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Osmotic Demyelination Syndrome

osmotic demyelination syndrome

Myelin is a special substance that covers neurons and serves as an electrically insulating sheath, or rather a group of substances since the composition of myelin varies in different parts of the body, but lipids and proteins are always the basis with a significant predominance of the former.

The destruction of the myelin sheath leads to disruption of normal electrochemical reactions and connections. Demyelinating diseases (multiple sclerosis, amyotrophy, etc.), in which, due to autoimmune or hereditary factors, the disintegration of the neuronal membranes begins, are severe and are manifested by gross imbalances in balance and coordination, progressive loss of memory, speech, etc.

Osmotic syndrome (“central pontine myelinolysis”) is a rare disease caused (among other reasons) by electrolyte imbalance, primarily sodium. Synonymous names are associated with the predominant localization of the process: it is mainly affected by the Varoliev bridge – one of the posterior parts of the brain. The disease can manifest itself at any age.

The first description of this syndrome by Adams et al. in 1959 (then it was called central pontine myelinolysis) was carried out on a population of patients with chronic alcoholism; the latter is the most common cause. Since then, other causes of ODS associated with the rapid correction of electrolyte disturbances have been identified:

  • chronic alcoholism;
  • chronic exhaustion;
  • transplant recipients.

Causes and symptoms of central pontine myelinolysis

Causes and symptoms of central pontine myelinolysis

Causes

Osmotic demyelination syndrome is a polyetiological pathology, and several factors are involved in its development. It should be admitted that not all of them are currently known and sufficiently studied, but the role of sodium ions has been proven. 

  1. The syndrome was originally discovered and described for cases of severe sodium deficiency in the blood (hyponatremia): intense and too rapid correction can lead to the destruction of myelin, for example, by the administration of a hypertonic solution.
  2. Later, a connection was established between osmotic demyelination with: 
  • alcoholism; 
  • exhaustion; 
  • dehydration; 
  • various eating disorders (anorexia, bulimia); 
  • renal or hepatic failure;
  • systemic autoimmune and endocrine diseases;
  • viral infections, etc.

Symptoms and differential diagnosis

The clinical picture of osmotic demyelination syndrome is so dependent on individual conditions and factors that it is almost impossible to identify a typical symptom complex. 

  • In different cases, paresis and paralysis are noted (up to pseudocoma, when motor activity is completely blocked); 
  • the phenomenon of epilepsy and parkinsonism;
  • visual impairment (for example, field loss);
  • coordination of movements;
  • speech;
  • the psyche. 

Some authors emphasize the paradoxical independence of the clinical severity of the syndrome (and the level of mortality) from the scale of demyelination.

The main method for differential diagnosis of osmotic demyelination syndrome is tomographic imaging – computed x-ray or magnetic resonance imaging. MRI in this case is considered a more informative method in comparison with CT. Areas of reduced density are identified, and they can remain for some time after the patient’s clinical recovery.

Clinical presentation of osmotic demyelination syndrome

Clinically, osmotic demyelination syndrome occurs in two phases. The first phase is the onset of electrolyte disturbances, while encephalopathy is observed in patients. After a quick correction of these disorders, the patient becomes better for a while, however, after 2-3 days, there is a progression to classic ODS. With a lesion of the bridge, the main clinical signs include:

  • spastic tetraparesis;
  • pseudobulbar syndrome;
  • change in the level of consciousness;
  • paresis of the facial muscles;

Radiographic features 

  • CT scan

On CT, myelinolysis foci appear as hypodense areas in the lower part of the pons. However, from the artifacts in the posterior fossa, interpretation of the CT picture is difficult, so MRI is the method of choice.

  • MRI

Early changes are visible on the DWI as diffusion restriction areas at the bottom of the bridge. This symptom of osmotic demyelination syndrome is observed in the first 24 hours after the onset of tetraparesis. The same area is hyperintense on T2-WI and hypointense on T1-WI. Changes in T1- and T2-WI can develop within 2 weeks. The affected area has a characteristic “trident” appearance. In some cases, the accumulation of contrast agents is characteristic, similar to the acute phase of multiple sclerosis. Peripheral fibers (ventrolateral longitudinal) remain intact.

Similar changes will be observed during localization in other areas of the GM: basal ganglia, midbrain, subcortical white matter.

Treatment and prognosis of osmotic demyelination syndrome

Treatment and prognosis of osmotic demyelination syndrome

Treatment

The issue of therapy for osmotic demyelination syndrome is currently open and urgent. The problem is still far from being solved. In some cases, according to publications in the specialist literature, large doses of corticosteroid hormones are effective, but in others, they are not effective. Great importance is attached to the restoration of a normal electrolyte balance (sometimes this is enough for a complete recovery), special care, control, and symptomatic treatment of possible complications, in particular, from the psyche.

