The usual predisposing factors are chronic alcoholism, malnutrition and rapid correction of. Can osmotic demyelination syndrome be a complication of. The osmotic demyelination syndrome ods has been a recognized complication of the rapid correction of hyponatremia for decades. Slow correction of serum na less than 12 mmollday is recommended to prevent the emergence of this syndrome. Pdf osmotic demyelination syndrome ods may be observed as a result of a rapid change in serum osmolarity, such as that. Osmotic demyelination syndrome following correction of. Plasma exchange as treatment for osmotic demyelination. Complete recovery of chronic osmotic demyelination. Magnetic resonance imaging of the brain revealed findings consistent with osmotic demyelination syndrome ods 5. Pdf osmotic demyelination syndrome ods is an acute demyelination process that usually occurs several days following an osmotic stress.
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. In a recent study of 34 patients with osmotic demyelination syndrome two died, and, of the remaining 32, a third recovered, a third were debilitated but independent, and a third were dependent. However, in recent years, a variety of other medical conditions have been associated with the development of ods, independent of changes in serum sodium. Imaging findings in osmotic demyelination syndrome. Osmotic demyelination syndrome ods is a disorder of the central myelin that is often associated with a precipitous rise of serum sodium. Osmotic demyelination syndrome presenting with chorea. The prognosis of the osmotic demyelination syndrome has long been regarded as bleak, primarily because before ctmri this was a postmortem diagnosis. The condition manifested as acute severe parkinsonism, bilateral ptosis and gaze impairment. We report a case of ods secondary to overcorrection of severe hyponatremia with pathognomonic clinical and radiologic signs making a complete. Osmotic demyelination syndrome central pontine and. Clinical resolution of osmotic demyelination syndrome following. Bilateral tongue fasciculations with denervation pattern in electromyogram is a manifestation. 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. 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 with evolving movement. Although it is recognised that osmotic demyelination syndrome is a rare disease, its dramatic clinical course points out the importance in different disciplines. Therapeutic effect of steroids in osmotic demyelination of infancy. The movement disorders of epm represent a treatable manifestation of the osmotic demyelination syndrome in that a. 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.
However, the same treatment results in fatal demyelination if. Risk factors and outcomes of rapid correction of severe. Since osmotic demyelination syndrome is an iatrogenic disease its early recognition and effective prevention is mandatory. Hameed raina 1, irfan yusuf wani 2 abstractpurpose. History 1959 victor and adams first described central pontine myelinolysis clinical findings quadriparesis, pseudobulbar paralysis pathology myelin loss confined within the central pons was felt as a sequela of alcoholism or malnutrition 1962 recognised that lesions can occur outside the pons. Clinical resolution of osmotic demyelination syndrome.
A case of osmotic demyelination syndrome occurred after the. Osmotic demyelination unrelated to hyponatremia is rarely reported. Complete recovery of chronic osmotic demyelination syndrome with plasma exchange published. Mar 14, 2018 the pathogenesis of osmotic demyelination syndrome is not completely understood and usually occurs with severe and prolonged hyponatremia, particularly with rapid correction. 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. Jul 08, 2014 although it is recognised that osmotic demyelination syndrome is a rare disease, its dramatic clinical course points out the importance in different disciplines. The neurologic manifestations associated with overly rapid correction have been called the osmotic demyelination syndrome ods. Osmotic demyelination syndrome due to hyperosmolar hyperglycemia. All authors contributed to revisions of the document. March 08, 2018 67 intravenous saline was continued at a rate of 150 mlhr for next 20 hours. Osmotic demyelination syndrome due to hyperosmolar.
