There is no scientifically proved effective treatment for myelinolysis, and severe. As will be described below, almost all patients who develop ods present with a serum sodium concentration of 120 meql or less. The treatment of hyponatremia saint lukes health system. 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. Osmotic demyelination syndrome encompasses a broad symptomatology from disorientation, slight confusion, paresis and memory loss to seizure, unresponsiveness and coma, depending on the degree of myelin loss in the pons and the presence of extrapontine lesions 1,2,3. In human beings, osmotic demyelination syndrome is termed central or extrapontine myelinolysis.
Magnetic resonance imaging of the brain revealed findings consistent with osmotic demyelination syndrome ods 5. Central pontine myelinolysis cpm is a syndrome that is characterised by rapid destruction of myelin sheaths of mainly oligodendritic cells,1 generally caused by a rapid rise in serum osmolality. Disorders of plasma sodium causes, consequences, and correction. Acute demyelination of the pons or extrapontine areas results in an osmotic demyelination syndrome ods, previously referred to as central pontine myelinolysis cpm or extra pontine myelinolysis epm.
Isolated extra pontine myelinolysis rajitha et al case. Pdf on jul 8, 2014, christian bornke and others published osmotic demyelination syndrome find, read and cite all the research you need on researchgate. Cpm and epm belong to the osmotic demyelination syndrome ods3,6,10,12. However, in recent years, a variety of other medical conditions have. The osmotic demyelination syndrome ods has been a recognized complication of the rapid correction of hyponatremia for decades. This case report demonstrates that if serum sodium is re lowered early in the course of osmotic demyelination syndrome.
Pdf movement disorders and the osmotic demyelination. A consecutive series of patients with osmotic demyelination syndrome. In the event that it occurs, plasmapheresis is promising in aiding neurological recovery. Osmotic demyelination syndrome has been reported in patients with endstage renal disease, but the speci. Osmotic demyelination syndrome definition of osmotic. Areas of the brain that are the slowest to recapture osmolytes are most susceptible to ods. It is caused by the destruction of the layer myelin sheath covering nerve cells in the middle of the brainstem pons. 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. While they are very similar conditions, ods is slightly different. March 08, 2018 67 intravenous saline was continued at a rate of 150 mlhr for next 20 hours. The osmotic demyelination syndrome is a complication of treatment of patients with profound, life.
Most lesions that were followed up resolved rapidly and almost completely, favoring transient edema rather than demyelination. May 01, 2009 the term osmotic demyelination syndrome is used to encompass both entities. The osmotic force created by the high sodium concentration about 40 mm hg for every difference in concentration of 1 mmol per liter maintains the high water content in the tissue, allowing it to withstand pressures that can exceed 20,000 mm hg during exercise. Reversible opercular syndrome secondary to osmotic demyelination. Complete recovery of chronic osmotic demyelination syndrome. An external file that holds a picture, illustration, etc.
Osmotic demyelination syndrome radiology reference. Osmotic demyelination syndrome ods is a lifethreatening demyelinating syndrome. Below are the links to the authors original submitted files for images. The association of ods with hyperosmolar hyperglycemic state hhs has been seldom reported.
Dec 22, 2017 osmotic demyelination unrelated to hyponatremia is rarely reported. Jul 21, 2011 central pontine myelinolysis cpm is a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly. Following supportive treatment he made a full recovery. Osmotic demyelination syndrome ods and overly rapid. Osmotic demyelination syndrome ods is a lifethreatening demyelinating syndrome, which usually occurs in the setting of a rapid correction of severe chronic hyponatremia 1, 2. Osmotic demyelination syndrome ods results from overrapid correction of hypona. Osmotic demyelination syndrome refers to acute demyelination seen in the setting of osmotic changes, typically with the rapid correction of hyponatremia. Osmotic demyelination syndrome ubaidur rahaman senior resident, critical care medicine sanjay gandhi post graduate institute of medical sciences, lucknow, india 2. Among all 106 cases, 49 presented with isolated central pontine myelinolysis. Osmotic demyelination unrelated to hyponatremia american. Movement disorders and the osmotic demyelination syndrome. Osmotic demyelination syndrome radiology reference article. Per contra, reversible ods is a rare state that can be seen in patients with endstage renal disease esrd on hemodialysis hd.
The disorder was first reported in 1959, and the majority of cases were in. The housestaff had set a maximum target for the rate of rise in her p na 12 mmollday 0. Osmotic demyelination syndrome due to hyperosmolar hyperglycemia takanobu hirosawa, taro shimizu cleveland clinic journal of medicine jul 2018, 85 7 5115. Background and objectives rapid correction of severe hyponatremia can result in serious neurologic complications, including osmotic demyelination.