In general, intensive research continues on demyelinating diseases, including central bridge myelinolysis; there is a reason to believe that with the study of all etiopathogenetic factors and mechanisms, effective ways will be found to restore the myelin sheaths.

Prognosis

Outcome options:

  1. Full recovery is possible.
  2. Minimal neurological deficit:

– Impaired memory, cognitive functions;

– Ataxia, muscle spasticity, diplopia.

  1. May progress to development:

– Spastic quadriparesis

– Syndrome of “locked person” with possible progression to coma, death

Variable behavioral and psychiatric, movement disorders, convulsive syndrome, depression, polyradiculopathy, and neuropathy

  • Often there are “comorbid” conditions, which worsen the prognosis.
  • Mortality from 6 to 90%.

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Lone Star Neurology
4.4
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Edward Medina
Edward Medina
15:34 30 Jun 22
Just such an amazing staff that makes you feel like part of their family. I’ve been going there for over 5 years now... and each visit I get the very best care and treatments that I have ever received in the 20+ years that I’ve been dealing with severe debilitating migraines. Since i started seeing them the number of my migraines has dropped from 15-20 a month to 2-3 every 3 month. I highly recommend them …they will change your life!read more
Daneisha Johnson
Daneisha Johnson
22:20 19 May 22
Dr. Askari was very kind and explained everything so I could understand. The other staff were nice as well. I would... have gave 5 stars but I was a little taken aback when I checked in and had to pay 600.00 upfront. I think that should have been discussed in a appointment confirmation call or email just so I could have been prepared.read more
Jean Cooper
Jean Cooper
16:54 29 Apr 22
I love the office staff they are friendly and very helpful. Dr. JODIE is very caring and understanding to your needs... and wants to help you. I will go back. would recommend Dr. Dr. Jodie to other Patients in a heart beat. The team works well together.read more
Linda M
Linda M
19:40 02 Apr 22
I was obviously stressed, needing to see a neurologist. The staff was so patient and Dr. Ansari was so kind. At one... point he told me to relax, we have time, when I was relaying my history of my condition. That helped ease my stress. I have seen 3 other neurologists and he was the only one who performed any assessment tests on my cognitive and physical skills. At one point I couldn't complete two assessments and got upset and cried. I was told, it's OK. That's why you're here. I was truly impressed, and super pleased with the whole experience!read more
Leslie Durham
Leslie Durham
15:05 01 Apr 22
I've been coming here for about 5 years. The staff are ALWAYS friendly and knowledgeable. The Doctors are the absolute... best!! Jodie Moore is always in such a great mood which is a plus when you are already stressed. Highly recommendedread more
Monica Del Bosque
Monica Del Bosque
14:13 25 Mar 22
Since my first post my thoughts have changed here. It's unfortunate. My doctor and PA were great, but the office staff... is horrible. They never call you back when they say they will, they misinform you, they cause you too much stress wondering what's going on, they don't keep you posted. They never answer the phone. At this point I've left four messages in the last week, and I have sent three messages. Twice from their portal and one direct email. No response. My appointment is on Monday morning at 8:30am, no confirmation on my insurance and what's going on. What the heck is going on, this is ridiculous!I've given up... the stress her office staff has put me through is just not worth it. You can do so much better, please clean house, either change out your office staff, or find a way for them to be more efficient please. You have to do something. This is not how you want to run your practice. It leaves a very bad impression on your business.read more
Ron Buckholz
Ron Buckholz
23:32 23 Mar 22
I was actually pleasantly surprised with this visit! It took me a long time to get the appointment scheduled because no... one answers your phones EVER! After a month, I finally got in, and your staff was warm, friendly, and I was totally impressed! I feel like you will take care of my needs!read more
Steve Nabavi
Steve Nabavi
16:28 16 Mar 22
It was a nice visit. Happy staff doing all they can do to comfort the patients in a very calming environment. You ask... me they are earned a big gold star on the fridge. My only complaint they didn't give me any cookies.read more
Katie Lewis
Katie Lewis
16:10 10 Feb 22
Had very positive appointments with Jodie and Dr. Sheth for my migraine care. Jodie was so fast with the injections and... has so much valuable info. I started to feel light headed during checkout and the staff was SO helpful—giving me a chair, water, and taking me into a private room until I felt better. Highly recommend this practice for migraine patients, they know what they’re doing!!read more
Joshua Martinez
Joshua Martinez
16:02 10 Dec 21
I was scheduled to be checked and just want to say that the staff was fantastic. They were kind and helpful. I was... asked many questions related to what was going on and not once did I feel as though I was being brushed off. The front desk staff was especially great in assisting me. I'm scheduled to go back for a mri and am glad that I'll be going there.read more
Isabel Ivy
Isabel Ivy
21:42 03 Nov 21
