Hyponatremia and Central Pontine Myelinolysis

What is hyponatremia? Information regarding CPM and EPM.

Archive for the tag “neurological disorders”

WHY?

I’ve been trying to stay focused on creating posts that are more about central pontine myelinolysis, what to expect, how to compare it to other brain injuries. I’ve been trying to stay away from writing about me.

Frankly, you can only take so much of listening to someone go on and on about horrible things are in their life. It’s hard living through it too, but most people don’t really care, and they don’t want to feel “bummed” about how bad someone else has it, so I’ve tried to refrain from going on and on about my feelings or my struggles with EPM. Tonight, I have to discuss about what’s going on with me.

So, you might be wondering, what’s wrong?

Today, I was told that the “basic” cognitive testing that I had a few weeks ago, showed that I have significant impairment, but that it’s not consistent. Basically, the doctor felt that my symptoms are being created or exasperated by psychological issues.

I have to say that I agree that stress, fatigue, and anxiety the issues I have worse. Isn’t that true for anyone? Even if you don’t have any brain injury, you’re just perfectly normal, doesn’t stress, fatigue, and anxiety make issues worse?

I’ve never been a strong test taker. Never. I was usually one of the last kids to turn in a test. The last college classes I took, I would run out of time, especially in chemistry. I was usually one of only 4 or 5, still taking a test at the end of the exam period, out of more than a 100 or more. I felt this was because of my perfectionism. In reality, I would just tend to over analyze questions. I would get stuck and read over the same question over and over again because I thought that there were multiple ways to interpret the question.

I have found since I’ve had the brain injury, I’ve had more issues with this. It becomes harder for me to shut off the internal dialogue I have with myself over directions, questions, etc. I will tend to confuse directions for one section of the test with other sections. For example, if you ask me to name all the animals that I can think of, my mental gears start spinning: birds. Well, birds aren’t really animals are they? Aren’t they considered more along the species of reptiles? They have a connection to dinosaurs. Do they want specific animals? Like Robin? Robin is a type of bird. I wish I could look up whether a bird is really an animal. Aren’t animals considered types of mammals? There are marsupials. I wish I could look up the answer. I really don’t want to sound stupid by saying birds if birds aren’t really animals. And what if they want specific animals.  Aren’t animals any living organism? There’s different types of Kingdoms. Shit, I’ve studied this stuff, why can’t think of the right answer? Humans are animals. Maybe I should just say humans. Good answer! My answer is humans. How about insects? FRICK!

SO, that’s just an example of how my mind works in the moment of answering ONE freaking stupid question….and they want me to to name as many animals as possible, as quickly as possible! It’s just not that easy any more. I used to be able to shift gears faster, think through things more quickly, and get to an actual correct response, but I don’t have that ability any more. I get caught, stuck.

Another example, they asked me to count the dots in the following pictures one by one, or maybe they actually said individually. I’m not sure exactly how it was worded. The first problem I have is, is staying focused and tracking the dots. The first group was scattered dots everywhere on a page. There must have been 20 or more. They weren’t organized, and when I started counting them I lost track of where I began, and I wanted to give the right answers, so I double counted them. I thought it was the answer that was important, but it was actually the amount of time that it took that they were monitoring. The next pages they started organizing the dots into groups. My first reaction, well that’s a group of five. There’s two groups of five. There’s three groups of three. It’s a total of 19. Wait, they said count them individually. Maybe this is an illusion.  They have those optical illusions where your mind looks at something and doesn’t process it correctly. Did I miss a dot? So, I counted the dots a second time. No, no I think there aren’t any that I’m missing. Are you sure? Yep, I’m sure. Ok. 19 is the answer.

So, if I had known that the answer didn’t matter, then I would have just blurted out numbers. If I realized that it wasn’t a trick question, I would have been able to respond more quickly. If I had just asked for clarification or asked them to start over once, I figured out what I was supposed to do. Frick!

Trust me. I feel stupid over the testing. I don’t know why that part of my mind is broken. I mean, I did have that problem to some extent prior to the injury, but at some point, my reason would take over, and I would just be able to answer the questions. I would be able to shut down that internal dialogue, and just take a test. At the very least, it didn’t interfere as much as it does now.

In other words, I was never quick at taking tests, but now, I’ve become discouragingly slow. It’s just harder for me to process information, directions, to figure out what I need to do and then do it.

 

I am going to say, in my defense, that I had only had four hours of sleep that night. I really wish that they did testing around the times that I’m normally awake. If I don’t fall asleep until 4 or 5 am, and I’m scheduled to take a test at 9am, I’m practically set up for failure, but they don’t start testing in the afternoon. Two pm would have been the best time for me, but doctors do testing around a typical 9am to 5pm schedule, not a 2pm to 10pm schedule.

So, what does all of this have to do with me?

The testing I did reflected poorly on me, and so my integrity has been questioned.

I am so extremely grateful that I know what my issues are, and that my friends and family believe me and know. My cognitive therapists and occupational therapists believe me and see the struggle that I experience, and they believe me.

It really seems that the testing itself is the only thing that doesn’t work in my favor, but I just don’t think that the tests account for the type of mental distractions that I have because of the brain injury. Well, I had these some of these issues before, it’s just so much worse now.

In the end, getting this news just stresses me out even more, but then I begin to regain my composure. I don’t really care what the tests say. I know what’s going on with me, and that’s what matters. I just have to brush off this bad news and regain my focus. Keep on, keeping on.

I know stress, fatigue, and anxiety complicate my problems, but I’ve always been able to work around those issues. They’ve never stopped me from doing what I wanted or needed to do.

They are causing issues now, and I’ve tried everything I can to control those factors, so that I can become more functional, but they aren’t the cause of the deficits that I have, and it has left me exasperated and frustrated.

Let me give you an example of how people can misinterpret a problem. A man is having a drink at a bar.  He’s chatting with his friends, and as he gets up to go home, his friends stop him and ask him if he needs a ride home. They suggest that maybe he call a taxi. He feels insulted because he’s only had one drink. He refuses the taxi. The next night, at the same bar, he sits down to have a drink. After a glass of wine, he gets up to leave and another person suggests that he not drive home. Again, the man scoffs at the suggestion. On the way home, he gets pulled over by a cop. The cop believes that the man is drunk. The man refuses to take sobriety test. He feels angered at the fact that people keep suggesting that he’s drunk. Since he refused to take the sobriety test, the cop takes him to jail to sleep it off. The next morning, they find the man dead in his jail cell. He wasn’t drunk at any time. He had had a stroke.

How do I feel that this story relates to my experiences? It is not uncommon for people to look at someone with a brain injury and because they do not see any physical injury on the outside, they assume that there is an external cause to the problems that you have. You don’t really have memory issues. You’re just stressed. You have trouble with concentration and reading because you’re mind is creating those problems, because you are focused on issues.

It’s so easy to judge someone when you’ve never experienced the same problem. It’s easier to put on a filter and say that these issues are mental when you’ve never lived with them.

I’ve been told by so MANY people that they’ve forgotten to pay bills. It’s normal. I’ve forgotten where I’ve parked. It’s normal. I’ve forgotten to take my medications. It’s normal.

Yesterday, I couldn’t figure out how old I was! I’m 35, and I forgot. I wasn’t sure if I was 33, 34 or 35. I could not figure it out. I forgot my son’s birthday. I forgot the significance of 9/11 (also my son’s birthday).

THIS is NOT normal for me! This was not who I was before the brain injury. I worked full time. I went to school full time. I took care of the bills. I did NOT have these issues. I’ve never ever had to have an 80 year old man have to shuttle around the parking lot trying to find my car because I couldn’t. I could do advanced math in my head without any issue. I could figure out patterns and trends. I could read through law books, Title 21 of the federal code of regulations.  Shakespeare was like a Dr. Seuss book to me. I could spend 6 to 8 hours reading through legal cases. I could spend 10 to 13 hours studying for the MCAT while working 32 hours or more in a week. What I live with now, IS NOT NORMAL FOR ME! I’ve always lived with stress, but it did not cause impairment.

To have some guy read through one to three tests and can tell me that he has my brain injury figured out, TOTALLY pisses me off. The most brilliant scientists in the world do NOT have a great understanding of how the brain works. They don’t!  Were the tests even designed for a person who has a brain injury? Does it take into consideration that the person has an issue with understanding directions, language, or writing.

