There are a lot of different neurological disorders that affect many people throughout the world. Neurological conditions are medical conditions that affect the brain and brain function which can limit mobility, speech, motor skills and overall development. We know about some medical conditions that relate to the brain but there are still neurological conditions we do not know much about and how they affect a person long-term. Neurological disorders are very hard to diagnose sometimes given the fact sometimes a person can not communicate very well so it might be difficult to find all of the signs and symptoms of a neurological disorder. One rare neurological disorder that is commonly found in children is called locked-in syndrome. This neurological condition is very rare and not many cases have been reported so there is not a lot we know about the disease as of now. There are a lot of interesting facts related to locked-in syndrome and a lot of reasons why this medical condition can be so debilitating.
Locked-in syndrome is defined as a state of awareness and wakefulness where you have paralysis of the lower cranial nerves and suffer from quadriplegia. There is a loss of facial movement and expression as well as lack of movement, communication and speaking except through eye movements. This rare neurological disorder makes you have complete paralysis in all of the voluntary muscles throughout the body, except for the muscles that control eye movement. People who suffer from locked-in syndrome can think and use reasoning just like everyone else and can still use their cognitive skills completely. Someone who suffers from locked-in syndrome has no way to move or communicate their brain activities and they can not feel most of their body. People who have locked-in syndrome still have cognitive functions and are fully awake with normal sleeping cycles.
Locked-in syndrome occurs in children when there is a pontine hemorrhage that causes quadriplegia and then damaged the cranial nerves. Locked-in syndrome can be a result of trauma to the brain, circulatory system disease or an overdose of medications. People who have locked-in syndrome due to circulatory system diseases often have damaged myelin sheaths which are the protective layer surrounding the nerve cells. The cranial nerves are the nerves that control the horizontal motor functions. Brainstem lesions are also another possible cause of locked-in syndrome and can be detected early during childhood. The rarity of locked-in syndrome cases is usually due to the condition being missed or misinterpreted during childhood which can result in worsening symptoms or an incorrect diagnosis. If your child is in a vegetative state then you should be looking for signs of locked-in syndrome such as eye movement and eye movements as a result of trying to respond to verbal commands. Knowing early on if your child has locked-in syndrome can help facilitate appropriate medical care and treatment especially for pain or for managing those important decisions regarding long-term care.
Since not much is known about locked-in syndrome, there are no real guidelines when it comes to treatment or care of someone with the condition. Diagnosis of locked-in syndrome is clinical based on some tests to rule out other conditions. Brain imaging is used such as a CT and MRI to help identify a potential pontine malformation. The brain imaging can also be used to help diagnose locked-in syndrome because it can show brainstem lesions. Pediatric physicians and medical staff do not know exactly how to care for locked-in syndrome and they really do not know how much the child feels or if they feel pain at all. One thing that makes locked-in syndrome difficult to care for is that the person suffering from it can not really communicate whether or not they are in pain or what symptoms they are feeling. Basically when it comes to possible care and long-term arrangements, it is up to the family on what they wish to do and if they wish to use the end-of-life services.
A lot of times the outcome of the locked-in syndrome diagnosis is terminal but sometimes it can be reversed. If the locked-in syndrome is caused by a stroke or a cause that is partially reversible, therapy can help reverse the paralysis. In order to have a favorable outcome locked-in syndrome needs to be caught early in childhood through the using of the diagnostic tests. Although there is no real treatment except supportive care for locked-in syndrome, it is important to keep the person comfortable. There needs to be preventative measures taken to ensure there are no systemic complications due to the lack of movement and good nutrition needs to also be provided. Physical therapy is also a treatment option if the locked-in syndrome is treatable so that motor skills and functions might return back to normal.
There was one case with an 18 year old female who showed an onset of dizziness and headaches when taken to the emergency room. The findings concluded from a computed tomography scan were normal but the magnetic resonance imaging revealed a large ventral pontine lesion. It was then that a diagnosis of locked-in syndrome was made in just eight hours after being admitted to emergency room. The female was intubated and was comatose during the first month. After a month the patient opened her eye but could not move her eyes. After three months she was transferred to a rehabilitation facility where she had exhibited limited movements of her eyes, right hand and right foot. After one year of being diagnosed, the endotracheal tube was removed and she did not have any facial paralysis. She had begun to laugh and cry and began vocalizing after two years of therapy and have six years had some eating functions back. She still had pain and a hard time swallowing food so gastronomy was still needed. She was able to communicate using an eye-coded alphabet system and can walk a little with assistance. The female has good social interactions with family and friends but has noted being depressed. Although she is not suicidal or manic-depressive she has expressed that no life-saving measures should be taken as a result of an emergency. This is just one of the few known cases of locked-in syndrome where the outcome is at least manageable with therapy and long-term care instead of being a terminal diagnosis.
Kenneth Maiese, MD, “Locked-in syndrome”, The Merck Manuals.
Bruno M-A, Schnakers C, Damas F, Pellas F, Lutte I, Bernheim J, Majerus S, Moonen G, Goldman S, Laureys S, “Locked-in syndrome in children: report of five cases and review of the literature”, Pediatric Neurology.
