Ra performed the literature search and wrote the first draft, and is guarantor for the report. Learn more about types of demyelination and why it happens. May 01, 2009 the term osmotic demyelination syndrome is used to encompass both entities. The osmotic demyelination syndrome ods has been identified as a complication of the rapid correction of hyponatremia for decades. In the background of such a history with normal mra, a diagnosis of central pontine. Throughout the 1970s and 1980s, it became clear that the disease not only occurred in alcoholic or malnourished patients but also was commonly associated with rapid correction of hyponatremia, in which sodium level increases by more than 12 mmolld, 4. Mar 23, 2020 osmotic demyelination syndrome ods is brain cell dysfunction. However, in recent years, a variety of other medical conditions have been associated with the development of ods, independent of changes in serum sodium. The extrapontine myelinolysis of osmotic demyelination syndrome ods is a wellknown but uncommon disorder of the central nervous system. Plasma exchange successfully treats central pontine. Damned if we do, damned if we dont oh ms, kim hi, carrol hj. A case of osmotic demyelination syndrome occurred after the. Acquired demyelinating syndromes of the central nervous system. Osmotic demyelination syndrome ods and overly rapid correction.
C the merged panel reveals that cells expressing chop also express. Osmotic demyelination syndrome ods is a very rare, but potentially fatal complication of overly rapid correction of hyponatremia. Relowering of serum na for osmotic demyelinating syndrome. Osmotic demyelination syndrome ods is a disorder characterized by the destruction of neuronal myelin sheaths in either the central area of the pons as in central pontine myelinolysis cpm, or in other susceptible areas such as the basal ganglia, hippocampi or cerebellum known as external pontine myelinolysis epm. Central pontine myelinolysis cpm now termed osmotic demyelination syndrome ods, characterized by damage to regions of the brain most commonly pontine white matter tracts after rapid correction of metabolic disturbances such as hyponatremia low amounts of sodium in the blood. Patient had a history of alcohol abuse and suffered from chronic alcoholic hepatopathy. Osmotic demyelination syndrome ods is brain cell dysfunction. Both had very different neurological outcomes, but the changes of central and extrapontine myelinolysis were apparent on neuroimaging. To assess the functional and clinical outcome in a sizeable cohort of patients with osmotic demyelination syndrome ods and to characterise the factors which could predict the final outcome. By continuing to use our website, you are agreeing to our use of cookies. This case report demonstrates that if serum sodium is re lowered early in the course of osmotic demyelination syndrome ods, many of its devastating consequences may be avoided.
Sodium levels were corrected following consensus guidelines, with clinical improve. Although the mechanism is not fully understood and the. Feb 11, 2017 we discussed the basics of hyponatremia workupmanagement, with an interesting discussion on osmotic demyelination syndrome. Axial fatsaturated t2weighted image showing hyperintensity in the pons with sparing of the peripheral fibers, the patient was an alcoholic admitted with a serum na of 101 treated with hypertonic saline, he was left with quadreparesis, dysarthria, and altered mental status.
In f, the merged image shows colocalization between the two markers, which. A 41yearold man was admitted with acute parkinsonism, which deteriorated over the next few days to a state of akinetic mutism video 1, segment 1. Oct 01, 2019 demyelination is damage to the myelin sheath around nerves. Osmotic demyelination is one among many causes of opercular syndrome. Complete recovery of chronic osmotic demyelination syndrome with plasma exchange published. Hyperglycaemic hyperosmolar state can cause isolated extrapontine osmotic demyelination. Osmotic demyelination syndrome has been reported in patients with endstage renal disease, but the specific mri findings in this patient group have not been documented in detail. The neurologic manifestations associated with overly rapid correction have been called the osmotic demyelination syndrome ods. March 08, 2018 67 intravenous saline was continued at a rate of 150 mlhr for next 20 hours. Osmotic demyelination syndrome, abbreviated ods, is a demyelination associated with osmotic imbalances. Two cases of osmotic demyelination syndrome occurring in children with transient severe hypophosphataemia during the course of their illness are presented. Case report osmotic demyelination syndrome following rapid.
