The patients motor function deteriorated to a nearly locked in syndrome. Correction of the serum sodium can result in an osmotic demyelination syndrome ods, a severe and potentially untreatable neurological condition. Derivation and validation of a novel risk score to predict. The pathogenesis of osmotic demyelination syndrome is not completely understood and usually occurs with severe and prolonged hyponatremia, particularly with rapid correction. 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. 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. Rapid overcorrection of hyponatremia can have devastating consequences. We dont see ods very much as its onset is delayed and usually sets in after the patient is admitted to hospital or worse, sent home. Treatment response in osmotic demyelination syndrome. Registration is now required for free access to ccjm content. Ajay panwar,a neurologist,here to answer your query. Background and objectives rapid correction of severe hyponatremia can result in serious neurologic complications, including osmotic demyelination. Osmotic demyelination syndrome due to hyperosmolar hyperglycemia takanobu hirosawa, taro shimizu cleveland clinic journal of medicine jul 2018, 85 7 5115. Osmotic demyelination syndrome occurs when wide fluxes in serum sodium levels are induced by too rapid correction of hyponatremia.
We report a patient with severe hyponatraemia who developed osmotic demyelination syndrome as a consequence of rapid sodium correction. She was diagnosed as a case of osmotic demyelination syndrome ods formerly known as central pontine myelinolysis cpm which was confirmed by mri. The knowledge of conditions associated with an increased risk of osmotic demyelination syndrome and. Central pontine myelinolysis cpm is now more commonly referred to as osmotic demyelination syndrome, which recognizes that the same phenomenon is also seen in other areas of the brain previously known as extrapontine myelinolysis. Magnetic resonance imaging of the brain revealed findings consistent with osmotic demyelination syndrome ods 5. It typically occurs in areas of brain that are slowest to uptake osmolytes, which include central. Osmotic demyelination syndrome as a cons equence of t reating. Demyelination occurs when the myelin sheath that surrounds and protects nerves gets damaged. Osmotic demyelination syndrome, demonstrating as cpm, is a devastating neurologic disorder that often develops after rapid correction of chronic hyponatremia. 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.
In turn, the reduction in conduction ability causes deficiency in sensation, movement, cognition, or other functions depending on which nerves are involved. Other osmotic demyelination lesions other neurological lesions have been linked to cpm and epm including cerebral cortical sclerosis and involvement of the posterior columns. Plasma exchange as treatment for osmotic demyelination. Accidental disruption of his intranasal desmopressin treatment 1 day before admission led to an increase of serum sodium of 29 mmoll in. Accidental disruption of his intranasal desmopressin treatment 1 day before admission led to an increase of serum sodium of 29 mmoll in less than 24 h. Osmotic demyelination syndrome is a wellknown clinicopathologic entity characterized by edema and demyelination in the pons and extrapontine areas 15.
There are limited clinical tools to aid in identifying individuals at high risk of overcorrection with severe hyponatremia. In his first hospital admission, his serum sodium was corrected. Rehabilitation of the pontine ataxiadysmetria syndrome. Slow correction of serum na less than 12 mmollday is recommended to prevent the emergence of this syndrome. However, in recent years, a variety of other medical conditions have been associated with the development of ods, independent. Noncontrast ct a showing symmetrical hypoattenuation of the internal capsules, thalami, and midbrain.
Osmotic demyelination syndrome ods and overly rapid correction. 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. The serum sodium concentration is the primary determinant of serum tonicity also known as effective serum osmolality see. Osmotic demyelination syndrome ods was first described in 1959 by adams and victor, who reported pontine myelinolysis in alcoholic patients. Osmotic demyelination syndrome ods is a rare condition 1 characterized by loss of myelin in various parts of the central nervous system. Oligodendrocytes, which forms the myelin sheaths, are particularly vulnerable to osmotic changes. Temporal evolution of the trident and piglet signs of osmotic. Classic clinical and anatomical descriptions associated with a central pontine location have been supplemented by descriptions of patients with unusual symptoms associated with demyelinating lesions in extrapontine locations. 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. In 1976 a link between these disorders and the rapid correction of sodium in hyponatraemic patients was.