I had cognitive testing done BEFORE my brain injury, and I would have difficulty completing tasks in an allotted time. I was able to get the puzzles, etc correct, but not within a standard time frame.

I was told by my cognitive therapist today that I was right. A few months ago, I told her that there is a belief that over time a person’s brain can turn to mush after they’ve had a brain injury. In a person who has had CPM/EPM who lives for longer than a few years, when they move the brain at autopsy, it crumbles. It turns to mush.

When I told my cognitive therapist this, she told me that it wouldn’t happen. (She has been working as a cognitive therapist for more than 20 years. She is an expert.) She went to a conference this weekend, and they are finding that these injuries can kill the brain slowly over time, that the brain can calcify after a brain injury. She told me that I was right.

In the end, what can I do? I have to keep moving on, but tonight, I raised my hands up to God and cried. (I don’t cry often because my immune system causes issues after.) I don’t understand, why?! WHY? Why do I have to live through this? I’ve already had a pretty tough life, but to go through this too! Why God? Why do I have to go through this too?

Can’t I just be normal again? God, I would give anything to have my old mind back! I wish I could just put this whole brain injury thing away! I wish I could get back to doing what I wanted to do. I just want to go back to school, get into medical school, work at saving people.

I wish I knew why.  I wish I could just get over it, as if I was getting over a cold. I don’t think they understand how frustrating these things are for me. I don’t think they understand how strong I am, and how hard I’ve worked, and how desperately I want to put all of this behind me, but it isn’t just a mental thing. It’s just not, and I have to learn how to work with the deficits I have and try to make the best of my life and my abilities as they are. I will continue to work with my therapists in trying to get new connections, with my doctors to get on the right medications, and try to become the closest to my old self as I can. I guess that’s all you can do when you’re living with a brain injury.

 

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Mutism after Brain Injury and Central Pontine Myelinolysis:

I am writing this post for my friend Michael.

Michael developed central pontine myelinolysis a few years ago. In the course of the past 18 months, he has seen a decline in his abilities. He has had ongoing issues with memory, attention, stuttering, movement issues (shakes, tremors, jerks, and spasms), and now he is having issues with mutism.

So, what is mutism? It’s the inability to speak, talk or make vocal noises. In some cases, this issue may be intermittent.

My friend has this issue. A few years after he suffered from central pontine myelinolysis, he began to have issues with mutism. He will go through periods of hours or days without being able to make any sounds. He is not even able to whistle.

Previous to the mutism, he did have issues with a common symptom to CPM/EPM, which was ataxic in nature, dysarthria (general speech issues, including stuttering, stammering, etc).

I have shared this issue. My dysarthria varies in severity. Actually, some days it is barely noticeable. On other days, it’s difficult to communicate because of the stammering.

There does not seem to be any clear reason for the variations, but I have noticed that stress, fatigue, and even fluctuations in my medications can cause the issue. It does get worse when I have to figure out what I want to say, but if I have something that I’m reading from (reciting words), the problem is less significant. I do not have any scientific evidence as to why this happens but my guess is the way that the brain works at processing information. There must be different neurological pathways for reading out loud versus forming ideas and speaking. There is less thought process in reading words out loud from a page versus forming the words for an idea and speaking it.

I find this idea complex. It makes me pause to consider why it is.

Because of this brain injury, I have issues with getting ideas to mind at all, and at times those ideas seem to evaporate as soon as they form. So, there are periods where I do not have anything in my mind. I am desperately trying to think of something, but my mind is blank. Before I had a brain injury, ideas would just be there. I had a “quick wit”. There was far less thinking required. Sure, I would have to manipulate my ideas, the words the that I wanted to use to make my point, based on the audience, but the thought was there. Now, it takes a significant amount of time to just come up with a thought, to form the sentence, and then be able to communicate it effectively. It’s a rather daunting process when it no longer comes to you naturally.

Anyway, Michael’s issue with mutism developed recently, and is sporadic. So, is his condition unique?

One of the first articles that I found was in regards to children who have developed mutism after having cerebellar surgery. Now, this was interesting because central pontine myelinolysis is an injury that generally impacts the pons. The pons is extremely close to the cerebellum.

Because of the locality of the damage to the pons, I am going to suggest that the white matter of the cerebellum can also be impacted. So according to the following research article, it showed that there were children who would have sporadic mutism after damage to the cerebellum, “Cerebellar mutism syndrome and its relation to cerebellar cognitive and affective function: Review of the literature”. http://www.annalsofian.org/article.asp?issn=0972-2327;year=2010;volume=13;issue=1;spage=23;epage=27;aulast=Yildiz

Recent research studies suggest that neurological and cognitive impairments in CMS (cerebellar mutism syndrome) often persist. A prospective study evaluated the neurological status of patients 1 year post-diagnosis based on the presence and severity of ataxia, language difficulties, and other cognitive deficits. [7] Of the 46 patients who had postoperative CMS initially rated severe, residual deficits were common, including 92% with ataxia, 66% with speech and language dysfunction, and 59% with global intellectual impairment. Of the 52 patients with moderate CMS, 78% had ataxia, 25% had speech and language dysfunction, and 17% had global intellectual impairment. Thus, impairment in these domains was common and was also directly related to the severity of CMS. Riva and Giorgi have shown neuropsychological problems a few weeks after cerebellar tumor resection, and prior to further treatment such as radiotherapy or chemotheraphy. [8] Their results reveal a localization related pattern, with problems of auditory sequential memory and language processing after right-sided cerebellar tumor and deficits in spatial and visual memory after left-sided tumor. Lesions to the vermis led to post-surgical mutism, which evolved into speech and language disorders as well as behavioral disturbances ranging from irritability to those reminiscent of mutism. [8]

Now, there is a belief that these issues with mutism are psychological in nature due to the trauma of the event, like car accident. However, this is definitely not the case with Michael, and there has been additional research showing that children that have a stuttering problem, do have injuries in their brains that have been shown to cause this condition. So, it is my belief that if there is an injury significant enough to cause a coma, that it is more likely that it is not a psychological trauma causing the mutism, but an injury to the brain.

So, if that’s the case, then why does the person experience the mutism intermittently?

In the cases of CPM and EPM, I think it is very possible that the injury can progress. Now, this thought goes against the opinions of most medical doctors. Most medical doctors believe that the injury is static; however, in my opinion, it is not CPM/EPM directly causing the injury, but the immune response to the injury. (I would encourage you to review my beliefs on late onset symptoms of CPM/EPM and brain injuries). Basically, the bodies natural response to injury is repair. In my opinion, it does not matter if this injury occurs in your foot, your heart, or your brain. Your immune system sends up a repair “team” no matter where the injury occurs; however, unlike other areas of the body, the brain does not have any non functioning areas, and as the repairs occur more damage is done to surrounding tissue. It creates a slow and steady deterioration, and as in other major structures of the body, scar tissue forms.

This opinion would also explain why a person who is treated with plasmaphoresis after head trauma (including after CPM/EPM) improves with fewer long lasting effects. Generally, it has been shown in previous studies (previously documented in my blog), that in persons who were treated with auto immune disabling treatments, recovered if not fully, significantly.

I also believe that for those who have awoken from a coma with mutism for months or years after, but eventually regain the ability to speak, it is because the brain has healed or has created new neuro- pathways. The following article describes a girl that suffered from a coma and suffered from mutism for 10 months. Eventually, she regained her ability to speak, but she continued to have issues with speaking, cognitive issues, etc.

The patient initially presened as comatose. A period of mutism subsequent to the coma extended for ten months. Following this protracted period of mutism the child demonstrated rapid and unexpected recovery of functional communication skills, despite the persistence of higher level language deficits.

Read More: http://informahealthcare.com/doi/abs/10.3109/02699059009026154

The following article has information that is about a girl that developed mutism after having an injury to the pons. (Bingo! There does seem to be a correlation and an explanation as to why Michael, who has lesions in his pons, has developed mutism.)

 As she was extubated one week later, she was found to have right hemiplegia and muteness. MRI showed a T2- bright lesion on the tegmentum of the left midbrain down to the upper pons. Right vertebral angiography disclosed an intimal ¯ap with stenosis at the C3 vertebral level presumably caused by a fracture of
the right C3 transverse process later con®rmed in a cervical 3D-CT scan. Her muteness lasted for 10 days, after which she began to utter some comprehensible words in a dysarthric fashion. Her neurological de®cits showed improvement within 3 months of her admission. Transient mutism after brain stem infarction has not been reported previously. We discuss the anatomical bases for this unusual reversible disorder in the light of previous observations and conclude that bilateral damage to the dentatothalamocortical ®bers at the decussation of the superior cerebellar peduncle may have been responsible for her transient mutism.