Osmotic demyelination syndrome encompasses central pontine demyelination as well extrapontine demyelination and is seen in alcoholics and malnourished individuals associated with rapid correction. However, conditions such as liver transplantation, dialysis disequilibrium syndrome, and congenital disorders causing hyperammonemia have been found in conjunction with the osmotic demyelination syndrome. Osmotic demyelination syndrome after correction of. On pathophysiological considerations, and as an increasing number of manifestation sites in addition to the pons were detected, central pontine myelinolysis cpm and extrapontine myelinolysis epm were combined into osmotic demyelination. We conclude that a rapid increase in serum sodium concentration and plasma tonicity even in the absence of preceding. The osmotic demyelination syndrome ods has been identified as a complication of the. Isolated extrapontine myelinolysis of osmotic demyelination syndrome. Mri imaging of osmotic demyelination syndrome bjstr. Osmotic demyelination syndrome, central pontine demyelination.
Osmotic demyelination syndrome in endstage renal disease. 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. However, it may occur even after slow correction of hyponatremia. Along with extrapontine myelinolysis, it is part of the osmotic demyelination syndrome. Osmotic demyelination syndrome ods is the irreversible demyelination of the central pons, among other areas of the brain, that is most often associated with rapid correction of hyponatremia. Plasmapheresis to treat osmotic demyelination syndrome. Osmotic demyelination syndrome ods is also referred to as central pontine myelinolysis cpm. In patients who develop osmotic demyelination syndrome after hemodialysis, the lesions may involve the pons or the pons and extrapontine sites. 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.
Osmotic demyelination syndrome in children sciencedirect. A case of osmotic demyelination syndrome occurred after the. Dense astrogliosis within demyelinated areas lfbcv and holzer stains. Case report osmotic demyelination syndrome following. It leads to damage to myelin of pontine and extrapontine structures. Recommendations for treatment of symptomatic hyponatremia.
The part of the brain called the pons is especially sensitive and too much sodium can damage nerve fibers. Treatment often involves relowering of the sodium concentration and aggressive. 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. There are no proven effective therapies for ods, but we describe the first case showing. Association of epm in hyponatraemia from sheehans syndrome is. Selective extrapontine myelinolysis epm following a marked change in osmolarity, usually due to the rapid correction of hyponatraemia. Osmotic demyelination syndrome ods was first described in 1959 by adams and victor, who reported pontine myelinolysis in alcoholic patients. 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. It is thought that alcoholics and malnourished patients have a general.
It is important to identify patients at risk from osmotic demyelination syndrome and to correct their hyponatraemia appropriately. Osmotic demyelination syndrome ods may be observed as a result of a rapid change in serum osmolarity, such as that induced by an overly rapid correction of serum sodium levels in. Osmotic demyelination syndrome in endstage renal disease after recent hemodialysis. Ra performed the literature search and wrote the first draft, and is guarantor for the report. Venkatanarasimha n, mukonoweshuro w, jones j 2008 ajr teaching file. Damned if we do, damned if we dont oh ms, kim hi, carrol hj. Isolated extra pontine myelinolysis rajitha et al case report. Osmotic demyelination syndrome ods occurs after rapid overcorrection of severe. Osmotic demyelination syndrome ods may be observed as a result of a rapid. Bodensteiner, md 10 days later, she began to have an unsteady gait, uncoordinated arm movements, and dysarthia treated with sodium benzoate and sodium. Central pontine myelinolysis cpm, also known as osmotic demyelination syndrome ods, is a rare demyelinating disorder characterized by the loss of myelin in the center of the basis pontis. Osmotic demyelination syndrome linkedin slideshare.
However, conditions such as liver transplantation, dialysis disequilibrium syndrome, and congenital disorders causing hyperammonemia have been found in conjunction with the osmotic demyelination syndrome in recent years. Mr demonstration of mesencephalic lesions in osmotic demyelination syndrome central pontine myelinolysis. Osmotic demyelination syndrome ods and overly rapid correction of hyponatremia uptodate. Plasma exchange successfully treats central pontine.
Few data exist on incidence and risk factors of rapid correction or osmotic demyelination. Psychiatric symptoms and limb tremors associated with. The aim of this study was to present and discuss previous cases and the pathophysiological mechanisms involved in ods secondary to hhs. 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. Osmotic demyelination syndrome in children pediatric neurology. Along with extrapontine myelinolysis, it is part of the osmotic demyelination syndrome ods. Her pontine and thalamic lesions regressed during the. Since the age of 3 he had received substitution therapy for central diabetes. A case of osmotic demyelination syndrome occurred after. On pathophysiological considerations, and as an increasing number of manifestation sites in addition to the pons were detected, central pontine myelinolysis cpm and extrapontine myelinolysis epm were combined into osmotic demyelination syndrome. This clinical case offers an alternative problem representation for acute. Central pontine myelinolysis cpm, also referred to as osmotic demyelination syndrome, is a demyelinating disease that is caused by significant damage to the myelin sheath of nerve cells in the. Kallakatta rn 2011 clinical and functional outcome and factors predicting prognosis in osmotic demyelination syndrome.