Hameed raina 1, irfan yusuf wani 2 abstractpurpose. It plays a part in several chronic conditions, including multiple sclerosis. 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. In the background of such a history with normal mra, a diagnosis of central pontine.
Risk factors and outcomes of rapid correction of severe. Osmotic demyelination syndrome affects men more often than women, and it is most common in middleaged patients, 2. As such the condition is described in the osmotic demyelination syndrome article. Can osmotic demyelination syndrome be a complication of. Here,i would just like to agree with the treating doctor because extrapontine myelinolysis osmotic demyelination is such a clinical condition where no medicines can make the things better overnight. Few data exist on incidence and risk factors of rapid correction or osmotic demyelination. 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.
Jan 02, 2015 osmotic demyelination syndrome is an permanent iatrogenic condition due to an overly rapid correction of serum sodium 12 meql in 24 hours, 25 meql in 48 hours or inadvertent hypernatremia during correction of hyponatremia. Osmotic demyelination syndrome is a devastating condition which is largely preventable. It is characterized by acute paralysis, dysphagia difficulty swallowing, and dysarthria difficulty speaking, and other neurological symptoms. Register once and log in for full access to articles and content.
Suggest treatment for osmotic demyelination syndrome and. Previous studies have noted the mri findings of osmotic demyelination syndrome in various patient groups 3, 4. Psychogenic polydipsia excessive fluid seeking behaviour pdf. 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 discussed the basics of hyponatremia workupmanagement, with an interesting discussion on osmotic demyelination syndrome. Osmotic demyelination syndrome, abbreviated ods, is a demyelination associated with osmotic imbalances. The condition manifested as acute severe parkinsonism, bilateral ptosis and gaze impairment. Hyponatremia at time of admission was 104 meq, corrected to 121 meq within 24hrs. Thus, some myelin disorders eg, guillainbarre syndrome, chronic inflammatory demyelinating polyneuropathy, some other peripheral nerve polyneuropathies tend to affect primarily the peripheral nerves, and others affect primarily the cns see table disorders that can cause demyelination of the central nervous system. Get the most important science stories of the day, free in your inbox.
Central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished. It classically affect the pons and was previously known as central pontine myelinolysis. Osmotic demyelination syndrome in endstage renal disease after recent hemodialysis. However, it may occur even after slow correction of hyponatremia. Overall, imaging was felt to be most consistent with a diagnosis of osmotic demyelination syndrome ods. This case report describes two subsequent admissions of a patient who was initially thought to be delirious but. The real lesson here is not to use one\u003cbr \\u003e\nname for a syndrome, osmotic demyelination syndrome, or \u0022ods\u0022, in the\u003cbr \\u003e\ntitle and then misuse the more correct one, which is central pontine\u003cbr \\u003e\nmyelinolysis, cpm, in the body of the text and the \u0027m\u0027 is for\u003cbr \\u003e\nmyelinolysis. On viewing the imaging findings, history of recent treatment was looked into. The mechanism of myelinolysis is not fully understood. 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. Role of risk factors in developing osmotic demyelination.
This article is from indian journal of critical care medicine. Because this patient developed the osmotic demyelination syndrome ods, attention was directed at whether her p na had risen too far, or too fast. The osmotic demyelination syndrome ods has been a recognized complication of the rapid correction of hyponatremia for decades. 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. Osmotic demyelination syndrome, central pontine demyelination. Central pontine myelinosis and osmotic demyelination syndrome. Plasma exchange successfully treats central pontine. Tongue fasciculations with denervation pattern in osmotic. Damned if we do, damned if we dont oh ms, kim hi, carrol hj. The housestaff had set a maximum target for the rate of rise in her p na 12 mmollday 0. 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. The onset can come after an infection, such as post viral demyelination, exposure to certain chemicals, or for unknown reasons. Both had very different neurological outcomes, but the changes of central and extrapontine myelinolysis were apparent on neuroimaging. Jul 08, 2014 it is important to identify patients at risk from osmotic demyelination syndrome and to correct their hyponatraemia appropriately.