Read more: http://www.springerlink.com/content/h952wk14rwd65798/

Another case of mutism after brain injury, however this person experienced relief with treatment of diazepam:

A 34-year-old woman with a severe closed-head injury had many impairments including apparent global aphasia. After a diazepam premedication for a motor point block she was heard to speak a few words. A trial of oral diazepam succeeded in restoring speech adequate to make her needs known, which persisted on a maintenance dose of 5 mg t.d.s. The possible diagnoses and reasons for this phenomenon are discussed. We suggest that diazepam may be useful in assessing speech in selected people with severe head injuries.

http://www.ncbi.nlm.nih.gov/pubmed/8877308

The following article is only available fully if you pay for it. However, according to the introduction, a woman developed delayed mutism after she had a brain injury caused by drug related issues:

A 49-year-old woman developed a catatonic mute state a few weeks after methadone overdose. Clinical, radiological and histological findings were consistent with toxic spongiform leukoencephalopathy, which adds a potentially deadly side-effect to a generally considered safe substitution for heroin……..

Mutism

The inability to generate oral-verbal expression, despite normal comprehension of speech. This may be associated with BRAIN DISEASES or MENTAL DISORDERS. Organic mutism may be associated with damage to the FRONTAL LOBE; BRAIN STEM; THALAMUS; and CEREBELLUM. Selective mutism is a psychological condition that usually affects children characterized by continuous refusal to speak in social situations by a child who is able and willing to speak to selected persons. Kussmal aphasia refers to mutism in psychosis. (From Fortschr Neurol Psychiatr 1994; 62(9):337-44)

http://www.bioportfolio.com/resources/pmarticle/38247/Brief-Communication-Delayed-Akinetic-Catatonic-Mutism-Following-Methadone-Overdose.html

The next article describes a girl that had issues with stunts in her brain. Her injury also happened in the cerebellar and tracts in the brain stem. The following has a detailed explanation of the researchers belief why akinetic mutism (AK) occurs:

Actually, in the latter situation, AM seems to be related to lesions that occur along pathways that originate in the mesencephalon ([Fig. 6]) and project widely to dopamine receptors in the spinal cord, brainstem, diencephalon, corpus striatum, and mesiofrontal lobe. The resultant behavioral abnormality causes the patient to remain awake but unable to initiate motor activity in response to sensory stimuli. Pressure transmitted to the diencephalon from the hydrocephalus can cause AM. The underlying mechanism is believed to be damage to the periventricular monoamine projections in the thalamus and hypothalamus caused by the expansion of the third ventricular wall. This is the theoretical basis for use of a dopamine agonist in humans with AM, giving gratifying results.

In posterior fossa surgery, damage of the dentate nuclei is the main factor for AM. Fibers emanate from the damaged dentate nuclei through the superior cerebellar peduncles to the contralateral red nucleus and the thalamus and supplementary motor area connected by the dentatothalamocortical pathway[11] ([Fig. 6]). As already mentioned, the supplementary motor area has proven necessary for the initiation of speech[9]

In contrast to AM secondary to hydrocephalus, in which the injured pathways are dopaminergic and/or monoaminergic, in the cerebellar mutism, the neurotransmitters consist of glutamate and aspartate that are found in cerebellorubral and cerebellothalamic fibers, whereas some GABA-containing cells give rise to cerebellopontine and cerebello-olivary fibers. Some cerebelloreticular projections may also contain GABA.

https://www.thieme-connect.de/ejournals/html/10.1055/s-0032-1313632

Now, I found the following article extremely interesting. It describes brain injuries that occur due to lack of blood flow and/or lack of oxygen. Now, why I found this next article extremely interesting because it documents improvements in symptoms initially, but months to a year or more later, the person’s symptoms progress. This is the same type of progression that has been reported in those with chronic concussions, and the majority of those  that I know with CPM/EPM. I believe that there is a connection that is not clearly understood at this time in regards to how the brain reacts to injury, and it can occur regardless of the injury. (HI stands for hypoxic- ischemic and BI stands for Brain Injury)

Delayed Post-Hypoxic Leukoencephalopathy

In rare cases, early and complete recovery from HI-BI is followed a few days to weeks later by a severe demyelinating syndrome; this syndrome, delayed post-hypoxic leukoencephalopathy, characterized by acute or subacute onset of severe and progressive neuropsychiatric problems such as delirium, psychosis, parkinsonism, and/or akinetic-mutism, and/or quadriparesis, among others. Although this condition is often described as a delayed sequelae of carbon monoxide-induced HI-BI, it has been associated with nearly all causes of HI-BI (Shprecher and Mehta 2010). The neural mechanisms of delayed post-hypoxic demyelination have not been established definitively. However, combinations of toxic exposure (e.g., carbon monoxide, inhaled heroin), genetic (e.g., pseudodeficiency of arylsulfatase A, abnormalities of other genes regulating myelin turnover), and age-associated vascular risk factors have been suggested as possible contributors to this unusual post-hypoxic condition. Regardless of mechanism, this syndrome is characterized neuropathologically by diffuse bihemispheric demyelination that generally spares the cerebellum and brainstem. Neurological and neurobehavioral improvement over the first 3 to 12 month periods following onset of this syndrome is typical, but many survivors experience persistent cognitive impairments (particularly impairments of attention, processing speed, and/or executive function), parkinsonism, and/or corticospinal tract signs. There are case reports describing symptomatic and functional improvement of the cognitive and parkinsonian sequelae of delayed post-hypoxic leukoencephalopathy during treatment with stimulants, amantadine or levodopa. The observation that these agents offer some benefit in this context despite their lack of efficacy for the same sequelae of HI-BI itself may reflect differences in the anatomy of these conditions: in HI-BI there is involvement of both gray and white matter, limiting the target of pharmacotherapies more severely than in delayed post-hypoxic leukoencephalopathy, which involves only white matter.

 

I have to say that this idea of mutism after brain injury is absolutely possible. It seems to be more studied in children who have experienced brain injuries vs adults. There seems to be some professionals who believe that it is a psychological issue and others that believe there is a neurological injury that causes it. I believe that you must rule out the physical injury before you consider the psychological cause. Keep in mind that it was only recently discovered that stuttering has a physical cause. This is because the brain is phenomenally complex, and we do not have the technological advancements nor the physical understanding to map the complexity of the brain. This means that you have to approach the subject with an open mind.

Despite the lack of information and understanding, there does appear to be a physiological link to the pons, the cerebellum, and possibly the basal ganglia and the ability to speak. It is also likely that not all of these injuries progress or heal at the same rate, which means that even after mild brain injuries there is a chance that mutism can develop or resolve.

I would HIGHLY recommend that after a brain injury, even mild brain injury, discuss the use of steroids (anti-inflammatory types of steroids that inhibit the immune system-not testosterone) or possibly plasmaphoresis. There has also been research that shows that hyperbariac oxygen exposure can also speed recovery and provide a better recovery. There seems to be a lot of scientific evidence that shows a person’s immune response is in part if not entirely responsible for late onset symptoms.

There will be more to come on this topic as I locate more information.

 

Related articles

 

Drawing a connection between general brain injuries and CPM/EPM:

A diagram of the forces on the brain in concussion

A diagram of the forces on the brain in concussion (Photo credit: Wikipedia)

I’ve said it before, but I believe it needs to be addressed further. Doctors do not know that much about CPM/EPM. Because there are only 2,000 to 2500 cases that are definitively diagnosed as CPM/EPM each year, there aren’t any “experts” that we can turn to. Because of this, it is necessary to draw understanding from what we know about brain injuries in general.

The brain is the most complex part of a human body, and the most interesting thing to remember is that we do not know that much about it.

Previously, it was believed that if you did not pass out from an injury (hit, fall, car accident) then a brain injury did not occur. Now, we know that is not always the case.

You can have short term to long term cognitive, physical or emotional issues from a simple bump on the head or even from whiplash.

So, let’s investigate brain injuries further:

The first type of more common and less recognized form of brain injury is a concussion. Concussion occurs when your brain is jostled, which results in impaired functioning. It can occur from a fall, a hit, a car accident, even from shaking (shaken baby syndrome). Generally, a concussion is determined from the symptoms that a person experiences. In other words, you may or may not have any outward physical signs of trauma, like bumps, bruising or bleeding. You may not even have a direct hit to the head. You may experience an impact to the body that leads to a jolt to the head that causes injury to the brain.