It is the more recent term replacing central pontine myelinolysis, recognizing that extrapontine structures can also be affected, previously known as extrapontine myelinolysis. It is a neurological condition in which patient suffers from over correction and rapid correction of decreased or low sodium level in serum. We conclude that a rapid increase in serum sodium concentration and plasma tonicity even in the absence of. Noncontrast ct a showing symmetrical hypoattenuation of the internal capsules, thalami, and midbrain. Article information, pdf download for osmotic demyelination syndrome as a consequence of treating hyperammonemia in a patient with. Osmotic demyelination syndrome ods is a general term that has become commonplace in the practice of medicine, encompassing both central pontine myelinolysis and extrapontine myelinolysis. In this state, brain cells are highly sensitive to rapid changes in concentrations of effective osmoles. Therapeutic relowering of plasma sodium after overly rapid. Osmotic demyelination syndrome ods occurs after rapid overcorrection of. Damned if we do, damned if we dont oh ms, kim hi, carrol hj. The pathogenesis of osmotic demyelination syndrome is not completely understood and usually occurs with severe and prolonged hyponatremia, particularly with rapid correction. Osmotic demyelination syndrome is extremely rare in patients with plasma sodium 120 meql incidence as low as 0.
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 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. Osmotic demyelination syndrome ubaidur rahaman senior resident, critical care medicine sanjay gandhi post graduate institute of medical sciences, lucknow, india 2. Overall, imaging was felt to be most consistent with a diagnosis of osmotic demyelination syndrome ods. Pdf on jul 8, 2014, christian bornke and others published osmotic demyelination syndrome find, read and cite all the research you need. Article alerts email article citation tools, share. We report the case of a 25yearold man with a diagnosis of osmotic demyelination syndrome. During chronic hyponatremia, brain cells gradually attain a new osmotic balance with the interstitial space and thereby limit cerebral edema. 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. On viewing the imaging findings, history of recent treatment was looked into. The osmotic demyelination syndrome ods is a neurologic complication associated with rapid. Tongue fasciculations with denervation pattern in osmotic. Osmotic demyelination syndrome symptoms, causes, treatment.
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. A limited number of cases have reported positive outcomes with plasma exchange in the treatment of ods associated with chronic alcohol abuse or liver transplantation. Recommendations for treatment of symptomatic hyponatremia. Two cases of osmotic demyelination syndrome occurring in children with transient severe hypophosphataemia during the course of their illness are presented. Remarkably, while the myelin and oligodendrocytes of specific brain areas degenerate during the disease, neighboring neurons and axons appear unspoiled, and neuroinflammation appears only once demyelination is well established. Mri revealed typical features of central pontine and. Here we report a case of ods occurred after correction of hyponatraemia with isotonic saline solution in a pregnant. Pdf a case of osmotic demyelination syndrome occurred after the. It is frequently seen with sodium dysregulation such as rapid correction of hyponatremia. It is caused by the destruction of the layer myelin sheath covering nerve cells in the middle of the brainstem pons. By continuing to use our website, you are agreeing to our use of cookies. 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.
Osmotic demyelination syndrome in children pediatric. However, brain adaptations that reduce the risk of cerebral edema make the brain vulnerable to injury if chronic hyponatremia is too rapidly corrected. Download figure open in new tab download powerpoint. Osmotic demyelination syndrome in children pediatric neurology. The patient was young healthy man with no history of chronic alcoholism or malnutrition. The knowledge of conditions associated with an increased risk of osmotic demyelination syndrome and careful management of. 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. Oct 08, 2005 it is important to identify patients at risk from osmotic demyelination syndrome and to correct their hyponatraemia appropriately. Osmotic brain injury induces demyelination in areas of graywhite apposition and, clinically, results in a delayed neurologic deterioration one to three days following the osmotic challenge. Osmotic demyelination unrelated to hyponatremia american.
In the background of such a history with normal mra, a diagnosis of central pontine. Osmotic demyelination syndrome symptoms, causes, treatment osmotic demyelination syndrome is also known as central pontine myelionysis cpm. Osmotic demyelination syndrome commonly affects the pons and infrequently involves the extrapontine region. The patients motor function deteriorated to a nearly locked in syndrome. Osmotic demyelination syndrome is an acute demyelination process that usually occurs several days following an osmotic stress. However, in recent years, a variety of other medical conditions have been associated with the development of ods, independent. However, it may occur even after slow correction of hyponatremia. Overly rapid correction of chronic 48 hours hyponatremia results in brain dehydration, rendering it susceptible to the osmotic demyelination syndrome. Among all 106 cases, 49 presented with isolated central pontine myelinolysis. Hyponatremia at time of admission was 104 meq, corrected to 121 meq within 24hrs. Clinical and functional outcome and factors predicting. Ods is a preventable consequence of rapid correction of hyponatremia and is associated with high morbidity and mortality.