The varied clinical phenotypes of initial acute cns demyelination, termed clinically isolated syndromes cis, include optic neuritis, transverse myelitis, hemisensory. The serum sodium concentration is the primary determinant of serum tonicity also known as effective serum osmolality see. Hameed raina 1, irfan yusuf wani 2 abstractpurpose. As will be described below, almost all patients who develop ods present with. It plays a part in several chronic conditions, including multiple sclerosis. Patients with opercular syndrome post osmotic demyelination has good prognosis. Osmotic demyelination syndrome presenting with chorea. This damage impairs the conduction of signals in the affected nerves. However, brain adaptations that reduce the risk of cerebral edema make the brain vulnerable to injury if chronic hyponatremia is too rapidly corrected. A demyelinating disease is any disease of the nervous system in which the myelin sheath of neurons is damaged. However, it may occur even after slow correction of hyponatremia. The rate of serum sodium concentration correction should be monitored closely to avoid osmotic demyelination syndrome in hyponatremic patients and cerebral. In turn, the reduction in conduction ability causes deficiency in sensation, movement, cognition, or other functions depending on which nerves are involved. Minocycline prevents osmotic demyelination syndrome by.
Osmotic demyelination syndrome symptoms, causes, treatment osmotic demyelination syndrome is also known as central pontine myelionysis cpm. Osmotic demyelination syndrome ods is a lifethreatening demyelinating syndrome. Bartley2 1 registrar in anaesthetics, university hospital of north tees, hardwick road, stockton, uk. Plasmapheresis to treat osmotic demyelination syndrome from. Osmotic demyelination syndrome presenting with chorea t ravindran1, paneerselvam2, radha2, t allwyn yabesh 3 abstract osmotic demyelination syndrome is an acute shift in serum osmolality causing demyelination, which may be due to rapid correction of hyponatremia, hyperglycaemia, hypokalemia and ketoacidosis. Minocycline prevents osmotic demyelination associated with. She was found with severe hyponatremia 103meql at the time of admission. Osmotic demyelination syndrome as the initial manifestation.
Recommendations for treatment of symptomatic hyponatremia. Complete recovery of chronic osmotic demyelination syndrome. Osmotic demyelination syndrome ods is an uncommon disorder, characterized by noninflammatory demyelination involving the pons and other areas of the central nervous system, which may occur for instance as a consequence of an overly rapid correction of hyponatraemia. Osmotic demyelination syndrome presenting with chorea t ravindran1, paneerselvam2, radha2, t allwyn yabesh 3 abstract osmotic demyelination syndrome is an acute shift in serum osmolality causing demyelination, which may be due to rapid correction of hyponatremia, hyperglycaemia, hypokalemia and. Jul 08, 2014 it is important to identify patients at risk from osmotic demyelination syndrome and to correct their hyponatraemia appropriately. In this state, brain cells are highly sensitive to rapid changes in concentrations of effective osmoles. It is important to identify patients at risk from osmotic demyelination syndrome and to correct their hyponatraemia appropriately. Osmotic demyelination syndrome ods primarily occurs after rapid correction of severe hyponatremia. Osmotic demyelination syndrome in endstage renal disease. It is the more recent term replacing central pontine myelinolysis, recognizing that extrapontine structures can also be affected, previously known as extrapontine myelinolysis.
Pdf extrapontine myelinolysis of osmotic demyelination. He obeyed commands to move his eyes but was mute and did not blink, grimace, or move his limbs either to command or pain stimulation. Osmotic demyelination syndrome, demonstrating as cpm, is a devastating neurologic disorder that often develops after rapid correction of chronic hyponatremia. Case report osmotic demyelination syndrome following rapid correction of hyponatraemia d. On viewing the imaging findings, history of recent treatment was looked into. The osmotic demyelination syndrome ods has been a recognized complication of the rapid correction of hyponatremia for decades. Osmotic demyelination syndrome ods, however, represents a very unique pattern of pathologic. Osmotic demyelination syndrome is a serious demyelinating. During chronic hyponatremia, brain cells gradually attain a new osmotic balance with the interstitial space and thereby limit cerebral edema. Demyelination is damage to the myelin sheath around nerves.
Clinical resolution of osmotic demyelination syndrome following. These patients are predisposed to chronic severe hyponatraemia because of a variety of mechanisms including psuedohyponatraemia, hypovolaemia, beer potomania syndrome, cerebral salt wasting syndrome, and reset osmostat syndrome. A 47yearold man arrived to the emergency room due to generalized tonicclonic. As will be described below, almost all patients who develop ods present with a serum sodium concentration of 120 meql or less. All authors contributed to revisions of the document. The name was changed as it is now recognized that it can occur extrapontine. Case definition for acquired demyelinating syndromes of the central nervous system report any child osmotic demyelination syndrome. Signs and symptoms consistent with demyelinating disease. Disorders of water imbalance request pdf researchgate. Slow correction of serum na less than 12 mmollday is recommended to prevent the emergence of this syndrome. Lesions located in bilateral corona radiata can manifest as opercular syndrome. Central pontine myelinosis and osmotic demyelination syndrome. Osmotic demyelination syndrome is a wellknown clinicopathologic entity characterized by edema and demyelination in the pons and extrapontine areas 15. Osmotic demyelination syndrome ucsf internal medicine chief.