Concussions cause microscopic injuries that are generally not detectable by CT scans and do not cause pronounced bleeding of the brain. It is believed that the damage in the brain is from cellular damage. It is also believed that the damage to the brain is widespread. This is why if there is bleeding, it will not typically show on a CT scan because it is not significant enough to pool in one area to be detectable.

So, concussions result from injuries to the way the brain cell (neuron) functions vs having damage to the blood vessels in the brain that causes more significant bleeding. This type of injury is similar to the cellular type of injury that those with central pontine myelinolysis or EPM. You will also find this type of physiological type of injury with MS too.

The brain cells (neurons) may be severed completely in concussions or there may be physiological damage that is done that impacts the way the cell functions. So, the brain cell itself may be damaged or the way it works may be damaged.

What do I mean by that? I would compare it to when you have a neck injury that causes paralysis or a neck injury that just causes numbness and tingling to an extremity. If you have paralysis, the damage is complete and there’s little or no function to the impacted sites, and it can not be repaired. The wiring is cut and the signals can’t get through. If you have an injury that causes numbness and tingling, there is some information being processed, but it is not being processed correctly. This would be comparable to having a short circuit in an electrical wire. Sometimes, the information will get from point A to point B, sometimes it won’t. In these instances, sometimes your body can repair the damage.

(The following is a picture of a neuron…the cells that compose your brain tissue. )

English: Complete neuron cell diagram. Neurons...

I would recommend checking out the following link for a little more information regarding the physiology of concussions (http://www.cordingleyneurology.com/contuseconcuss.html)

Based on what type of injury occurs, concussions can be mild (a person does not lose consciousness) or severe (a person can lose consciousness or even slip into a coma).

So how do you know if a concussion is mild or severe?

Generally, hospitals will look at the person’s symptoms to determine how severe a concussion is and also on if the person lost consciousness and for how long. That said, symptoms may or may not develop right when the injury takes place, and because of typical limitations on insurance plans, hospital staffing, and resources, most emergency rooms will dismiss the person to the care of family or friends within a few hours if the did not lose consciousness from the injury.

It is suspected that there are 1.6 to 3.8 million sports related concussions each year. Each year approximately 1.4 million people seek care for brain injuries. It’s obvious from the numbers I just mentioned that a significant number of people, especially those who participate in sports, do not seek medical treatment for the injuries that they have.

It can mean that a person does not suspect that their injury is significant enough to require treatment, or it might be that people do not realize a connection between their symptoms to the injury that they experience. I believe it is the latter.

This means it is important to recognize the symptoms of a concussion. Typical indicators of a concussion:

Physical Issues:                   Cognitive Issues:  

• Headache                            • Feeling mentally
• Nausea                                  “foggy”
• Vomiting                             • Feeling slowed  down
Balance problems             • Difficulty Concentrating
• Dizziness                              • Difficulty Remembering

• Visual problems                • Forgetful of recent information or conversations

• Fatigue                                • Confused about recent events

Sensitivity to light           • Answers questions slowly

• Sensitivity to noise          • Repeats questions

• Numbness/ Tingling

• Dazed or stunned

•Seizures may also occur immediately or even up to a year or more later.

Emotional Issues:                           Sleep Issues:

• Irritability                                        • Drowsiness

• Sadness                                            • Sleeping less

• More emotional than usual             • Sleeping more

• Nervousness                                      • Trouble falling asleep

I HIGHLY, HIGHLY recommend checking out the following link to learn more about the effects of concussion and other brain injuries (this is a great tool for those who have a brain injury as well as those who live with them)— http://www.brainline.org/landing_pages/TBI.html

Check out the following on how scientists are determining the function of how the brain works : http://connectedsocialmedia.com/5697/future-lab-mapping-the-network-in-the-brain/

It is also important to understand that you may not develop all of these symptoms, and the symptoms may not appear immediately after the injury. It may take days or weeks before the symptoms appear. It may happen a few hours after the injury. And unlike other brain injuries, these injuries do not typically appear on CT scans or MRI scans.

You may experience the following longer lasting issues in your daily life:

• Increased problems paying attention/concentrating
• Increased problems remembering/learning new information
• Longer time required to complete tasks
• Increased symptoms (e.g., headache, fatigue) during school/work
• Greater irritability, less tolerance for stressors
Until a full recovery is achieved, you may need the following supports:

• Time off from school/ work
• Shortened day
• Shortened classes (i.e., more frequent breaks)
• Rest breaks during the day
• Allowances for extended time to complete work/assignments/tests
• Reduced homework/work load
• No signiicant classroom or standardized testing at this time
Physicians and school personnel should monitor the student’s symptoms
with cognitive exertion (mental effort such as concentration, studying) to
evaluate the need and length of time supports should be provided.

The information above is from the CDC: http://www.cdc.gov/concussion/HeadsUp/physicians_tool_kit.html

Generally, a person will recover from mild concussions in a few weeks, but it is also important to remember that concussions can “build”. If a person, experiences a concussion and it is mild, and then experiences an additional injury, days,weeks or even months later, the injury can be catastrophic. It can actually lead to death. For this reason, there are new policies being implemented in schools and college athletic programs throughout the country that bench players for weeks or months following minor concussions.

Until concussions are understood more fully, I believe this should be a mandatory step for the protection of the individual.

Ok, so how does this relate to CPM/EPM? Concussions can impact any area of the brain, but as mentioned above the type of injury found in a concussion is believed to impact the physiology of the brain cells. It impacts how brain cells relay chemical signals, and this is true for CPM/EPM too. This is why there are similarities in the emotional, behavioral, cognitive and sleep symptoms of CPM/EPM and concussions.

I plan to research brain injuries further to hopefully discover answers as to why our experiences are so vast and different, and hopefully to determine what we can anticipate in how the injury responds to treatments.

Have a great night!

Additional symptoms related to CPM:

 

I’ve previously described movement issues like dystonia, Parkinson like tremors, other tremors, and random jerking movements, but this is something I have not heard about previously, choreic.

I had no idea what the word meant or what it is related to before a few days ago, so please feel free to add any input you might have about it.

 

The dictionary definition is: “An involuntary spasmodic twitching or jerking in muscle groups not associated with the production of definite purposeful movements.”

The American Heritage® Medical Dictionary Copyright © 2007, 2004 by Houghton Mifflin Company. Published by Houghton Mifflin Company. All rights reserved.
Basically, these movements are involuntary movements and jerks, so I guess in a way, I have discussed this issue before. I have jerks a lot.
If I get really stressed there seems to be this movement that my left leg does. It’s weird, and if I every experience it for a long period, I will upload a video of it. It really feels like I should have control of it. It seems ridiculous that I can’t, but it’s like my body has a mind of its own and this is one thing that I never had an issue with before. It really bugs me, but others don’t seem to notice. I guess some might consider it a  nervous tic.
I believe that the following videos really do what the motions are like for those who have these types of jerks.
This is kind of what I have experienced, so I’m posting it. I don’t have issues with my face so much as I do with kind of a rolling to my left and a rocking of my left leg and rolling of my body. It  really seems like I’m jittery or nervous or can’t sit still.  I don’t experience it very often and the periods that I go through are brief. I believe this is a positive sign.
With the following video, the little girl developed this because of scarlet fever, not huntington’s disease. She was able to recover almost completely so the following videos show her before and after:
Another good example. I believe these kind of show the extremes. Some people just seem fidgety. Others are extremely disabled.
The information that I have found has been sparse when it comes to directly attributing these choreic movements to CPM/EPM. However, it has been documented. It may not be an immediate appearing symptoms. In some cases it did not appear until months after CPM/EPM was first diagnosed. I have read that this in not an uncommon theme regarding EPM. It seems that movement disorders with EPM can appear months after the injury. I really noticed my issue develop at a doctor’s appointment. I was becoming extremely agitated, and I realized that my left kept moving as well as my left shoulder. I kept crossing and uncrossing my leg as well as moving in my chair. I’ve noticed those movements at other times of stress.
Thankfully, I don’t think it is getting worse for me.
The following information is a chart that describes that chorea can be caused by electrolyte issues:
J Neurol Neurosurg Psychiatry 1998;65:436-445 doi:10.1136/jnnp.65.4.436

  • Review: Neurology and medicine

Dystonia and chorea in acquired systemic disorders

Table 6

Metabolic aetiologies of dystonia and chorea

Hyperthyroidism
Hypocalcaemia (hypoparathyroidism)
Hypoglycaemia
Hyperglycaemia
Hypernatraemia
Hyponatraemia
Hypomagnesaemia
Osmotic demyelination syndrome (central pontine myelinolysis)
Splenorenal shunt

 

Literature also seems to suggest that these reasons that these choreic movements occur is because of injury the putanem or basal ganglia. It suggests that there is a decreased amount of GABA, and there there are issues with Dopamine and glutamate.

