He Entrapment syndrome or Locked-in syndrome (LIS) is a rare neurological disorder characterized by generalized and complete paralysis of the body's voluntary muscles, with the exception of those that control ocular movements (National Institute of Neurological Disorders and Stroke, 2007).
This pathology leaves the individuals totally paralyzed and worlds, reason why in many occasions it is frequent that the terms are used and?? Captivity Syndrome ??? O?? Closure Syndrome ??? To refer to it.
Enclaustration syndrome results in a secondary condition to a significant brain stem lesion with an involvement of the corticospinal and corticobulbar pathways (Collado-Vázquez and Carrillo, 2012).
In addition, these brain damage can occur as a result of suffering from various conditions: Traumatic brain injury , Several pathologies related to the circulatory system, diseases that destroy the Myelin Of nerve cells or overdose of some drugs (National Institute of Neurological Disorders and Stroke, 2007).
People with the syndrome of cloistering are fully conscious, that is, they can think and reason, but they will not be able to speak or to make movements. However, they may be able to communicate through eye movement (National Institute of Neurological Disorders and Stroke, 2007).
Despite this, most cases in which there is an alteration or severe damage to the brainstem do not exist a specific surgical or pharmacological repair and, therefore, neurological deficits can be chronic (Collado-Vázquez and Carrillo , 2012).
The mortality In the acute phase occurs in approximately 60% of the cases And there is rarely a recovery of motor function, although patients with a nonvascular etiology have a better prognosis (Riquelme Sepúlveda et al., 2011).
Therefore, the therapeutic intervention in this type of pathologies focuses on the basic care that maintains the vital functions of the patient and the treatment of possible health complications (Collado-Vázquez and Carrillo, 2012).
What is locking-in syndrome or locked-in syndrome?
Closing syndrome presents a clinical picture characterized by presenting Tetraplegia (Total paralysis of the four limbs) and Anartria (Inability to articulate sounds), with preservation of the state of consciousness, respiratory function, vision, coordination of eye movements and hearing (Mellado et al., 2004).
At the observational level, a patient with closure syndrome is represented awake , still , Unable to communicate through language and with Preserved eye movements (Mellado et al., 2004).
This pathology was first described in the novel The Count of Monte Cristo Of Alejandro Dumas, about the year 1845. It was not until 1875 that the cloister syndrome was described in the medical literature by Darolles with the first clinical case (Collado-Vázquez and Carrillo, 2012).
Already in the novel of dumas the main clinical features of this affectation were described:
The old Noirtier de Villefort has been totally paralyzed for six years:"motionless as a corpse", perfectly lucid and communicating through a code of ocular movements .
Due to the devastating consequences of the cloistered syndrome has been described in presenting in many works both cinematographic and television.
About us
Enclaustration or captivity syndrome is a frequent disease. Although its prevalence is not known accurately, in 2009, they had been documented and published 33 cases , So some investigations estimate its prevalence in (Orphanet, 2012).
Because there are many cases of undiagnosed or misdiagnosed captive syndrome, it is difficult to determine the actual number of people suffering from or suffering from this type of pathology in the general population. National Organization for Rare Diseases, 2010).
As for sex, Affects women and men equally plus, Can affect a person of any age , However, is more common in older adults due to brain ischemia or bleeding (National Organization for Rare Diseases, 2010).
The estimated average age for the occurrence of locked-in syndrome due to Vascular causes is 56 years , While the average age for their condition due to Non-vascular factors , Is estimated around the 40 years (Collado-Vázquez and Carrillo, 2012).
Signs and symptoms
The American Congress of Rehabilitative Medicine (1995) defined the Locked-in Syndrome (LIS) as a pathology characterized by the preservation of consciousness and cognitive functions, with one for the realization of movements and communication through language (Brain Foundation , 2016).