Hyponatremia at time of admission was 104 meq, corrected to 121 meq within 24hrs. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. Oct 08, 2005 it is important to identify patients at risk from osmotic demyelination syndrome and to correct their hyponatraemia appropriately. Osmotic demyelination of the brain ods is a dreaded complication that typically occurs several days after aggressive therapy for chronic hyponatrae we use cookies to enhance your experience on our website. Risk factors and outcomes of rapid correction of severe. Osmotic stressinduced defective glial proteostasis contributes to. Osmotic demyelination syndrome refers to acute demyelination seen in the setting of osmotic changes, typically with the rapid correction of hyponatremia. Apr 18, 2016 osmotic demyelination syndrome ods is a disorder characterized by the destruction of neuronal myelin sheaths in either the central area of the pons as in central pontine myelinolysis cpm, or in other susceptible areas such as the basal ganglia, hippocampi or cerebellum known as external pontine myelinolysis epm. Patients with chronic alcoholism are commonly admitted to hospital and given intravenous fluids as part of the treatment of alcohol withdrawal. Recent investigation of the molecular basis of the demyelinating process and the adaptive responses of the brain to dysosmolar challenge has allowed for the refinement of standard treatments and has made the osmotic demyelination syndrome a treatable condition with a better than expected prognosis. Reversible opercular syndrome secondary to osmotic demyelination. Understanding and ending ms cant come fast enough it will take all of us working together.
Cns demyelination is currently not practiced in most centres. The association of ods with hyperosmolar hyperglycemic state hhs has been seldom reported. Previous studies have noted the mri findings of osmotic demyelination syndrome in various patient groups 3, 4. Minocycline prevents osmotic demyelination syndrome by inhibiting the activation of microglia. Role of risk factors in developing osmotic demyelination. Osmotic demyelination syndrome ods, which embraces central pontine myelinolysis cpm and extrapontine myelinosis epm, is often underdiagnosed in clinical practice, but can be fatal. Osmotic demyelination syndrome symptoms, causes, treatment.
Osmotic demyelination syndrome, central pontine demyelination. It is a neurological condition in which patient suffers from over correction and rapid correction of decreased or low sodium level in serum. It is caused by the destruction of the layer myelin sheath covering nerve cells in the middle of the brainstem pons. Osmotic demyelination syndrome ubaidur rahaman senior resident, critical care medicine sanjay gandhi post graduate institute of medical sciences, lucknow, india 2. Bodensteiner, md 10 days later, she began to have an unsteady gait, uncoordinated arm movements, and dysarthia treated with sodium benzoate and sodium.
Although it is recognised that osmotic demyelination syndrome is a rare disease, its dramatic clinical course points out the importance in different disciplines. Apr 11, 2014 osmotic demyelination syndrome ods is an uncommon disorder, characterized by noninflammatory demyelination involving the pons and other areas of the central nervous system, which may occur for instance as a consequence of an overly rapid correction of hyponatraemia. Osmotic demyelination syndrome results from overly rapid serum sodium correction and is often iatrogenic. Case definition for acquired demyelinating syndromes of the central nervous system report any child 12 meql in 24 hours, 25 meql in 48 hours or inadvertent hypernatremia during correction of hyponatremia. From these tables, we produced merged consensus evidence tables for. It classically affect the pons and was previously known as central pontine myelinolysis. Rachel abbott, senior house officer, eli silber, consultant neurologist. Osmotic demyelination syndrome with evolving movement. Osmotic demyelination syndrome ods, however, represents a very unique. Its easy to be a champion for ms research join us and proudly let everyone know that youre helping to lead the ms research revolution. Osmotic demyelination syndrome ods occurs after rapid overcorrection of severe chronic hyponatremia usually in those with a. There are no proven effective therapies for ods, but we describe the first case showing the successful treatment of central pontine myelinolysis cpm by plasma exchange, which occurred after rapid development of hypernatremia from intravenous sodium bicarbonate therapy. Rapid correction was defined as serum sodium increase of 8 meql at 24 hours.