Frankly, folks, I simply can’t read through this very detailed information from the following journal link, but it goes into great explanation why both dystonia and chorea are found in a variety of brain damage injuries, including CPM/EPM, Huntington’s disease, and many others.

Here is the quote:

As discussed earlier, dystonia and chorea most commonly result from striatal dysfunction, and hypoxia-ischaemia has been shown to alter several neurotransmitter systems in the striatum. Glutamate is the main neurotransmitter in cortical neurons projecting to the striatum and may contribute excitotoxic injury. Hypoxia-ischaemia has been shown to increase striatal extracellular glutamate, and decrease glutamate transporter concentrations. Direct lesioning of the globus pallidus with excitatory amino acids in monkeys produces cocontraction of opposing muscle groups on reaching, as in dystonia.9Extracellular dopamine concentrations rise and concentrations of dopamine metabolites fall after hypoxia-ischaemia.710Dopamine may also potentiate the excitotoxic properties of glutamate, and depleting the striatum of dopamine before hypoxia-ischaemia decreases the degree of striatal injury. In the neonatal rat model of cerebral hypoxia-ischaemia, striatal D1 and D2 dopamine receptor numbers fluctuate until 9 to 11 weeks after injury, at which time the D1 receptor number has returned to normal but the reduction in D2 receptors persists.11 Hypoxia-ischaemia also results in areas of complete loss of preproenkephalin mRNA in the dorsal striatum of the rat brain.12 Enkephalin, together with GABA, is an inhibitory neurotransmitter in the projections from the putamen to the external pallidum. Hypoxic-ischaemic necrosis of medium sized spiny striatal neurons may be responsible for decreased concentrations of the inhibitory neurotransmitter, GABA. By contrast, the striatal cholinergic system remains relatively preserved or even upregulated after hypoxia-ischaemia, as evidenced by an increase in cholinergic fibres and cell bodies, and an increase in acetylcholine release.13This is interesting in that anticholinergic medications often ameliorate dystonic movements.

http://jnnp.bmj.com/content/65/4/436.full

J Neurol Neurosurg Psychiatry 1998;65:436-445 doi:10.1136/jnnp.65.4.436

  • Review: Neurology and medicine

Dystonia and chorea in acquired systemic disorders

I will try to add more to this post in the future if I can, but right now, I can’t. Please feel free to leave questions or suggestions as you like.

 

Have a great night 🙂

 

What’s wrong with me: psychological impact of CPM/EPM:

A few days ago I posted regarding how CPM/EPM has impacted my emotional abilities as well as my cognitive abilities. At that time, I didn’t have a lot of information regarding if this is a typical symptom of CPM/EPM.

Now, I have to stress what I’m sure I’ve mentioned previously; CPM/EPM is RARE. Hyponatremia is not rare, but developing CPM/EPM after it does not happen very often.

It is because it is so rare, there is not very much information, especially detailed information or studies that diagnose the symptoms. So, if  you approach a doctor to get answers, you might very well be given a blank stare. Let’s face it, if we had heart disease or cancer, we would get more information as to what to expect, but CPM isn’t widely seen by the medical profession, and even more importantly there aren’t long term studies or follow up of these patients. You’ll also find a lot of discrepancy in the research articles that are written.

I’ve been to several doctors who have never seen a patient with it.

What does this mean for us?

Don’t set high expectations for doctors who treat you, and as I’ve said before with CPM/EPM, ANYTHING GOES. NO one can tell you with absolution what is happening to you or things that have changed after you developed CPM/EPM isn’t normal or typical, because they DON’T KNOW. They really don’t.

I hope that over time, more research will be done for us who suffer from it, but in the mean time, I hope you find that my blog provides the most detailed information on what to expect.

SO, here’s what I found:

There is a link to emotional issues after CPM/EPM. There’s also a very solid link to cognitive issues. I’m also still trying to find links to the impulsiveness.

The following two links provide brief descriptions in their abstracts about having behavioral changes as well as cognitive changes. Now, here’s the thing; these articles require you to pay for access. I am citing their links, but I will only be able to post them after I gain access to them when I go to my local university, which is what I recommend if you don’t want to pay for them. Simply write down the name of the article, the publication date, etc and go to your local or major university library to access them, usually for free.

http://www.ncbi.nlm.nih.gov/pubmed/10514953

The following link provides information on the cognitive deficits a person experienced after developing CPM/EPM (but again to access full article requires payment):

http://www.tandfonline.com/doi/abs/10.1080/13554799808410619#preview

The following research article gives a fantastic description of how the damage occurs, but I will post that under the information regarding hyponatremia and the CPM section that describes how the damage occurs. The following quotes, I’m including gives an example of why I believe articles are pretty vague, but does give a more detailed account of the cognitive symptoms that we may experience:

A more recent study examined 12 individuals with CPEPM related to a variety of medical causes. In this more diverse population, four patients died in the acute  phase, and two were lost to follow-up. The remaining six were reported to have “good motor and cerebellar recovery.” However, all five of the patients who received neuropsychological testing had evidence of subcortical/frontal dysfunction, and most of these (4/5) were unable to return to work.

The next quote also describes another study that was researched:

Almost half (12/25) died either during the acute phase (2) or after hospital discharge (10). One was lost to follow-up. At final follow-up (mean 2.2 years; median: 1 year; range: 0 – 8 years), 29% (7/24) were normal; 17% (4/24) had mild cognitive or extrapyramidal deficits; and 54% (13/24) had a poor outcome (died or were dependent).

To clarify the above study: 2 people died immediately, 10 died after hospital discharge (but it doesn’t say from what); one died but not sure from what; 7 were “normal”, but it doesn’t clarify what that means; and 4 had deficits. Now, if you do the math these numbers don’t add up to 25…so what does that mean? There must be a mistake or error somewhere, and I think that helps to emphasize my point. The research articles on CPM/EPM are vague.

The next quote provides information from this research article on some of the cognitive impairments experienced:

A patient with only EPM (lesions in
the basal ganglia) had severely impaired attention, verbal and visual memory, visuospatial function, frontal
executive function, recognition memory, free recall
memory, and naming, with preservation of other language-related functions.
29
All these deficits are consistent with previous reports in patients with basal ganglia
lesions. In the other case, the patient had CPEPM (lesions in the pons, caudate, lentiform nucleus, thalamus,
and internal capsules).
28
At 1 week, the patient had
prominent deficits in attention and concentration (e.g.,
high distractibility, slow visual scanning), memory (immediate verbal recall and memory for daily events),
visuomotor functioning, and fine motor speed.

The above information really defines what I’ve been experiencing. My lesions were in the basal ganglia, so I have to say it’s pretty accurate in my regards.

The study goes on to explain that there were additional cognitive dysfunctions that occurred after the initial damage occurred and resulted in “pathological crying and laughter at 6 months after symptom onset, all consistent with a brainstem process.”

Doesn’t that sound a bit familiar. I’m not sure exactly what the pathological crying means. I’m guessing they mean it was inappropriate.

THE ABOVE QUOTES COME FROM THIS ARTICLE: http://neuro.psychiatryonline.org/data/Journals/NP/4399/jnp00411000369.pdf

It is very insightful, but I recommend breaking it up into sections because it can become a bit overwhelming.

So this is the information that I have found up to this point, but I’m sure there will be further information to come. There’s so much to go through..dud links…search results that don’t have anything to do with what you want, etc. Consider this post, like all of mine, a work in progress.

I hope it helps, and if you find something, message me with the link so I can add it. I REALLY appreciate your feedback. Truly the only way we can find out what is happening with CPM/EPM is through your feedback of what’s happening to you, so LEAVE comments, and details, etc. You’ll be helping other people!!