Generally, 5 criteria are considered that can define the presence of the cloister syndrome in most cases (National Rehabilitation Information Center, 2013):
- Tetraplegia or Tetraparesis
- Maintenance of superior cortical functions.
- Afony or severe hypophony.
- Ability to open the eyes and perform vertical movements.
- Use eye movements and blinking as a means of communication.
However, according to the location and severity of the lesion, the presentation of several more signs and symptoms can be indicated (Luján-Ramos et al., 2011):
- Anticipatory signs: headache, vertigo, paresthesia, hemiparesis, diplopia.
- Preservation of the waking state and consciousness.
- Motor alterations: tetraplegia, anarthria, facial diaplegia, decerebration rigidity (abnormal posture with arms and legs extended and head and neck arched backwards).
- Ocular signs: Paralysis of bilateral conjugation of horizontal eye movements, maintenance of blinking and vertical eye movements.
- Remaining motor activity: distal movements of the fingers, facial and lingual movement, flexion of the head.
- Involuntary episodes: grunting, crying, oral automatisms, among others.
In addition, the presentation of the captive syndrome can distinguish two phases or moments (National Organization for Rare Diseases, 2010):
- Initial or acute phase : The first phase is characterized by anarthria, respiratory affection, total muscular paralysis and episodes of loss of consciousness.
- Post-acute or chronic phase : Consciousness, respiratory function and vertical eye movements are fully restored.
Types of Captivity Syndrome or Locked-in Syndrome
The captive syndrome has been classified according to different criteria: severity, evolution and etiology. Despite this, etiology and severity are generally the factors that give us more information about the patient's future prognosis (Sandoval and Mellado, 2000).
Depending on the severity, some authors like Bauer have described three categories for the enclaustración syndrome (Brain Foundation, 2016):
- Classic lock-in syndrome (Classic LIS) : Preservation of consciousness, total muscle paralysis except for vertical eye movements and blinking.
- Complete lock-in syndrome (Complete or total LIS) : Preservation of consciousness, in the absence of linguistic communication and eye movements. Complete motor and muscle paralysis.
- Incomplete closed-in syndrome (Incomplete LIST) : Preservation of consciousness, recovery of some voluntary movements and maintenance of eye movements.
In addition, in any of these types, a patient with captive syndrome may present two conditions:
- Transient locked-in syndrome: Is characterized by a neurological improvement that can become complete in the absence of permanent brain damage (Orphanet, 2012).
- Transient locked-in syndrome: There is no substantial neurological improvement and is often associated with permanent and irreparable brain damage (Orphanet, 2012).
Causes
Classically, Locked-in syndrome occurs as a consequence of the existence of lesions in the brainstem , Occlusion or injury of the vertebral or basilar artery or the compression of the Cerebral peduncles (Orphanet, 2012).
In many cases, they are specifically caused by boss (Brainstem area). The protuberance presents important neural pathways that connect the rest of the brain areas with the spinal cord (National Organization for Rare Diseases, 2010).
The lesions usually occur at the level of the corticobulbar, corticospinal and corticopontin pathways, which is why muscular paralysis and anarthria develop. Normally, the somatosensory and ascending neuronal pathways remain intact, thus maintaining the level of consciousness, wakefulness / sleep cycles and cessability to external stimulation (Samaniego, 2009; Riquelme Sepúlveda et al., 2011).
Etiologic Factors of Brain Damage
In general, the etiological factors are usually divided into two categories: vascular and non-vascular events.
Both in adults and children, the main cause is dance thrombosis, occurs in 60% of cases (Sepúlveda et al., 2011).
On the other hand, among the non-vascular causes, the most frequent are traumatic accidents, due to a contusion in areas of the brainstem (Sepúlveda et al., 2011).
In addition, it is also possible that the development of secondary clo- sure syndrome to tumors, encephalitis , multiple sclerosis , Disease of Guillain Barré , Amyotrophic Lateral Sclerosis or myasthenia gravis , Among others (Sepúlveda et al., 2011).
