Locked-in syndrome – Rare neurological disorder

Locked-in syndrome  is usually caused by pontine hemorrhage or infarction, causing quadriplegia, disruption and damage to the inferior cranial nerves and centers that control transverse vision. So what are the specific symptoms of this syndrome? Can it be treated? All will be answered through the following article.

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1.  What is locked-in syndrome  ?

Locked-in syndrome  is also known as Locked-in syndrome or LiS, locked-in syndrome. This is a rare neurological disorder. This includes complete paralysis of all striated muscles, except for the one that controls eye movements. People with Locked-in Syndrome  are conscious and alert. However, they are incapable of making movements (outside of eye movements) or speaking (aphasia).

Locked-in syndrome – Rare neurological disorder

Locked-in syndrome

Cognitive function is usually not affected. Can communicate through eye movements or blinking. LiS syndrome is caused by damage to the pons. The part of the brain stem that contains nerve fibers that relay information to other areas of the brain.

2.  Symptoms of Locked-in Syndrome

Locked-in syndrome  is often characterized by quadriplegia and an inability to speak in cognitively intact individuals. People with locked-in syndrome can communicate with others through encrypted messages. By blinking or moving your eyes.

See also: Chiari malformation: Structural abnormalities affecting the nervous system

Symptoms are similar to those of sleep paralysis. Patients with locked-in syndrome are conscious and aware. They did not lose cognitive function. Sometimes they can maintain the ability to perceive and feel throughout the body.

Some patients may be able to move some facial muscles and often some or all of the extraocular muscles. People with this syndrome lack coordination between breathing and voice. This prevents them from making voluntary sounds, although the vocal cords may not be paralyzed.

Locked-in syndrome – Rare neurological disorder

People with locked-in syndrome can move their eyes normally

People with this syndrome may communicate through intentional eye movements or blinking, or both. They can understand people talking or reading to them. People with locked-in syndrome often go into a coma before gradually regaining consciousness. However, they are still paralyzed and cannot speak.

3.  Clinical forms of Locked-in Syndrome

Incarceration syndrome can be classified in three different clinical forms, according to the traditional Bauer classification. This classification is based on the number of motor outputs, which are preserved in the patient.

It refers to a pure form where the patient loses control of all body movements, except blinking and vertical eye movements. Incomplete form when some voluntary movement other than eye movement is preserved.

And finally the total form if complete loss of motor function occurs. The last situation is particularly serious. Because the patient cannot be exposed to the environment at all. Inability to express one's needs and thoughts.

4.  Quality of life

Locked-in syndrome presents itself as the most severe form of motor disability imaginable. However, some scientific reports indicate that the patient's quality of life is not so bad.

Locked-in syndrome – Rare neurological disorder

The quality of life of people with this syndrome is not greatly impaired

A recent survey investigated self-reported quality of life of chronic patients with Locked in Syndrome . And they concluded that many patients lead happy and meaningful lives. Especially when appropriate social services help the patient have a normal role at home as well as in the community.

5.  Additional symptoms being studied

Patients with locked-in syndrome have traditionally been considered cognitively intact. Because all brain structures except the ventral part of the pons appear to be preserved. However, recent evidence suggests that patients may develop some non-motor symptoms.

Locked-in syndrome – Rare neurological disorder

Movement paralysis

These include motor image defects, laughing and crying pathologies. And difficulties in recognizing some facial expressions. Disruption of nerve pathways due to the same lesion causes locked-in syndrome. This may be the cause leading to the appearance of these clinical manifestations.

However, these symptoms were not detected in all affected individuals and are currently under further investigation. Recognition of motor image defects deserves special attention. Because these symptoms can hinder the success of rehabilitation strategies.

6.  Causes of locked-in syndrome

Unlike a persistent vegetative state, in which the upper parts of the brain are damaged and the lower parts are removed. Locked in syndrome is caused by damage to specific parts of the lower brain and brain stem, with no damage to the upper brain.