UPDATED: 04/20/12….Ok, so folks, so I have been trying to find more references to the psychological impacts of CPM/EPM.  The following link is in reference to a man who developed CPM/EPM after quitting drinking. They performed an MRI that showed lesions in his brain correlating to CPM. His behavior and symptoms progressed, and he began to develop angry outbursts, etc. They performed another MRI that showed demeylination was spreading further in the basal ganglia and the pons.

Two days after the admission, he showed violent behavior, agitation and irritability, getting angry on the slightest provocation without any mental changes or Parkinson symptoms or aggravation of his dysarthria. At first, we considered his symptoms to be alcohol withdrawal psychosis and started antipsychotics to control him, but his symptoms worsened. We performed MRI again 5 days after he developed psychiatric symptoms. The second MRI showed extended lesions in the bilateral basal ganglia and pons, as compared with the previous MRI.

The previous quote and information comes from: http://alcalc.oxfordjournals.org/content/43/6/647.full

This research article states that damage specifically associated with the basal ganglia areas are documented to cause behavioral and cognitive changes:

Abnormality of the basal ganglia is known to cause various cognitive dysfunctions and abnormal behavior via the involvement of the corticostriatothalamic or cortical–subcortical circuit through the basal ganglia (Carlsson,1988), while the role of pontine pathology for cognitive function and personality remains unclear.

UPDATE: 11/14/12

I have found this great research article that sites long term effects of brain injuries. In subsequent posts, I have decided that is safe to draw correlations between all brain injuries, so the following article describes what may happen psychologically after developing a brain injury. I have found that I have experienced a number of these issues, especially with distancing myself emotionally from people. There seems to be an emotional disconnect on a personal level, but I have the ability to cry over anything I experience regarding my brain injury. I don’t have all the answers for what is happening on a psychological level, but the following article does describe a lot:

http://apt.rcpsych.org/content/5/4/250.full.pdf —Psychiatric Sequelae of Acquired Brain Injury-Ken Barrett, APT 1999, 5:250-258

 

I am adding this quote from another research article that I found:

A patient with central pontine myelinolysis (CPM) underwent neurological and mental status examination, as well as neuropsychological testing, during the acute stage of the disease. After correction of the hyponatremia, a gross change in his neuropsychiatric status was observed. The patient underwent extensive neurological, psychiatric, and neuropsychological testing during the acute phase of the disease and at follow-up 4 months later. All major neurological and neuropsychiatric symptoms present at onset were fully reversible. Neuropsychological examination revealed deficits in the domains of attention and concentration, short-term memory and memory consolidation, visual motor and fine motor speeds, and learning ability. Although improved, neuropsychological testing still revealed remarkable deficits at follow-up. We conclude that neuropsychological deficits can accompany CPM, and that these deficits do not necessarily diminish simultaneously with the radiological or clinical neurological findings but may persist for a longer period of time, or even become permanent. In his recovery the patient started to manifest new neurological symptoms consisting of a mild resting tremor of both hands and slow choreoathetotic movements of the trunk and the head, which we considered to be late neurological sequelae of CPM. The significance of CPM in the differential diagnosis of acute behavioral changes after correction of hyponatremia is stressed, even if correction is achieved slowly and carefully.

This really explains the problems that I’ve experienced, and even mentions that you can have late onset symptoms related to CPM/EPM. The above quote comes from http://www.ncbi.nlm.nih.gov/pubmed/10514953

 

02/17/2012

This week I had yet another appointment, and this appointment has stuck with me the rest of this week.

First, I want to apologize for not posting more recently than this. If you have CPM/EPM, you will find that your life seems to be full of the unexpected. You will find that there are days that seem normal and days that you wonder how will you be able to live the rest of your life in this manner.

It’s exhausting. It’s frustrating. It’s unfair.

It seems like everyday is chaotic and for someone who used to be so focused and moderately organized, this is driving me freaking crazy!!!

IT’S STRESSFUL!

SO, now I’m starting yet another category for my blog, but I still have to go back and add to hyponatremia, to CPM/EPM, and to my story!!! However, what I experienced this week needs to be addressed before I forget it, and it has really consumed me, so I feel I have to write about it.

C’est la vie!

This week I traveled 4 hours to meet with a neurologist who is a movement disorder specialist. I also had a MRI and something else….what was it. For real!! Another five minutes gone to trying to figure out what it was that I actually did while I was there. I only had three appointments. Oh, yes, the skin biopsy.

I thought I was going to go to the neurologist to get a TREATMENT for my tremors, jerks, shakes, twitches, spasms, etc. In other words, my neurological problems with movement.

I guess this is where I made my mistake. I had already met with a neurologist who is treating me for EPM, and she was sending me to get treatment for my EPM movement issues. She told me that she was sending me to a movement specialist neurologist for this purpose. I assumed that this was going to be the reason for the appointment: I was going to get medicine for my neurological issues related to EPM.

If I thought that I was going to be examined to determine on whether or not my movement issues were related to EPM, I would have been more prepared. I would have brought materials on EPM.

Here’s the thing: EPM IS RARE. CPM IS RARE. MOST DOCTORS HAVE NEVER TREATED A PATIENT WITH THIS INJURY, and it’s not that they are stupid or trying to be judgmental, they are purely ignorant!

This doctor was the same way.

I had no idea what the true intention for this appointment was, and this set me up for disaster.

This doctor did a complete neurological exam. He was pretty thorough.

After the examination, he told me: Well, you have an essential tremor and it is not related to your EPM. It’s fairly mild, but I can give you medication to treat it. I would also like you to test for Wilson’s disease. You don’t have any of the symptoms for Wilson’s, but it is a cause for tremors in a person who is younger. I don’t think you have it, but we’ll do the test as a precaution.

Before, having EPM, I would have just nodded my head and left. I would have spent the rest of the day biting my lip and waiting to say the things I wanted to say.

I don’t know what would have been better. I really don’t.

I literally started arguing with the doctor. He told me that because my MRI images were normal that the tremors weren’t caused by EPM.

Ok, folks, you know I’ve done research. I’ve spent the past 8 MONTHS researching this injury, and my first question to the doctor was: how many patients have you treated with this? His answer: ONE!!!

I then went on to say: My MRI still shows the injury (and it does). This is what my other neurologist has told me. However, if you were more familiar with EPM/CPM, you would understand that there is no correlation to the findings on an MRI and a person’s symptoms.

The doctor didn’t cotton to my pointing this out to him.

We literally started to ARGUE.

He basically told me that he wasn’t going to have me lecture him on this, but then I explained to him that I wasn’t pulling this information from WebMD. My information comes from credible medical research documents, and that I was preparing to go to medical school.

He warmed up a little bit at that point.

He tried to explain to me that the ONE patient that he’s treated with this disorder had the Parkinson’s like tremor that is associated with EPM/CPM. He told me that he had a video that he took on that patient. This particular person had both significant injury to the pontine region as well as the extra pontine regions AND that this person’s MRI still showed the injury.

He believed that my movement issues have nothing to do with EPM. He thought they were random.

He told me that I did not have Parkinson’s, and I did not have a Parkinson’s tremor.

Okay, so what’s wrong with what he was telling me, and how could I have better handled it? How could have this appointment gone better? What should I have done?

First, I should have been prepared. Really. I’ve been to hundreds of appointments. When you have something rare or not clearly understood, you need to come ready for everything. You need to have any research that you’ve found regarding your disorder. Make copies of your labs, of your reports, of research that you have found.

I had no idea what a Parkinson’s tremor was and how it was different from the tremors that I have. I will make a separate post on tremors and how they differ. Of course, with everything that is medical, there is disagreements on what is and isn’t a Parkinson’s tremor.

Basically, if you have a tremor or movements that impact one side of the body (at least in the beginning stages) that are present when you ARE NOT moving, they suspect Parkinson’s.

The tremor that the movement neurologist suspected is something called essential tremor. This type of tremor is usually found in both sides of the body. It generally becomes worse when you are moving. For instance, if you are trying to get food to your mouth or trying to get a cup to your mouth, but your hands shake so severely that your food falls off your fork or you spill liquids from your cup, they suspect essential tremors.

Now, I had no CLUE what the difference was. I had no idea that there was a difference. All I knew was that this issue became extreme when I developed EPM.

If I was prepared for this appointment, then I would have been able to produce information regarding my tremors. I would have also been able to represent the different types of tremors that are associated with EPM.

I did not know until after the appointment that this doctor really did not know what he was talking about: EPM/ CPM can have both, either, or neither…Parkinson-like or bilateral tremors.

In other words, my “essential” tremors, are probably caused by the EPM.

