

• Neurotoxic venoms
• Blood-brain barrier
• Brainstem stroke
• The disease of the circulatory system
• Overdose of medication
• Damage to nerve cell
• Destruction of the myelin sheath
• Brain hemorrhage
• Traumatic brain injury

Differential diagnosis of the locked-in syndrome:

• Amyotrophic lateral sclerosis
• Bilateral brainstem tumors
• Brain death
• Coma
• Guillain–Barre syndrome
• Myasthenia gravis
• Poliomyelitis
• Polyneuritis
• Vegetative state

Mechanism of injury:

The most common mechanism of injury is occurred by ventral pontine stroke. most commonly ventral pontine infarction is the result of artery occlusion. more than 20 different mechanisms of the locked-in syndrome have been described. frequent causes of the locked-in syndrome include tumors, infection, trauma, lesions, and brains.

Can you feel pain with locked-in syndrome?

Depending on which type of locked-in syndrome you have, you may be able to feel physical pain or may not be able to feel physical pain.
If you have total immobility type of locked-in syndrome patient won’t be able to feel physical pain due to total paralysis of her body.
If you have to incomplete type of locked-in syndrome patients may be able to feel physical pain and other sensations in some regions of their body.

What tests will be done to diagnose locked-in syndrome?

MRI scan and CT scan :
MRI scans and CT scans can show damage to your pons and other portions of your brain.

Cerebral angiography:
A cerebral angiography test can reveal a blood clot in the arteries of your brainstem or somewhere else in your brain.

Electroencephalogram:
Electroencephalogram tests can measure the electrical activity of the brain. Electroencephalograms can help experience normal brain activity and sleep-wake cycles.

Evoked potential:
Evoked potentials test can measure the electrical activity in the certain brain and spinal cord areas.

Electromyography:
An electromyography test can measure your muscle and nerve work.

Blood test:
A blood test is a metabolic panel. it’s checked by the sodium level of the blood. blood tests can help determine the pontine myelinolysis.

Cerebrospinal fluid examination:
The cerebrospinal fluid examination can help determine the infection or autoimmune condition.

Treatment of locked-in syndrome:

Locked-in syndrome is currently no cure.

Example of

• Medication
• Deep brain stimulation
• Supportive therapies

Medication:

Certain drugs and groups of drugs can help muscle tremors and movement issues:

• Levodopa
• Dopamine agonists
• Monoamine oxidase B inhibitors

Deep brain stimulation:

A surgical procedure that involves inserting wires beneath the skin and into areas of the brain affected by Parkinson’s disease.

Supportive therapies:
Supportive therapies can help a person manage the symptoms and improve the quality of life.

Physical therapy:
Physical therapy can help muscle stiffness and improve flexibility and walking.

To Improve flexibility exercise & release muscle stiffness following exercise are useful:

• forward lunges
• Side lunges
• Cross over
• Standing quad stretch
• Seat Straddle lotus
• Seat side straddle
• seat stretch
• Knee to chest
• standing side stretch
• Standing calf stretch
• Seat Hamstring Stretch
• Shoulder stretch
• Backstretch
• Forward hang
• Butterfly Groin Stretch
• Forward bend stretch

There is no permanent cure for the locked-in syndrome. one therapy is called functional neuromuscular stimulation. electrodes are used to stimulate muscle relaxation.

Summary:

locked-in syndrome is a complex medical condition presenting with quadriplegia, bulbar sign, and loss of sensation in the whole body due to damage to the brain stem, mostly the anterior pons. Locked-in syndrome more commonly affects people who are 30-50 years old.

There are three types of locked-in syndrome. Classical, incomplete, and total immobility.

Treatment of locked-in syndrome is medication, deep brain stimulation, and Monoamine oxidase B inhibitors. Physiotherapy treatment of locked-in syndrome is currently can help to reduce muscle stiffness and improve flexibility and walking.

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