Central Pontine Myelinolysis

Central pontine mylineolysis

I was given the request last night to scan this womans brain. She was a young lady with a history of alcohol abuse and seizures. I talked to her over the phone to make sure we could get a good medical history before the MRI scan. Her speech was very slurred and she told me she had difficulty swallowing. To my supprise she said she had been in the hospital for 2 weeks, with these symptoms for 3 weeks. I imediatelly thought she infarcted her pons becuase she had no physical weakness in her arms or legs. I figured possible lacunar infarct. To my suprise she was Diagnosed with Central Pontine Mylineolysis from the MRI scan by our Radiologist. This was the first time I had heard of this . I learn Something new everyday. The following is Information on the topic:

Adams et al described central pontine myelinolysis (CPM) as a unique clinical entity. They published their findings in 1958, observing that patients who suffered from alcoholism or malnutrition developed spastic quadriplegia, pseudobulbar palsy, and varying degrees of encephalopathy or coma from acute, noninflammatory demyelination that centered within the basis pontis.

physicians recognize that CPM occurs inconsistently as a complication of severe and prolonged hyponatremia, particularly when corrected too rapidly. Standard of care requires judicious treatment of electrolyte disturbances to reduce the incidence of osmotic myelinolysis.

abnormal normal

Central pontine myelinolysis is a neurologic disease caused by severe damage of the myelin sheath of nerve cells in the brainstem, more precisely in the area termed the pons. The most common cause is the rapid correction of low blood sodium levels (hyponatremia). Some scholars postulate that the real cause might be the lack of a substance that is essential for brain activity and is lacking due to malnutrition. The fact that this condition is most frequently observed in patients with general ill health (alcoholism, cachexia etc.) is in accordance with this assumption. Imaging by MRI demonstrates an area of high signal return on T2 weighted images. Frequently observed symptoms in this disorder are weakness, double vision and loss of consciousness. To avoid myelinolysis, the correction of hyponatremia should not exceed 1 mEq/L per hour.

The rapid rise in sodium concentration is accompanied by the movement of small molecules and pulls water from brain cells. Through a mechanism that is only partly understood, the shift in water and brain molecules leads to the destruction of myelin, a substance that surrounds and protects nerve fibers. Nerve cells (neurons) can also be damaged. Certain areas of the brain are particularly susceptible to myelinolysis, especially the part of the brainstem called the pons. Some individuals will also have damage in other areas of the brain, which is called extrapontine myelinolysis (EPM). Experts estimate that 10 percent of those with CPM will also have areas of EPM.

The ideal treatment for myelinolysis is to prevent the disorder by identifying individuals at risk and following careful guidelines for evaluation and correction of hyponatremia. These guidelines aim to safely restore the serum sodium level, while protecting the brain. For those who have hyponatremia for at least 2 days, or for whom the duration is not known, the rate of rise in the serum sodium concentration should be kept below 10 mmol/L during any 24-hour period, if possible

The prognosis for myelinolysis is variable. Some individuals die and others recover completely. Although the disorder was originally considered to have a mortality rate of 50 percent or more, improved imaging techniques and early diagnosis have led to a better prognosis for many people. Most individuals improve gradually, but still continue to have challenges with speech, walking, emotional ups and downs, and forgetfulness.
What research is being done? (source NINDS)

This syndrome is characterized by a gradual neurologic deterioration developing one to several days after complete or partial correction of chronic hyponatremia. Neurologic deterioration may often be preceded by a transient improvement paralling the correction of electrolyte disturbance. Fluctuating levels of conciousness, convulsions, hypoventilation or hypotension may herald the onset of this syndrome. Eventually pseudobulbar palsy and quariparesis develops. Swallowing dysfunction (often with episodes of aspiration) and inability to speak may be dominant features. In severe cases, the patient may develop a “locked-in syndrome” – they are awake but unable to move or communicate. Marked improvement may occur presumably because of reyelination. Although various imaging modalities may be confirmatory, positive diagnostic tests are not essential to diagnose the osmotic demyelination syndrome. If psedubulbar palsy and limb paralysis gradually develop after hyponatremia is corrected, the clinician should strongly suspect a demelinating lesion of the pons. (source)

MRI IMAGING of Potine Myelinolysis

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18 thoughts on “Central Pontine Myelinolysis