Furthermore, the doctor told me that there were no reasons to think that these tremors would not be long lasting if they were indeed caused by EPM. He believed that because the MRI images were improving then my symptoms would also improve.

I can not say whether or not this is true. I would have to point out to anyone who states that because your MRI images get better does NOT mean that your symptoms will improve. Further, if your MRI images DO NOT improve, that does NOT mean that you will not improve. The MRI, at this time, WILL NOT show anything more than that you had this injury.

The doctor also tried to state that symptoms will NOT get worse after the injury has happened. THIS IS TOTALLY NOT TRUE. DOCTORS DO NOT KNOW WHAT WILL HAPPEN WITH CPM/EPM!!! They do NOT KNOW.  People do see a progression in their symptoms even AFTER 8 and 9 months.

There has not been enough research in this area to know with any certainty what will happen. I know of 4 individuals with CPM/EPM that had improvements, but after a period of 1 to 2 years, their symptoms worsened. I really believe that this is related to just normal aging.

I would compare it to my cell phone that I dropped in the toilet. (It was an unused toilet at the time). I made the mistake of turning it on as soon as I fished it out. Now, anyone who is familiar with electronics knows this was a mistake. It basically fried it. However, I did not want to go out and buy a new phone. So, I took it apart. I sprayed it down with electronic cleaner. I let completely dry out and put it back together. IT WORKED! 🙂 BUT, there were certain keys that did not work, the pound and star button. I was perfectly fine with that because I didn’t really use those buttons that much any way.

So, I was happy, but several months later, other keys started not working properly. Some numbers would repeat a dozen times when I pushed them once. Sometimes, letters wouldn’t show up.

And this is my point, truly the brain works in a fairly similar way. We don’t understand how it works completely. Medicine is really archaic in this field. However, we know as we age the body breaks down. They don’t work as well. This is true for the brain. So, if you already have an injury in the brain, yes, you are likely to see improvements, but it’s like my cell phone, you just don’t know how long those improvements will last, and you are most likely to see these areas degrade over time as your brain ages.

Getting back to my appointment:

I tried to explain that the delay in new symptoms is believed to be caused NOT BY CPM/EPM INJURY directly, BUT BECAUSE YOUR BRAIN TRIES TO REBUILD CONNECTIONS, and it is believed that these new pathways can cause the new symptoms.

Therefore, people have seen NEW symptoms months and EVEN years after the injury. The doctor I saw agree with this, but he felt that new symptoms would not be seen after 1 or 2 months after injury.

See this article for a description on how this isn’t true:

Journal of Clinical Neuroscience 19 (2012) 179–180

And this one:

Journal of  Neurology (1995) 242:450-454
© Springer-Verlag 1995

Regarding the types of tremors that are experienced in CPM/EPM injuries, most are considered Parkinson’s like, resting tremors; however there are also studies that show that both types of tremors can be present, parkinson’s and tremors that worsen with movements.

Here is information from Wikipedia http://en.wikipedia.org/wiki/Central_pontine_myelinolysis:

 Permanent disabilities range from minor tremors and ataxia to signs of severe brain damage, such as spastic quadriparesis andlocked-in syndrome.[14]

Okay, the following is what I have. I do have a bilateral resting tremor that gets worse with movement:

A 56-year-old man developed drooling and bilateral hand tremors 3 weeks after correction of hyponatremia from 103 to 125 mmol/L over 14 h. He had a prominent 6 Hz resting tremor which worsened with action and mild cogwheel rigidity. Magnetic resonance imaging (MRI) showed changes consistent with central pontine myelinolysis and increased signal on T1-weighted images in the putamen bilaterally. His tremor responded well to L-dopa therapy.

(http://www.ncbi.nlm.nih.gov/pubmed/10833626)

I could go on all day quoting journals regarding tremors and EPM/CPM. Here’s another:

Rigidity was present in all four limbs, with orofacial dystonia and dystonic posture of hands and feet and with tremor in both hands.

http://dmc.academia.edu/MahmudurSiddiqui/Papers/893372/Selective_Extrapontine_Myelinolysis_in_Osmotic_Demyelination_Syndrome_in_a_Case_of_Previously_Undiagnosed_Sheehans_Syndrome_with_Recurrent_Hyponatraemia_-_A_Rare_Association

If I had been better prepared, I would have been able to bring these things to his attention.

More importantly, I showed him two videos of my tremors. He believed these videos demonstrated that I indeed have essential tremors. And I cannot disagree with this statement. I don’t know. I’m not an expert in tremors.

That said, I only recently started recording my tremors, and more importantly, I have spasms and jerks in certain fingers, in my legs, or feet, but these jerks are fleeting. So, I will have three or four twitches in my finger and there’s no way that I can record those particular movements without making a video diary of my every waking moment. I never know when these movements will occur.

This is extremely frustrating because by this point in my appointment, there was no trying to communicate with the doctor. I was too emotionally involved and so was he.

His take away message was: you’re going to be fine. You aren’t going to get worse. You are going to get better. You should try this medication to help with the essential tremor that you have, and I am almost positive that your tremors are not related to EPM. Even if they are, the medicine should help them. You do not have Parkinson’s.

Now, this seemed to be a crux in the conversation that I haven’t hit upon previously. He kept stressing that I did not have Parkinson’s. He stated that he was an expert for Parkinson’s.

I really did not understand why he kept bringing up Parkinson’s disease. I DO NOT HAVE PARKINSON’s. I did not think that I had Parkinson’s. I believe that I have Parkinsonism. Now, I’ve previously discussed Parkinsonism. From the knowledge that I have, it is any tremor that a person has, along with dystonia, and dyskinesia, and possible issues with your voice. I am not an expert on this. I know what I’ve read, and I promise to research this further and try to post on this more when I post specifically on tremors.

What’s wrong with his message:

HE HAS ONE FREAKING PATIENT WHO HAS HAD CPM/EPM. He certainly hasn’t spent the past 8 months researching every possible thing you can find on it.

He made incorrect comparison’s: Parkinson’s and EPM. People with EPM/CPM DO NOT HAVE PARKINSON’s. They have a Parkinson’s like tremor, and that isn’t even true for everyone with EPM/CPM. He did not understand that there IS NO SET STANDARD for CPM/EPM. It’s like saying someone who has colon cancer will have the same symptoms and issues as someone with esophageal cancer. It’s like saying there is only one cause for heart disease.

He was unwilling to say: I DON’T KNOW. I will need to evaluate you further. Please spend time making more videos of your issues and feel free to contact me when your symptoms change or if you have a video of something different. He didn’t even ask me when I took the videos I made.

I tried to explain that my symptoms vary in severity from day to day. Truthfully, they vary from hour to hour. Right now, I barely have any tremor at all. However, an hour ago, I did. I have twitches and spasms even at night when I’m trying to go to sleep, at rest. They make it difficult to fall asleep. I don’t have this problem EVERY night, but probably three or four times a week.

So, what could I have done?

I should have made a journal of these things. I should be keeping a daily record of my symptoms, the times, places, how much caffeine have I consumed, etc. Doctors really like data. They like it when you have detailed information for them to look at.

I really haven’t documented my symptoms and when I have them. I should be keeping more videos. I feel embarrassed to pull out my cell phone or video recorder to make these videos in public places when I’m experiencing these problems. I believe that those localized spasms that I get in my fingers or feet don’t last long, so I shouldn’t bother trying to record them.

I AM MY ONLY ADVOCATE. NO ONE ELSE IS GOING TO DO IT. You really need to take this to heart if you are reading my posts.

No one else is going to go to bat for you, and if you are willing and able, you need to make as many records as you can regarding your situation. It can and will help you out. It might help your doctor more fully understand your situation. More importantly, you can post it here on my blog and help others with CPM/EPM.

I also shouldn’t have bothered correcting this doctor when I didn’t have access to the medical journals or very good videos of my symptoms. Now, this doctor has formerly based his opinion. He isn’t going to bother to do anything else with me. If I ever need to go back to him, I will have to have a huge collection of evidence proving him wrong.

Let me stress, it’s not important to prove him wrong for the sake of being right, but because I will not get help from him unless that happens. I also won’t get the correct medications for the types of neurological issues that I have.

It’s also important to realize that it’s important to realize when you’ve lost a battle. It’s your choice on whether or not you are going to waste your precious time and health on trying to bring this person up to speed on CPM/EPM.