Osmotic demyelination syndrome ods and overly rapid correction of hyponatremia uptodate. Osmotic demyelination syndrome is extremely rare in patients with plasma sodium 120 meql incidence as. Ra performed the literature search and wrote the first draft, and is guarantor for the report. Jul 01, 2018 a 55yearold man with a 20year history of type 2 diabetes mellitus was admitted to the hospital after presenting to the emergency department with an acute change in mental status. It can occur even in normonatremic patients, especially who have risk factors like alcoholism, malnutrition and liver disease.
Complete recovery of chronic osmotic demyelination syndrome with plasma exchange sumbal nasir mahmood 1, kunwer naveed mukhtar 2, osama kunwer naveed 3 and ahmed kunwer naveed 4 1 professor and head of nephrology department at ziauddin hospital, karachi, pakistan 2 professor and head of nephrology at liaquat national hospital, pakistan. Both had very different neurological outcomes, but the changes of central and extrapontine myelinolysis were apparent on neuroimaging. Three days earlier, he had begun to feel abdominal discomfort and dizziness, which gradually worsened. A case of osmotic demyelination syndrome occurred after. Central pontine myelinosis and osmotic demyelination syndrome. We report a patient with severe hyponatraemia who developed osmotic demyelination syndrome as a consequence of rapid sodium correction. Bodensteiner, md 10 days later, she began to have an unsteady gait, uncoordinated arm movements, and dysarthia treated with sodium benzoate and sodium. Osmotic demyelination syndrome ods is characterized by widespread degeneration of myelin within the central nervous system and has no established treatment.
May 01, 2009 unenhanced ct performed shortly after the patient was transferred to the tertiary care center revealed a large wellcircumscribed area of hypoattenuation within the pons, fig 1a, with bilateral symmetric foci of hypoattenuation in the thalami, fig 1b, findings suggestive of osmotic demyelination syndrome. Complete recovery of chronic osmotic demyelination syndrome. Osmotic demyelination syndrome ods is brain cell dysfunction. Ods has been described in patients after liver transplant, after treatment for hyperammonemic disease or with dialysis disequilibrium syndrome. Osmotic demyelination syndrome after correction of. Relowering of serum na for osmotic demyelinating syndrome. Osmotic demyelination syndrome radiology reference article. Osmotic demyelination syndrome ods is characterized by degeneration of myelin within the central nervous system cns with sharply demarcated lesions within the brain, especially the pons, and is diagnosed by magnetic resonance imaging mri 1,2. Osmotic brain injury induces demyelination in areas of graywhite apposition and. 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. Osmotic demyelination syndrome, demonstrating as cpm, is a devastating neurologic disorder that often develops after rapid correction of chronic hyponatremia. The prognosis is very variable from complete regression of clinical symptomatology to signs of significant quadruparesis, a vegetative state and death. The osmotic demyelination syndrome ods is an uncommon disorder, characterised by noninflammatory demyelination involving the pons and other areas of the central nervous system. The osmotic demyelination syndrome is a complication of treatment of patients.
Osmotic demyelination syndrome definition of osmotic. 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, central pontine demyelination. Role of risk factors in developing osmotic demyelination. Treatment response in osmotic demyelination syndrome. Osmotic demyelination syndrome as a consequence of treating. Plasma exchange successfully treats central pontine. Osmotic demyelination syndrome refers to acute demyelination seen in the setting of osmotic changes, typically with the rapid correction of hyponatremia. Osmotic demyelination syndrome ods occurs after rapid overcorrection of severe chronic hyponatremia usually in those with a predisposition such as chronic alcoholism, malnutrition, or liver disease.
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