  1. Before going any further, my appeciation goes directly to the Blogger as its taken me almost four years since my “Official Diagnosis of Central Pontine Myelinolysis,” to drive the awareness to where its presently at today. From the Senate Offices in Washington, D.C. to several National Organizational Health Conferences to my work starting with the NIH/NINDS who was kind enough to recently add CPM to their Information Pages as seen in this blog to become as they call me at the ICSCI at Kennedy Krieger and Johns Hopkins, the CPM Survivor who now is the “Number One CPM Layman Advocate in the World.”I hope whoever is kind enough to read this wonderful blog and comment will mention a few words to your associates in regard to CPM. CPM in known as an “Iatrogenic” Acquired Toxic Neuromuscular disease that has had the unforseen ability to fall through the cracks since Dr. R.D. Adams and Associates brought this white matter disease to the forefront in 1959, almost 50 (FIFTY) Years ago. As unknown knowledge, I have started the “CPM Awareness Foundation.” We who have been bless to communicate with one another (Sometimes with the help of family or friends.) still live on in the many symptoms and complications of this horrific chronic illness with the hope and dream that we have finally been able to break free to the public. It is stated in most informational pieces that there is “No Cure,” for CPM which is partially true, however the actual “Cure” rides with the Awareness and Training that is so badly need. My prayer is that YOU don’t have to find out the way we, the CPM Survivors have. G-d Bless…

  2. TO: CoolMRIstuff and those who may have an interest in “Cental Pontine Myelinolysis.”

    We, (those alive and who have passed including families of) CPM victims who continue to communicate with each other truly appreciate any and all possible awareness that is definitely being given to this rare but “Not as rare as many are told really appreciate the assistence give to us. In fact, this perticular Blog by CoolMRIstuff has been given great reviews from those all over the world. As you will note, many CPM victims lose life within thirty days post it’s diagnosis. Personaly, it will be seven years I’m blessed not only to have life but also to have the needed qualifications to continue my own personal research for CPM. Trust me I have my own severe issues that constantly come and go.

    Look forward to all who may have a chance to add to all the “Central Pontine Myelinolysis Awareness.” In closing, please note that the CPM 50 Year Anniversary since it’s initial naming and available info started will be this coming year of 2009. It’s Author, Dr. Raymond D. Adams (currently 97 years old) lives here in the US inthe New England area near Boston I believe. I’ve had the opportunity to chat a few times with his daughter. As CoolMRIstuff listed NIH/NINDS as a source, you will notice how pleased I was to be at the NORD Conference and was able to bring this info to NINDS.

    Both Franky (my Boston Terrier neuro service companion) and I wish you all a wonderful Holiday Season and G-d Bless…

    Thanks for your kind words.

  3. I heard of this rare brain disorder due to my husband death almost 5 years ago. After 36 years of marrige I found myself in shock. He had lower back surgery which went wrong, he had a hard time walking, balance caused many terrible falls where he hit his head many times. In the 42 days he was in the hospital no one had answers. I had gone to the hospital cafeteria for lunch, when I left Ted, he was talking normally. He was the one who said I should go to lunch. I came back in about 15 to 20 minutes and he was gone. They had put him on life support. In the 42 days they did not do a CAT scan on his brain until he died then I saw the medical records where they did a scan on his brain after he died and the head doctor on his case said in the records “Oh my God he has pontine”. I had no idea what it was I did not find out what it was after the coroner finally sent the death certificate which I received about 6 months after I had to make the call to unplug him from life support. I’m sorry I wrote so much just hard to have so much happen with no family left.

    ______________________
    Please don’t be Sorry,

    It is hard to deal with such loss. I wish you peace.

    Chris

  4. Pingback: NEJM Central pontine myelinolysis « Coolmristuff

  5. I am sooooooooo pleased to be able to watch such wonderful individuals who have been able to take it among themselves to assist me and all those Central Pontine Myelinolysis victims as well as those families of my CPM associates that try to help each other. You all may have seen my self made organization called the, “Central Pontine Myelinolysis Awareness Foundation. I who always use the Username of WhataBreeze along with the very first Canine Neurological Service Companion, now a five year old Boston Terrier named Franky (male whose birthday yesterday, January 9th, 2009) I’ll let the cat out of the bag when I tell you my personal research on cpm has been going for 7 years, “Yes, Seven Years.” I have not had one day in those 7 years that I’ve had 24 hours when going without feeling ill. My life with Franky started in 2004 when he was 10 weeks old. I’m hoping to make actual first hand communication with I believe is Chris.(coolmristuff) When 50 to 75% of those who acquire CPM which cost their life in 30 days, have the chance or opportunity to live on with all facilities in different patterns of loss they have been blessed as I have.