Magnetic resonance imaging in central pontine myelinolysis. 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. Extrapontine myelinolysis radiology reference article. Osmotic demyelination syndrome in a 40dayold infant. 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. Osmotic demyelination syndrome ods is brain cell dysfunction. Risk factors and outcomes of rapid correction of severe. Relowering of serum na for osmotic demyelinating syndrome. Osmotic demyelination syndrome in a patient with slowly equalized. Axial fatsaturated t2weighted image showing hyperintensity in the pons with sparing. Osmotic demyelination syndrome as the initial manifestation. Pdf osmotic demyelination syndrome ods is a general term that has become commonplace in the practice of medicine, encompassing. Oct 08, 2005 it is important to identify patients at risk from osmotic demyelination syndrome and to correct their hyponatraemia appropriately.
When the myelin sheath that covers nerve cells is destroyed, signals from one nerve to another arent properly transmitted. These include the syndrome of inappropriate antidiuretic hormone, burns, alcoholism, and psychogenic polydipsia. Slow correction of serum na less than 12 mmollday is recommended to prevent the emergence of this syndrome. Any information contained in this pdf file is automatically generated from digital material submitted to epos by third parties in the form of scientific presentations. Among them, osmotic stress caused by rapid correction of. Extrapontine myelinolysis epm is one of the complications occurring secondary to rapid correction of hyponatremia, and is, along with central pontine myelinolysis encompassed by the more recent term osmotic demyelination syndrome. This places brain cells, particularly oligodendrocytes, at risk of cell shrinkage and hence demyelination. All authors contributed to revisions of the document. Osmotic demyelination syndrome ods is a relatively uncommon neurological complication produced by rapid correction of hyponatraemia. Hameed raina 1, irfan yusuf wani 2 abstractpurpose.
Central pontine myelinolysis genetic and rare diseases. It is caused by osmotic dysregulation in the brain. It leads to damage to myelin of pontine and extrapontine. Osmotic demyelination syndrome encompasses a broad symptomatology from disorientation, slight confusion, paresis and memory loss to seizure, unresponsiveness and. The controversy began in the early 1980s with the suggestion that a neurologic disorder called central pontine myelinolysis was associ.
Role of risk factors in developing osmotic demyelination. Osmotic demyelination syndrome ods, or central pontine myelinolysis cpm, is a complication of. However, in recent years, a variety of other medical conditions have been associated with the development of ods, independent. Osmotic demyelination syndrome is an acute demyelination process that usually occurs several days following an osmotic stress.
Prevention of the osmotic demyelination syndrome after liver. Clinical resolution of osmotic demyelination syndrome following. The neurologic manifestations associated with overly rapid correction have been called the osmotic demyelination syndrome ods. Osmotic demyelination syndrome presenting with chorea. At admission the patient appeared restless and confused and severe. Osmotic shock or osmotic stress is physiologic dysfunction caused by a sudden change in the solute concentration around a cell, which causes a rapid change in the movement of water across its cell. The osmotic demyelination syndrome ods is a seri ous neurologic. Supportive treatment was continued and patient was discharged after 2 wks of stay in hospital. Hyponatremia, hypertonic saline, osmotic demyelination syndrome, myelinolysis, cerebral edema f or nearly 25 years, the treatment of hyponatremia has been called controversial. Osmotic demyelination syndrome, demonstrating as cpm, is a devastating neurologic disorder that often develops after rapid correction of chronic hyponatremia. Multiple risk factors have been known to contribute to these osmotic disturbances. Osmotic demyelination syndrome ods is an acute demyelination process that usually occurs several days following an osmotic stress.
The next sections will discuss the treatment of these causes of. However, brain adaptations that reduce the risk of cerebral edema make the brain vulnerable to injury if chronic hyponatremia is too rapidly corrected. Apr 11, 2014 osmotic demyelination syndrome ods may be observed as a result of a rapid change in serum osmolarity, such as that induced by an overly rapid correction of serum sodium levels in hyponatraemic patients. It is the more recent term replacing central pontine myelinolysis, recognizing that extrapontine structures can also be affected, previously known as extrapontine myelinolysis.
Complete recovery of chronic osmotic demyelination syndrome with plasma exchange published. Central pontine myelinosis and osmotic demyelination syndrome. Osmotic demyelination syndrome is a devastating condition which is largely preventable. The patients motor function deteriorated to a nearly locked in syndrome.
Severe hyponatraemia carries a risk of cerebral oedema with a signi. A 25yearold man presented with progressive impairment of consciousness, ocular bobbing and tetraparesis. Because this patient developed the osmotic demyelination syndrome ods, attention was directed at whether her p na had risen too far, or too fast. Patients with opercular syndrome post osmotic demyelination has good prognosis. Now rarely seen, ods is a clinical syndrome characterized by altered mental status, quadriparesis, dyspnea, dysarthria, and dysphagia which all occur characteristically five to seven days after the correction of serum sodium 3. Recapturing of osmolytes opens up channels in bbb for immunecompetent proteins that attack myelin. Request pdf osmotic demyelination syndrome central pontine myelinolysis is a noninflammatory, symmetric, demyelinating condition involving the central pons. Osmotic demyelination syndrome in children pediatric. The osmotic demyelination syndrome ods has been a recognized complication of the rapid correction of. Case report osmotic demyelination syndrome following rapid. Osmotic demyelination syndrome ods primarily occurs after rapid correction of severe hyponatremia.