Possible causes of locked-in syndrome include:

Poisoning cases – More often from the bites of scalloped snakes and other neurovenoms. Because they usually cannot cross the blood-brain barrier.

  • Brain stem stroke.
  • Diseases of the circulatory system.
  • Overdosing on antipsychotics.
  • Nerve cell damage, especially destruction of the myelin sheath. It is caused by osmotic demyelination disease or syndrome (previously indicated as central demyelination). Occurs secondary to too rapid correction of hyponatremia.
  • Stroke or bleeding in the brain, usually from the basilar artery.
  • Traumatic brain injury.
  • Consequences of brain stem damage.
  • Muscular atrophy in amyotrophic lateral sclerosis.
  • Polymyositis .

Locked-in syndrome – Rare neurological disorder

Brain damage is a common cause

7.  Population Affected

Locked-in syndrome  is a rare neurological disorder that affects men and women equally. This syndrome can affect people of all ages. This includes children, but is more commonly seen in adults, who are more at risk for stroke and bleeding.

Locked-in syndrome – Rare neurological disorder

Incarceration syndrome is common in adults

Because cases of locked-in syndrome can go undetected or misdiagnosed. It is therefore difficult to determine the actual number of individuals with the disorder in the general population.

8.  Diagnosing locked-in syndrome

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

Locked-in syndrome – Rare neurological disorder

Brain MRI scan

An electroencephalogram (EEG) is a test that measures the electrical activity of the brain. It can reveal the normal functioning of the brain. Simultaneous determination of sleep-wake cycles in individuals with locked-in syndrome. Electromyography and nerve conduction studies may be used to rule out muscle and nerve damage.

Potentials are elicited, tests that average the EEG signal in response to stimulus (pain or auditory or visual). It allows to look at damaged responses in the brain stem and conserved responses in the brain.

9.  How is Locked-in Syndrome treated  ?

9.1. Treat the cause

Treatment should first target the underlying cause of the disorder. For example, thrombolytic therapy with intra-arterial thrombolysis can be done within six hours of symptom onset. Tumors can be treated with intravenous steroids or radiation.

Locked-in syndrome – Rare neurological disorder

Treatment of brain tumors with radiation therapy

Patients usually need artificial support to breathe and will have a tracheostomy to help them breathe. Feeding and drinking will be provided through a small tube inserted into the stomach called a nasogastric tube.

9.2. Coding method in treatment

It is important to establish encrypted eye contact as soon as possible. Communication is then limited to closed yes-no questions. The next can be replaced by eye-coded alphabetic sentences. For example, say the alphabet and ask the affected person to look down to select their letter.

9.3. Restorative treatment

Next, treatment should be aimed at early restoration of minor voluntary movements that remain. Usually in fingers or feet, or swallowing and making sounds. Communication aids and other assistive technologies have been shown to be beneficial. As well as allowing individuals to become active members of society.

Infrared eye-tracking devices now allow patients to use computers with artificial voices. Simultaneously control their environment, surf the web and send email.

Locked-in syndrome – Rare neurological disorder

Rehabilitation

In rare cases, some individuals have recovered limited mobility. In most people, however, that recovery doesn't happen. People who have recovered hand or head motor control can use this to communicate with computers. Sometimes maneuver their wheelchair.

10.  Prognosis

Very rarely any vital motor function returns. Most patients with incarceration do not regain motor control. However, some people with the condition go on to live longer. A small number of cases may spontaneously recover completely.

11.  Conclusion

Many recent studies and articles in the medical literature have noted that despite being affected by significant mobility impairment, those affected are able to maintain a good quality of life.

In addition, quality of life was not related to the degree of physical impairment. With advances in care and support technology, individuals with Locked In Syndrome can become productive members of society.

Dr. Nguyen Lam Giang 


Locked-in syndrome – Rare neurological disorder

Locked-in syndrome – Rare neurological disorder

Post by Bs. Nguyen Lam Giang about Locked-in syndrome, which causes quadriplegia, disrupts and damages cranial nerves

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