The condition can be called demyelination syndrome regardless of what. Central pontine myelinosis and osmotic demyelination syndrome. Osmotic demyelination syndrome is a debilitating, potentially fatal disorder which includes two components central pontine myelinolysis cpm. Osmotic demyelination syndrome ubaidur rahaman senior resident, critical care medicine sanjay gandhi post graduate institute of medical. Relowering of serum na for osmotic demyelinating syndrome. Demyelinating disorders are any conditions that damage myelin. Osmotic demyelination syndrome ucsf internal medicine. Following supportive treatment he made a full recovery. Osmotic demyelination syndrome in children pediatric. Correcting the serum sodium at a rate not greater than 0. Prevention and treatment of the osmotic demyelination syndrome. Osmotic demyelination syndrome due to hyperosmolar. It is important to identify patients at risk from osmotic demyelination syndrome and to correct their hyponatraemia appropriately. Case report osmotic demyelination syndrome following rapid.
Osmotic demyelination syndrome ods and overly rapid correction. Osmotic demyelination syndrome ods was first described in 1959 by adams and victor, who reported pontine myelinolysis in alcoholic. However, in recent years, a variety of other medical conditions have. Patients with osmotic demyelination syndrome typically present with severe electrolyte disturbances, which lead to seizures or encephalopathy. After the diagnosis was confirmed by imaging, we started to relower. Pdf atypical forms of the osmotic demyelination syndrome. Pdf osmotic demyelination syndrome ods is an acute demyelination process that usually occurs several days following an osmotic stress. Demyelinating disorders are a subgroup of white matter disorders characterized by the destruction or damage of normally myelinated structures. Axial fatsaturated t2weighted image showing hyperintensity in the pons with sparing. Definitionosmotic demyelination syndrome ods is brain cell dysfunction. Brain signals cant move across scar tissue as quickly, so your nerves dont.
However, if the hyponatraemia is known to be chronic. Osmotic demyelination syndrome ods also known as central pontine myelinolysis cpm was first described by adams et al. Anup kumar bhattacharya, amarendra bikas bera, suman kr roy. We report a case in whom slow correction of hyponatremia 5 mmolday for 3 days induced central pontine myelinolysis cpm. Osmotic demyelination syndrome uf health, university of. The osmotic demyelination syndrome ods is a central nervous system disorder that results from neuronal damage related to abrupt. Thank you teja, for presenting a case during our renal report today a middle aged woman with postoperative hyponatremia. Pdf osmotic demyelination syndrome in children researchgate. Takanobu hirosawa, md and taro shimizu, md, mph, mba. Severe hyponatraemia carries a risk of cerebral oedema with a signi. 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. Osmotic myelinolysis, or central pontine myelinolysis, is seen when rapid osmolality changes occur, typically after rapid correction of.
Demyelination is damage to the myelin sheath around nerves. Because this patient developed the osmotic demyelination syndrome ods, attention was directed at whether her p na had risen too far, or too fast. Osmotic demyelination syndrome, abbreviated ods, is a demyelination associated with osmotic imbalances it classically affect the pons and was previously known as central pontine myelinolysis. There is also no consensus about the best rate of correction. Osmotic demyelination syndrome is an iatrogenic disease related to inappropriately rapid correction of plasma osmolar disorders. Since the age of 3 he had received substitution therapy for central diabetes. 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. 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. Overview of demyelinating disorders neurologic disorders. 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. Typical symptoms include pseudobulbar palsy with dysphagia, dysarthria and paraparesis or quadriparesis while some may go on to develop lockedin syndrome. 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. You do not have access to the full text of this article, the first page of the pdf of this article appears above. Osmotic demyelination syndrome linkedin slideshare.
Osmotic demyelination syndrome in an adolescent with neuromyelitis optica. It is caused by the destruction of the layer myelin sheath covering nerve cells in the middle of the brainstem pons. A 25yearold man presented with progressive impairment of consciousness, ocular bobbing and tetraparesis. The neurologic manifestations associated with overly rapid correction have been called the osmotic demyelination syndrome ods.
The disorder was first reported in 1959, and the majority of cases were in. The serum sodium concentration is the primary determinant of serum tonicity also known as effective serum osmolality see. He is started on 3% normal saline over the next several hours. Relowering of the patients serum sodium with dextrose 5% and desmopressin. Osmotic demyelination syndrome american journal of.
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 brain injury induces demyelination in areas of graywhite apposition and, clinically, results in a delayed neurologic deterioration one to three days following the osmotic. As normonatremia is restored, mental status improves and may return to normal within 4872 hours, only to rapidly deteriorate days later. Osmotic demyelination syndrome ods is the damage over the central nervous system caused by several electrolytes, metabolic and toxic disorders. Clinical resolution of osmotic demyelination syndrome. Osmotic demyelination syndrome ods, which embraces central pontine myelinolysis cpm and extrapontine myelinosis epm, is often underdiagnosed in clinical practice, but can be fatal. Plasmapheresis to treat osmotic demyelination syndrome. When the myelin sheath that covers nerve cells is destroyed, signals from one nerve to another arent properly transmitted.
The osmotic demyelination syndrome ods has been a recognized complication of the rapid correction of hyponatremia for decades. A case of osmotic demyelination syndrome occurred after. The university of florida academic health center the most comprehensive academic health center in the southeast is. A central nervous system disorder caused by demyelination within the central basis pontis of the brain. The neurologic manifestations associated with overly rapid correction have been called the osmotic demyelination syndrome. Osmotic demyelination syndrome is an acute demyelination process that usually occurs several days following an osmotic stress. These disorders may be inflammatory, infective, ischemic. The term demyelination syndrome describes the effects of the condition on the human body, such as the symptoms and results. The original description of osmotic demyelination syndrome was by adams, victor, and mancall in 1959 adams et al 1959. It plays a part in several chronic conditions, including multiple sclerosis. The osmotic demyelination syndrome ods has been identified as a complication of the rapid correction of hyponatremia for decades.
The osmotic demyelination syndrome ods has been a recognized complication of the rapid correction of. The patient was diagnosed to be suffering from osmotic demyelinating syndrome. We aimed to describe cases of unusual forms of ods. Although it is recognised that osmotic demyelination syndrome. Osmotic demyelination syndrome in endstage renal disease. Demyelinating disorders radiology reference article. Pdf on jul 8, 2014, christian bornke and others published osmotic demyelination syndrome find, read and cite all the research you need. Osmotic demyelination syndrome ods is the term used for both central pontine myelinolysis and extrapontine myelinolysis.
Kathleen bateman, mbchb, mrcp uk, fc neurol sa a 17yearold girl presented with intractable. Learn more about types of demyelination and why it. The clinical presentation of osmotic demyelination syndrome is highly variable. 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. Osmotic demyelination syndrome, central pontine demyelination. Osmotic demyelination syndrome after correction of hyponatremia. This article requires you to have a account to view the full text. Microscopically, the lesions show symmetrical myelin destruction affecting all the fibre tracts, with a loss of oligodendrocytes. In human beings, osmotic demyelination syndrome is termed central or extrapontine myelinolysis. Osmotic demyelination syndrome ods is brain cell dysfunction. Although it is recognised that osmotic demyelination syndrome is a rare disease, its dramatic clinical course points out the importance in different disciplines. It is characterized by spastic quadriplegia, pseudobulbar palsy and encephalopathy. Osmotic demyelination syndrome with evolving movement.
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