As more and more records become easier to access, hospitals will become able to share information on your medical history. This means that this doctor may be able to pollute the opinions of future doctors that I see, even at different hospitals, in different cities or states.

So, you really need to make a decision on whether or not you are going to spend your time and resources on “educating” a doctor on your condition.

If you choose to, I think it’s really important to “educate” him on the first appointment. First impressions make the biggest impact, and this is most certainly true for your doctors.

In other words, you need to be prepared for EVERYTHING on that first appointment. You have to have research articles on the types of symptoms and issues that you have, especially when those symptoms are rare. You need to have videos and if at all possible, personal testimony from family and friends (hopefully someone can go with you for your appointment that can attest to your issues and their severity). It’s great if you have a journal of your symptoms from each day.

You need to ask your doctors questions like: how many patients have you treated or seen with CPM/EPM? And it’s important for you to make a decision on whether or not you are going to continue to see this person if the appointment does not go well. Be prepared. Don’t be afraid to ask questions.

I’m sorry if this post was a bit repetitive. I believe that when I have something that I feel especially strongly about, I tend to repeat it. I will try to edit in the future for those type of repetitions.

For now, I’m going to retire. and as always, please feel free to write me with any questions or your personal story.

CPM: Treatments

I hope you are doing well. It has been several days since I posted last. I could list several crazy reasons: I was in the hospital having a sleep study. They didn’t have WiFi at the hospital, so I couldn’t use my laptop. Tom was sick. However, honestly, the biggest reason is I didn’t know how to continue with this topic.

It might seem obvious that it is easy for me to get distracted and get off topic. (I hope not). I think my last post on CPM/EPM might have demonstrated this a little more than normal.

I covered a lot of material in my last post. Several of the topics I mentioned, I feel, could be made in to separate posts. I might try to do this at a later time, which means that I might have some topics come up more than once. Please be patient.

Okay, so CPM/EPM treatments:

There really aren’t any treatments, as far as a cure. You will find this information on any resource regarding CPM. For your convince, I’ve included this quote from, http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001779/.

There is no known cure for central pontine myelinolysis. Treatment is focused on relieving symptoms.

The following quote is from a study that suggests the following has been used in the treatment of it:

Case reports have suggested that steroids, intravenous immunoglobulin, and thyrotropin-releasing hormone may be helpful; however, there are no findings from a large-scale trial to support the use of these therapies.

(http://radiographics.rsna.org/content/29/3/933.full)

You may be given prescriptions for movement disorders (tremors, shakes, twitches). These are usually the same type of drugs that treat parkinsons. You may be given pain meds. You might need to see a pain management specialist. You may need anti anxiety/anti depressants. You may need medicines for insomnia or for central nervous system sleep apnea.

You may need on going physical and occupational therapy. You will probably need speech therapy.

You will probably be given anti-depressants or anti- anxiety medicines because let’s face it, the pill you are forced to swallow is unbelievably bitter.

I know that sounds a bit scary. It is. It is terrifying because the doctors have no way to know what is going to happen to you, so they won’t be able to provide you with much information.

If they’re honest with you, they will tell you that you could slip into a coma at any point in time, die, or slip into something called locked in syndrome.

In a series of 44 patients, myelinolysis
occurred after a mean of 6.3 days (range 3–11)
and resulted in a “locked-in” syndrome in 23
patients.

The above information was provided by: http://www.ccjm.org/content/74/5/377.full.pdf

Personally, I think the locked in syndrome is the most terrifying because you will lose all ability to move. It’s a FULL body paralysis. The only thing that you will be able to move is your eyes, but you’re aware of what is happening.

Some studies state that you are at risk to develop these severe health issues (coma, death or locked in syndrome) up to 12 weeks after developing CPM/EPM. Other studies, suggest that it is up to 8 weeks. Frustratingly, there is not enough information regarding CPM/EPM to know for sure.

In most cases, the hospital will keep you under observation for at least 7 days depending on how severe your symptoms are. In other cases, you may be hospitalized for up to 21 days for observation. If you go into a coma or locked in syndrome, you may be hospitalized for 4 to 12 weeks, if not longer.

Here’s the thing, if you are being released in this 7 to 21 day period, I highly recommend that you remain in contact with your doctors that were monitoring your for the CPM/EPM. If you experience ANY changes after being discharged, GO TO THE ER. Err on the side of caution with CPM/EPM. Being wrong is better than being dead.

If you’ve developed CPM/EPM, you’re already unlucky. You’ve already fallen into the less than 1% to 5% range by developing it, and NO ONE really knows what will happen, so err on the side of caution if you experience ANYTHING that gives you concern.

I want to stress that it’s important to return to the hospital that was treating you because most hospitals have never treated a patient with CPM/EPM. Most doctors have only read about it in textbooks. If your hospital treated you, then they might have experience with it.

Your life has just been changed tremendously. If you’re reading this, then you are EXTREMELY luck and terribly unfortunate at the same time. You are terribly unfortunate in developing CPM, but EXTREMELY lucky that you are even alive.

It’s going to take time to adjust to your new abilities, and your journey is just beginning.

Now, here’s the thing. No ONE knows what is going to really happen with you.

Depending on the severity of your symptoms, you may improve significantly. Some research suggests that you may recover COMPLETELY.  However, I question this on the basis that research is vague.

In long-term
follow-up of 32 survivors of the acute phase of
central pontine myelinolysis, 11 had no functional deficit, 11 had minor neurologic
deficits, and 10 had severe deficits requiring
dependent (ie, long-term) care.

The above quote comes from the article used previously. Use the ccjm.org link above to access it.

The widely used study above suggests, that 1/3 of patients will recover, a 1/3 of patients will have symptoms but live independently, and a 1/3 will need to have assisted living.

This study was vague. It didn’t say how long the patients had CPM. Was it 3 months, 6 months, 10 years after developing it?

I would like to suggest that unless you die, you are going to improve. I would like to suggest that everyone who has CPM (except for those who die) will improve to some degree to an almost normal, pre-injury state. However, it is not known if this type of improvement is absolute or to what degree you will improve.

The study quoted above, also explained that depending upon the location of the lesions, a person might experience a decline in neurological abilities. I know several persons who have it, and after a period 2 to 3 years, they begin to experience a deterioration in their neurological symptoms.

Some studies have stated that the majority of persons who have CPM, die within 5 to 10 years after developing. More than half commit suicide.

Please be aware, if you are a care giver for someone who has CPM, that more than half commit suicide.

Because of this alarming statistic, I highly recommend getting your loved one supportive psychological therapy. I also recommend that they participate in online support groups.

Brain injury support groups offer a great help.

I looked for months, and found only a handful of people who have CPM through inspire.com.  We’re kind of outcasts since there are so few of us, so we don’t really fit into any other neurological support groups.

I hope to change that at some point in time, but right now, please contact me with questions. Please post your story for others to read. Together, I hope we can prevent people from going through this horrible experience.

 

UPDATE:

It is always depressing to learn that there are TREATMENTS for CPM/EPM, and to know that I could have been fixed if I received one of these treatments! I hope and pray that if you have CPM/EPM or know of someone who has been diagnosed that you will get this information during a period when it can provide relief. The following quote comes from an abstract, so if you provide this information to the doctor, they should be able to get the treatment information:

Clin Neuropharmacol. ;23 (2):110-3  10803802  Cit:11

go to Pubmedgo to Scholargo to Googleshow EndNote Citationshow BibTex Citation

Update citations of this paper

        Neurological Department, Neurological Hospital Rosenhügel, Vienna, Austria.
Although the exact pathogenesis of central pontine myelinolysis (CPM) is unknown, correction of hyponatremia, thyreotropin releasing hormone, plasmapheresis, and corticosteroids seem to be effective. Assuming intravenous immunoglobulins (IVIG) to also be effective in CPM, 0.4 g/kg body weight/d immunoglobulins were applied to a 48-year-old patient who developed CPM with double vision, dysarthria, dysphagia, and left-sided hemiparesis 3 weeks after spontaneous normalization of hyponatremia. After 5 days of IVIG, his symptoms markedly improved, confirmed by improvement in the Norris score (42%), Frenchay score (19%), Kurtzke score (20%), Disability score (54%), vital capacity (26%), and peak torque (69%). The promising clinical effect of IVIG was assumed to be caused by the reduction of myelinotoxic substances, the development of antimyelin antibodies, and the promotion of remyelination. In conclusion, IVIG appear to be a promising therapeutic option in CPM.

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