Chiari - A Silent Brain Disorder

hello and welcome to fire of learning today i'd like to interrupt the standard content on this channel to take time to raise awareness for a group that could very much use it the individuals comprising this group suffer from a silent disorder this disorder is largely unheard of even among medical professionals yet the anatomical abnormality responsible for their strife is believed to be present in millions of people who don't even know it this disorder is called a type 1 chiari malformation in this video we're going to be talking about this rather unusual disorder this is not medical advice i am not a doctor i cannot diagnose you i definitely can't do surgery on you however as you will be able to see in the description everything i say here comes from medical professionals or where appropriate accounts from individuals who suffer from this disorder i'd like to thank my friends brendan and casey for their assistance in providing information about this disorder what is type 1 chiari malformation a chiari malformation is a structural defect involving the back of the skull and brain hans kiari was the physician who discovered it malformation means simply of course improper formation as the name implies chiari type 1 belongs to a group of different types of chiari malformations type 1 is by far the most common in chiari 1 the cerebellum herniates into what's called the foramen magnum in english this means that the lower back part of the brain is squeezed into the opening at the base of the skull where your spinal cord connects to your brain stem that sounds unpleasant but what happens to people with this abnormality and why specifically is it a problem well truth be told for some people it isn't a problem at least not that doctors know of we'll talk more about this later but this has to do with the amazing flexibility of our brains which is a part of what makes them so interesting however for others there are negative effects which is why we're here this is a problem for two primary umbrella reasons the first is that the flow of cerebrospinal fluid could be blocked cerebral spinal fluid or brain fluid is a fluid with the consistency of water that surrounds the brain and spinal cord its purpose is not actually fully understood but it appears to play a role in things like cushioning the brain from impacts and removing waste products from it for some people with chiari malformation the cerebellar tonsils not to be confused with the tonsils in your throat act like a cork blocking the normal flow of brain fluid this can cause a number of problems including disorders such as hydrocephalus and syringomyelia you've likely heard of hydrocephalus or water on the brain this refers to an excessive buildup of cerebrospinal fluid in the brain causing pressure and damage syringomyelia however you've probably not heard of this happens when the buildup of cerebrospinal fluid creates a fluid-filled cyst within the spinal cord not everyone with chiari develops syringomyelia and vice versa but the two are strongly associated with each other much more so than with hydrocephalus the second reason that this is an issue is the direct compression of tissue and nerves in the area itself the foramen magnum is only supposed to contain your brain stem and cranial nerves when the area becomes crowded with the cerebellar tonsils it can lead to problems what are the signs and symptoms the brain is a complicated organ so the number of possible symptoms is rather long people will not necessarily have the same combination of symptoms some people have no symptoms whatsoever other people have rarely life threatening symptoms the main symptoms include headaches specifically headaches in the back of the head towards the base of the skull which are made worse by exertion neck pain numbness weakness and or tingling especially in the extremities trouble of swallowing nausea and vomiting dizziness balance and coordination issues memory and concentration problems vision problems ringing in the ears also called tinnitus and more these symptoms are rarely obvious to others making this very much an invisible illness unsurprisingly these main symptoms are what one would expect from a problem with the cerebellum brain stem and cranial nerves specifically however other structures can be affected as well in some cases as there is evidence that malfunction in one area of the brain can sometimes cause issues across the brain a point often included in discussions about concussions the cerebellum's primary job is motor control the brain stem's primary job is to connect the brain to the spinal cord and regulate many of the body's involuntary functions like digestion and heart rate however it's of course much more complicated than this with these areas almost certainly handling tasks that science does not yet understand rarely symptoms such as paralysis and breathing issues can occur most of the time chiari type 1 is only life-threatening when paired with one of the many disorders associated with it like syringomyelia however it can lead to what's called central sleep apnea on its own this is different from the obstructive sleep apnea that you're probably more familiar with central sleep apnea signals a problem in the brain and its ability to keep you breathing while you're unconscious this can be lethal this however is again quite rare however there are also indirect ways in which people with chiari die such as from an increased sensitivity to head injury and suicide the frequency of this is not as well documented and so the life expectancy while it's probably fairly normal cannot yet be ascertained so what causes this to happen and what do you do to treat it uncommonly chiari can be acquired from something like a head injury brain tumor or a cerebrospinal fluid leak