Diagnosis
The diagnosis of this pathology is based both on the observation of clinical indicators and on the use of various diagnostic tests.
Often eye movements can be observed spontaneously and the willingness to communicate through it can also be identified by family and caregivers. For these reasons, it is essential to examine changes in ocular movement in response to simple orders (Orphanet, 2012). To make a differential diagnosis with the vegetative state or of minimal consciousness.
In addition, it will be essential to perform a physical examination to confirm muscle paralysis and inability to articulate the language.
On the other hand, the use of functional neuroimaging techniques can detect the location of brain damage and examine the signs of consciousness.
Some of the techniques used in the diagnosis of captive syndrome or Locked-in syndrome are (National Organization for Rare Diseases, 2010):
- Magnetic Resonance Imaging ( IRM ): Are usually used to confirm brain damage in areas related to the syndrome of captivity.
- Magnetic resonance angiography: Are used to identify the possible presence of a blood clot or thrombus in the arteries that supply the brainstem.
- Electroencephalogram (EEG): Is used to measure brain activity, may reveal the presence of signal processing in the absence of explicit awareness, wakefulness and sleep cycles, among other aspects.
- Electromyography And nerve conduction studies: Are used to rule out damage to the peripheral nerves and muscles.
Treatment
There is currently no cure for captive syndrome, nor a protocol or standard course of treatment (National Institute of Neurological Disorders and Stroke, 2007).
The initial treatment in this pathology is directed to the treatment of the etiologic cause of the disorder (National Organization for Rare Diseases, 2010).
In the early stages it will be necessary to use basic medical assistance both to protect the patient's life and to control possible complications. They often require the use of artificial breathing or feeding measures through gastrostomy (feeding through a small had inserted into the stomach (National Organization for Rare Diseases, 2010).
Once the acute phase is over, the therapeutic interventions are oriented towards autonomous recovery of respiratory function, recovery of swallowing and independent micturition, development of the motor of the head and neck, and finally, the establishment of a communicative code through Of eye movement (Orphanet, 2012).
Subsequently, the treatment is directed toward the recovery of voluntary movements. In some cases, movement of the fingers, control of swallowing or production of some sounds is achieved, while in others the control of no voluntary movement is not achieved. National Organization for Rare Diseases, 2010).
References
- Brain Foundation. (2016). Locked-in Syndrome (LIS) . Obtained from Disorders. Brain Foundation: http://brainfoundation.org.au/
- Collado-Vázquez, S., & Carrillo, J. (2012). Captivity syndrome in literature, cinema. Rev Neurol, 54 (9), 564-570.
- MD. (2011). Locked In Syndrome . Retrieved from WebMD: http://www.webmd.com/stroke/locked-in-syndrome
- Mellado, P., Sandoval, P., Tevah, J., Huete, I., & Castillo, L. (2004). Intraarterial thrombolysis in thrombosis of the basilar artery. Recovery in two patients with enclaemytemen syndrome. Rev Med Chil , 357-360.
- NARIC. (2012). What is Enclaustration Syndrome? Retrieved from the National Rehabilitation Information Center: http://www.naric.com/
- NIH. (2007). Locked-In Syndrome . Retrieved from the National Institute of Neurological Disorders and Stroke: http://www.ninds.nih.gov/
- NORD. (2010). Locked In Syndrome . Retrieved from the National Organization for Rare Disorders: http://rarediseases.org/
- Orphanet. (2012). Enclaustration syndrome . Retrieved from Orphanet: http://www.orpha.net/
- Riquelme Sepúlveda, V., Errázuriz Puelma, J., & González Hernández, J. (2011). Enclaustration syndrome: Clinical case and review of the literature. Rev. Mem., 8 , 1-9.
- Sandoval, P., & Mellado, P. (2000). Locked-in syndrome . Retrieved from"The Book of Neurology": http://escuela.med.puc.cl/