I had such a good experience with Lone Star Neurology, Brent my MRI Tech was so awesome and made sure I was very... comfortable during the appointment. He gave me ear plugs, a pillow, leg support and blanket, easiest MRI ever lol 🤣 My 72 hour EEG nurse Amanda was also so awesome. She made sure I was take care of over the 3 days and took her time with the electrodes to make sure it was comfortable for me! Paige was also a huge help in answering all my questions when it came to my test results, and letting me know her honest opinions about how I should go forth with my treatment.read more
Leslie Luce
Leslie Luce
17:37 20 Oct 21
The professionalism and want to help attitude of this office was present from the moment I contacted them. The follow... up and follow through as well as their willingness to find a way to schedule my dad was above and beyond. We visited two offices in the same day with the same experience. I am appreciative of this—we spend a lot of time with doctors and this was top notch start to finish.read more
robert Parker
robert Parker
16:38 16 Apr 21
I love going to this office. The staff is friendly and helpful. The doctor is great. I am getting the best... neurological tests and treatment I have ever had. The only reason I did not give them a 5 star rating is because it is impossible to reach a live person at the office to reschedule appointments. Every time I have tried to get through to the office it says all people are busy and I am sent to a voicemail. If they could get their phone answering fixed, I would give them a strong 5 stars.read more
MaryAnn Hornbaker
MaryAnn Hornbaker
00:26 25 Feb 21
Dr. Harney is an excellent Dr. I found him friendly , personable and thorough. I evidently am an unusual case. ... Therefore he spent a Hugh amount of time educating me. He even gave me literature to further explain my condition and how to follow up. This is something you rarely get from your doctors. So I am more than please with my doctor and his staff.read more
Roger Arguello
Roger Arguello
03:05 29 Jan 21
Always courteous, professional. The staff is very friendly and always work with you to find the best appointment time.... The care team has been great. Always taking the time to listen to your concerns and to find the best treatment.read more
Margaret Rowland
Margaret Rowland
01:12 27 Jan 21
I have been a patient at Lone Star Neurology for several years. Now both my adult daughters also are patients there. I... love Jodie. She is always so prompt whether it is a teleamed call are a visit in the office. She takes the time to explain everything to me and answers all my questions. I am so blessed to have Jodie as my doctor.read more
Susan Miller
Susan Miller
03:01 13 Jan 21
My husband had an accident 5 years ago and Lone Star Neurology has been such a blessing to us with my husbands care.... Jodie Moore is his provider and she is amazing! Jodie is very knowledgeable, caring, and thorough. She takes her time with you, making sure your needs are met and she is happy to answer any questions you may have. Lone Star Neurology’s patients are very lucky to have Jodie providing their care. Thank you Lone Star Neurology and especially Jodie for everything you have done for us. Jodie, you are the best!read more
Windalyn C
Windalyn C
01:32 09 Jan 21
Jodie is wonderful. She is very caring and knowledgeable. I have been to over a dozen neurologists, and none were able... to help me as much as they have here. Thanks!read more
Katie Kordel
Katie Kordel
00:40 09 Jan 21
Jodi Moore, nurse practitioner, is amazing. I have suffered from frequent, debilitating headaches for almost 20 years.... She has provided the best proactive and responsive care I have ever received. My quality of life has been greatly improved by her caring approach and tenacity in finding solutions.read more
Ellie Natsis
Ellie Natsis
15:41 07 Jan 21
I have had the best experience at this neurologist's office! For over a year I have been receiving iv treatments here... each month and my nurse, Bobbie is beyond wonderful!! She's so attentive, knowledgeable, caring, and detail oriented. She makes an otherwise uncomfortable experience much more pleasant and definitely puts me at ease! She also helps me with my insurance,ordering this specialty medication and dealing with the ordering process which is no easy feat.Needless to say, she goes above a beyond in every way and I'm so grateful to this office and to Bobbie for all they do for me!read more
Matt Morris
Matt Morris
15:39 07 Jan 21
Let me start by saying that I have been coming here for years. Due to my autoimmune disease, I am in this office... once every three weeks for multiple hours at a time. The office is very clean and the staff very friendly. My only complaint would be there communication via phone. They aren't the best at responding if you leave a voicemail and expect a call back. I understand that this is prob just due to the sheer number of alls they receive daily. What I can say I like the best about the office are the people. Bobby who handles my infusions is great. I never have any issues with her setting up my infusions. She is very quick to reply to messages sent via text and if she were to leave then my whole opinion of the office may change. I also enjoy people like Matt, Lauren, and Jodi. I appreciate all that they do for me and without this team I'm not sure I would be as happy as I am to visit the office as frequently as I have to. Please ensure that these folks are recognized as they are what makes my visit to this office so tolerable :).read more
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