    Please make contact with me as your kindness and my facts in regard to CPM will eventually be a major positive in lifes meaning. G-d Bless and please show me where these donations need to be sent. I wish I had the knowledge that these Radiology Specialist have already forgot. You are sooooooooo important to all of us with CPM.

    Franky and WhataBreeze (~8

    FYI: My Tremors Make Me Stop At This Point.

  6. My father has CPM. He was a very healthy 65 yr old man who had high blood pressure, high cholesterol, and hypothryoidism. He sodium level was 102 when he went to the ER on March 30, 2009. He has been in the hospital ever since. He has delirium. He can feed himself, walk, walk up and down the stairs, and they are working on getting him used to go to the bathroom again. He was speaking very clearly almost a week ago today and now it is just jibber and then he was say a few words together. I know that he will probably have his good days and then his bad days. He is having physical therapy everyday, speech and occupational therapy five days a week. I am just inquiring if your family members had recovery from this condition and were eventually able to come home? I would appreciate any input anyone could give me and my family.

  7. Hi

    My father is diaognised with CPM. He is at home, he can walk , talk little bit , has emotional ups and downs and has blackness in front of his eyes.

    His doctor says he is ot of danger and recovery will be slow. Can someone please advise if this is true as all articles say intial 30 days is crucial?

    Your help will be much appreciated.

    thanks

    • I am the above. I can be reached at 314 – 997 -3749.

      Jeffrey M. Amitin

      Note: I am not a physician, however I’m a patient of CPM and started the first CPM Awareness Foundation. G-d Bless…

  8. MYELINOLYSIS: 8+ hours perfomance in their operative suite.

    hey chris, wanted to say hi and just learned my new bud’s of ataxia just decided to pop out (maybe to add to the surreal look for this MRI. Hard to fathom the defense thoughts that this all dropped out of the sky as I hurried myself for positioning myself to make the catch. oh yeah I forgot to tell you this was one of them large med schools 4 teaching. Pssssst… they rank in the top 10. I atleast got a real chuckle when learning the true numbers as if I could of made it two floors below as the initial report done by a few of the neuro’s had it laid out there for trusty house crew. Would be good to chat ear 2 ear.

  9. WHATA BREEZE , I REMEMBER YOU FROM THE NEUROLOGICAL SITE I USE TO VISIT MY EMAIL NAME WAS BONSIE ,I HAVE HAD CPM SINCE 2001 .I CANNOT WALK ,TALK, OR WRITE . I HAVE HAD MANY BOUTS WITH ASPIRATION PNEUMONIA ,BECAUSE OF THE DIFFICULTY WITH SWOLLOWING ..

  10. To anyone who was associated with Jeffrey (what a breeze), he has succumbed to CPM in 09/2011. I had the pleasure of his assistance with CPM when i contracted it myself in June of 2011. I have started a blog myself through wordpress, whatishyponatremia.org. I am trying to spread the word regarding hyponatremia and Cpm/EPM. I really believe that everyone should be familiar with hyponatremia which is the most common metabolic disorder in the united states, as well as CPM which has an incidence of at least 2000/ year.

    Chris, I would love your input, if possible, regarding the information that I have posted on hyponatremia and CPM. It is a work in progress, but I really believe awareness is key to prevention. If you have any information regarding life expectancy for CPM/EPM victims, please let me know.

    To anyone who is searching for support with CPM/EPM, please visit my blog. I also recommend inspire.com. you can search CPM/EPM and find out other survivors.

  11. I’m sorry to hear about Jeffery. He was a true advocate for cpm. Your site is great!! Awareness and education are the key here. Keep spreading the word.

  12. I am a 35 year old male who suffered this condition I progressed from locked-in to living alone but still have some issues. I would like to help study this. I just want it to be more understood.

  13. My sister nw 22 yr old .she is suffering from this condition. She is having severe condition she is suffer from last 4 year please help us gave some suggestion

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