As will be described below, almost all patients who develop ods present with a serum sodium concentration of 120 meql or less. Microscopically, the lesions show symmetrical myelin destruction affecting all the fibre tracts, with a loss of oligodendrocytes. Osmotic demyelination syndrome ucsf internal medicine chief. The neurologic manifestations associated with overly rapid correction have been called the osmotic demyelination syndrome ods. Ods osmotic demyelination syndrome developing with slow correction of sodium in a patient of hyponatremia has been reported earlier. A sh r afn,l ockl eyr ii t z d nu p tm with death or neurologic damage in outpatients. Demyelination can occur anywhere in the central nervous system. Central pontine myelinolysis radiology reference article.
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. Complete recovery of chronic osmotic demyelination syndrome. 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. Central pontine myelinolysis an overview sciencedirect topics. Overview of demyelinating disorders neurologic disorders. This is caused by diseases that damage the myelin sheath or the cells that form it. Osmotic demyelination syndromes, which consist of central pontine myelinolysis cpm and extrapontine myelinolysis epm, are rapidly progressing, often fatal focal symmetric syndromes, with clinical features ranging from a mild tremor or dysarthria to a progressive quadraparesis and a lockedin 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. Osmotic demyelination syndrome in endstage renal disease. Learn more about types of demyelination and why it happens. This is free video of neurology osmotic demyelination syndrome from doctor in training usmle step 1 2019 freemedtube. Central pontine myelinolysis cpm is a neurological disorder caused by severe damage of the myelin sheath of nerve cells in the area of the brainstem termed the pons, predominately of iatrogenic, treatmentinduced cause.
Myelin demyelination and dysmyelination medical news. In 1976 a link between these disorders and the rapid correction of sodium in hyponatraemic patients was suggested, and. It can occur even in normonatremic patients, especially who have risk factors like alcoholism, malnutrition and liver disease. Neurology osmotic demyelination syndrome doctor in.
Complete recovery of chronic osmotic demyelination. Pdf osmotic demyelination syndrome ods may be observed as a result of a rapid change in serum osmolarity, such. One week after discharge, he returned with confusion, ataxia and dysphagia, and he ultimately turned out to have developed an osmotic demyelination syndrome ods. Osmotic demyelination syndrome ods primarily occurs after rapid correction of severe hyponatremia.
Using a rat model of osmotic demyelination, the researchers showed that. Do not hesitate to contact me to discuss a possible project or learn more about my work. Osmotic demyelination syndrome hku elearning platform in. A case of osmotic demyelination syndrome occurred after the. Osmotic demyelination syndrome due to hyperosmolar hyperglycemia. Patients with chronic alcoholism are commonly admitted to hospital and given intravenous fluids as part of the treatment of alcohol withdrawal. Top pearls osmotic demyelination syndrome ods is a very rare, but potentially fatal complication of overly rapid. Two cases of osmotic demyelination syndrome occurring in children with transient severe hypophosphataemia during the course of their illness are presented. It is subdivided into central pontine myelinolysis cpm and extrapontine myelinolysis epm, depending on what level the demyelination occurs 2.
In the event that it occurs, plasmapheresis is promising in aiding neurological recovery. Pdf on jul 8, 2014, christian bornke and others published osmotic demyelination syndrome find, read and cite all the research you need on. The separation of patients with myelinolysis in central pontine and extrapontine locations is possible on the basis of. Osmotic demyelination syndrome ods is a general term that has become commonplace. Central pontine myelinolysis cpm is a neurological condition involving severe damage to the. Osmotic demyelination syndrome ods is brain cell dysfunction. Osmotic demyelination syndrome ods was first reported in 1959 by adams and victor among malnourished patient and patient with chronic alcoholism. The knowledge of conditions associated with an increased risk of osmotic demyelination syndrome and careful management.