the latter of which is another often misdisorder however overall chiari is believed to be mostly congenital which means people are typically born with it it's probably mostly a genetic issue despite being congenital most people are not diagnosed until their 20s and 30s as it seems for many people for unknown reasons symptoms are not present or at least are much more subtle until early adulthood this is where chiari starts to become complicated and confusing and it becomes clear how little is actually known to science about kiari which is part of why raising awareness is so important let's back up how is kiari diagnosed well generally it seems to be with great difficulty the symptoms of chiari malformation can resemble those of many other disorders some of which like migraines are much less concerning most doctors have never even heard the word chiari before and so are not often quick to identify it thus misdiagnosis and even maltreatment is unfortunately not uncommon some individuals with chiari suffer in confusion and fear for years possibly even decades before they find out what's wrong with them kyary is generally diagnosed based on the images produced by an mri machine often it is held that the cerebellar tonsils must be descending by at least five millimeters to meet diagnostic criteria this requirement is not justified by current science however the degree of herniation has not been shown to closely correlate with symptoms individuals at three millimeters who don't meet the criteria of some doctors may suffer debilitating symptoms attributable to chiari compression and csf blockage whereas someone with 25 millimeters of herniation may have no symptoms whatsoever and live a completely normal life without any known deficits why then do some individuals become symptomatic while others remain symptom-free this is a great mystery there are not clear differences in imaging between symptomatic and asymptomatic patients which is a part of what makes this disease so confusing it is not uncommon for head injury to be associated with the onset of symptoms however it seems that it's most common for symptoms to be triggered for no known reason in early adulthood how many people have type 1 chiari malformation and within that group how commonly do symptoms arise it's hard to say the official estimate is that 1 in 1 000 people have a chiari malformation but according to most researchers that is almost certainly a great underestimate caused by the fact that most people with chiari do not get mris and are therefore never diagnosed it's probably more like 1 in 100 people again though this is the number based on estimates of people who meet the three to five millimeter criteria it seems that overall three to five percent of the population have some degree of tonsillar ectopia that's a fancy term for a descent of the tonsils but do one in 20 people have symptomatic chiari no do one in 100 no it may be the case that only 1 in 1 000 have symptoms clearly attributable to chiari what separates them from the majority is a medical mystery however the other asymptomatic 90 are perhaps not totally asymptomatic this 10 of chiari patient's number would only represent patients with clear symptoms attributable to the abnormality there has been very little research done on people who may be in between debilitating symptoms and the symptom free it may be worthwhile to establish how often supposedly benign chiari cases are associated with things like migraines anxiety disorders mood disorders autism insomnia and other conditions which would almost never warranted mri on their own and so finally what can be done for people with this disorder well there is no cure it's kind of hard to cure anatomical abnormalities like this in the traditional sense of the word cure but there is treatment because of the limits of what an mri can tell doctors treatment often depends on self-reporting of symptoms most of the time asymptomatic patients don't need treatment and may simply be monitored most symptomatic patients respond well to things like medication and avoiding activities that could worsen their condition such as contact sports chiropractors and roller coasters however about 20 percent of individuals diagnosed with chiari will require decompression surgery the surgery is usually successful and rarely dangerous although it's not totally risk-free and is quite invasive thus it's reserved for patients who will clearly benefit from surgery the process involves removing bone in the back of the skull to make the foramen magnum bigger the intention is to allow more room for the tonsils and restore the flow of cerebrospinal fluid the goal of surgery is to stop symptoms from getting worse not to improve them totally reversing symptoms can be difficult due to the complicated nature of nerve damage however in most cases there is at least partial symptom improvement before surgery it is very important to know whether a patient has hydrocephalus idiopathic intracranial hypertension a connective tissue disorder such as ehlers-danlos syndrome or another comorbidity which could cause an unsuccessful surgery chiari 1 malformation is not a typically life-threatening disorder but it is clearly often life-altering and can be debilitating the pain it causes the millions of people around the world who have it is often amplified by how difficult it can be to be understood and to find medical professionals who are familiar with and trained to treat this disorder raising awareness is the first step towards change [Music]

Channel: Fire of Learning

Views: 203,564

Rating: 4.93993 out of 5

Keywords: Syringomyelia, Hydrocephalus, Ehlers Danlos, IIC, Intracranial Hypertension, Cerebrospinal fluid leak, Intracranial hypotension

Id: VpbPspqZ0M8

Length: 12min 48sec (768 seconds)

Published: Mon Sep 27 2021