Pdf a case of osmotic demyelination syndrome occurred after the. This occurs in many different neurological diseases, including multiple sclerosis. Hyperammonemia in a patient with ornithine transcarbamylase deficiency. This damage impairs the conduction of signals in the affected nerves. 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. Since the age of 3 he had received substitution therapy for central diabetes insipidus after surgical treatment of a craniopharyngioma. Abrupt correction of chronic hyponatraemia can cause osmotic demyelination syndrome ods. Oct 01, 2019 demyelination is damage to the myelin sheath around nerves. Ods is a preventable consequence of rapid correction of hyponatremia and is associated with high morbidity and mortality. Clinical resolution of osmotic demyelination syndrome.
A case of osmotic demyelination syndrome occurred after. She underwent plasma exchange pe on the 20th day since her symptoms started and underwent 7 cycles of pe with complete neurological recovery. Osmotic demyelination syndrome linkedin slideshare. Bilateral tongue fasciculations with denervation pattern in electromyogram is a manifestation. When the myelin sheath that covers nerve cells is destroyed, signals from one nerve to another arent properly transmitted. A 25yearold man presented with progressive impairment of consciousness, ocular bobbing and tetraparesis. Our case demonstrates that osmotic demyelination syndrome develops also with graded correction of hyponatremia. Osmotic demyelination syndrome central pontine myelinolysis flashcards from eva gonzaless class online, or in brainscapes iphone or android app. Feb 27, 2019 demyelination is a term used to describe the destruction of a substance called myelin that surrounds axonal fibres. 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 ubaidur rahaman senior resident, critical care medicine sanjay gandhi post graduate institute of medical sciences, lucknow, india 2. Severe hyponatremia is strongly associated with increased mortality and should therefore always be treated 1,2. 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 with evolving movement. At admission the patient appeared restless and confused. It is caused by the destruction of the layer myelin sheath covering nerve cells in the middle of the brainstem pons. Central pontine myelinolysis, and osmotic demyelination syndrome, present most commonly as a complication of. Background and objectives osmotic demyelination syndrome is the most concerning complication of severe hyponatremia, occurring with an overly rapid rate of serum sodium correction. This is a pdf file of an unedited manuscript that has been accepted for publication. Osmotic demyelination syndrome after correction of. This case report describes a 57yearold man who presented first with lethargy and dysarthria due to hyponatremia resulting from poor intake and diuretics. Although it is recognised that osmotic demyelination syndrome is a rare disease, its dramatic clinical course points out the importance in different disciplines. Osmotic demyelination syndrome presenting with chorea. Relowering of serum na for osmotic demyelinating syndrome.
Bodensteiner, md 10 days later, she began to have an unsteady gait, uncoordinated arm movements, and dysarthia treated with sodium benzoate and sodium. Feb 11, 2017 thank you teja, for presenting a case during our renal report today a middle aged woman with postoperative hyponatremia. As will be described below, almost all patients who develop ods present with. We report a case of ods secondary to overcorrection of severe hyponatremia with pathognomonic clinical and radiologic signs making a complete. Imaging findings in osmotic demyelination syndrome. We describe the case of a 21yearold woman who was hospitalized at week 10 of gestation because of severe hyperemesis. This case report describes two subsequent admissions of a patient who was initially thought to be delirious but turned. A demyelinating disease is any disease of the nervous system in which the myelin sheath of neurons is damaged. Trident sign in osmotic demyelination syndrome qjm. Osmotic demyelination syndrome due to hyperosmolar. Aug 21, 2014 this is a set of cranial mris from a patient with osmotic demyelination syndrome, formerly called central pontine myelinolysis.
Central pontine myelinolysis wikipedia republished wiki 2. Plasmapheresis to treat osmotic demyelination syndrome. 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. Recommendations for treatment of symptomatic hyponatremia. At admission the patient appeared restless and confused and severe. Osmotic demyelination syndrome commonly affects the pons and infrequently involves the extrapontine region.
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