Central pontine myelinolysis cpm is a rare condition characterised by spastic tetraparesis, pseudobulbar palsy and the lockedin syndrome. Extra and central pontine myelinolysis in a child with adrenal insufficiency. Central pontine myelinolysis fulltext european neurology 2009. As the name suggests cpm involves the central pons whereas epm involves the basal ganglia, thalami and cerebellum. On viewing the imaging findings, history of recent treatment was looked into. Central pontine myelinolysis is another demyelinating disease that affects the. Laureno and karp suggest that it may be impossible to define a level of correction that is always completely free of risk. Osmotic demyelination syndrome with evolving movement. This book is well suited for the medical student, neurology resident, diagnostic radiology resident, neurosurgery resident, advanced practice provider, and practicing clinician who wishes to supplement their course work, show to patients in the clinical setting, or simply broaden their knowledge of neurological disease. Studies show that lesions can occur with or without cpm. A case of osmotic demyelination syndrome occurred after the.
It is characterized by acute paralysis, dysphagia difficulty swallowing, and dysarthria difficulty speaking, and other neurological symptoms. However, it may develop in the context of normal serum. Apr 11, 2014 in about 50% of cases isolated central pontine myelinolysis cpm occurs, whereas the remaining cases are more or less equally divided into isolated extra pontine myelinolysis epm or a combination of cpm and epm, with a slight prevalence of the latter condition. Central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished adams et al. Severe hypernatremia, defined as a rise in the serum sodium concentration to a value exceeding 160meql, leads to the failure of the brain defense mechanism to adapt itself to change, and therefore, it is associated with high mortality due to encephalopathy1,2,3. Central pontine myelinolysis, also known as osmotic demyelination syndrome, is an uncommon disorder associated with rapid correction of severe hyponatremia. In 1976 a link between these disorders and the rapid correction of sodium in hyponatraemic patients was. Marked clinical recovery occurred despite persistent radiologic abnormalities. We present the case of a healthy young pregnant woman with hyperemesis gravidarum who developed severe hyponatremia serum sodium 103 meql. Access to this free content requires users to be registered and logged in. Central pontine myelinolysis cpm and extra pontine myelinolysis epm are osmotic demyelination syndromes.
Among them 4 cases were male, 2 cases were female, with age 24 to 55 years old and average age 36 years old. Osmotic demyelination syndrome ods is a noninflammatory demyelinating disease that usually involves the base of the pons central pontine myelinolysis, cpm and may often extend to the extrapontine structures extrapontine myelinolysis, epm 14. Health, general hospital care analysis hospitalization myelination sodium nutrient health aspects research sodium in the body. Primary bilateral pontine demyelination in a cat with similarity to central pontine myelinolysis article pdf available in veterinary pathology 483. Recovery from probable central pontine myelinolysis. Jul 21, 2011 central pontine myelinolysis cpm is a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly. Central pontine myelinolysis cpm is a demyelinating disorder of central nervous system which involves central portion of the pons and sometimes extrapontine areas also. Central pontine myelinolysis cpm, a frequently fatal disorder, is often associated with hyponatremia. When the myelin sheath that covers nerve cells is destroyed, signals from one nerve to another arent properly transmitted. Albeit mild, the righthanded clumsiness, the dysarthria, and the rate of clinical resolution are consistent with this diagnosis. Sequential observation of movement disorders and brain images in the case of central pontine myelinolysis and extrapontine myelinolysis.
Central pontine myelinolysis complicating treatment of. Central pontine myelinolysis definition of central pontine. This paper analyzed clinical data of 6 patients with central pontine myelinolysis cpm. A symptomfree or stable clinical interval is then followed by ods. Serial magnetic resonance imaging mri demonstrated characteristic abnormalities within the pons at the. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency hyponatremia. Neuroimaging revealed a striking triangular central pontine defect in keeping with central pontine myelinolysis cpm. Striking central pontine myelinolysis in a patient with. Rapid correction of hyponatremia was probably related to the development of the central pontine myelinolysis. In about 50% of cases isolated central pontine myelinolysis cpm occurs, whereas the remaining cases are more or less equally divided into isolated extra pontine myelinolysis epm or a combination of cpm and epm, with a slight prevalence of the latter condition. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly. Central pontine myelinolysis in a patient admitted for.
In 1976 a link between these disorders and the rapid correction of sodium in hyponatraemic patients was suggested, and. Central pontine myelinolysis, pathology international 10. Central pontine myelinolysis cpm is a neurological condition involving severe damage to the myelin sheath of nerve cells in the pons an area of the brainstem. Central pontine myelinolysis cpm occurs in the setting of rapidly corrected hyponatremia, especially in chronically debilitated patients. This condition can be fatal or cause long term disability. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Central pontine myelinolysis an overview sciencedirect. We report a case of a 39yearold woman with a history of chronic alcoholism. The said disease affects specifically the central portion of the. Central pontine myelinolysis can be defined as a medical condition, in which the myelin sheath of the nerve cells in the brainstem become severely damaged. The clinical features vary depending on the extent of involvement. Learn what causes it and which symptoms to watch out for.
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 ods is brain cell dysfunction. Pdf extra and central pontine myelinolysis in a child. Central pontine myelinolysis annals of internal medicine.
Central pontine myelinolysis genetic and rare diseases. Osmotic demyelination syndrome ods, which embraces central pontine myelinolysis cpm and extrapontine myelinosis epm, is often underdiagnosed in clinical practice, but can be. Central pontine myelinolysis cpm, steroidresponsive encephalopathy associated with autoimmune thyroiditis sreat, bickerstaffs brainstem encephalitis bbe are some of the disorders that might present with a localized lesion that specifically involves the pontine area 3. Extrapontine myelinolysis following extreme hypernatremia. It is predominately iatrogenic treatmentinduced, and is characterized by acute paralysis, dysphagia difficulty swallowing, dysarthria difficulty speaking, and other neurological symptoms. These two constitute osmotic demyelination syndrome ods characterized. Osmotic myelinolysis central pontine myelinolysis and. Early diagnosis of central pontine myelinolysis with. Osmotic demyelination syndrome in type 1 diabetes in the.
A young pregnant woman showing clinical symptoms of numbness, vertigo, diplopia, and amnesia with associated hypernatremia ran a fulminant course. Central pontine myelinolysis radiology reference article. The cause is traditionally associated with overzealous correction of hyponatraemia in patients who are malnourished, alcoholic or chronically ill. Central pontine myelinolysis pathophysiology, causes. Its clinical presentation varies, but may include acute paralysis, dysarthria and dysphagia. Mar 20, 2020 central pontine myelinolysis is a serious neurological condition caused by damage to the nerves in the area of the brainstem known as the pons. Pdf primary bilateral pontine demyelination in a cat. 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. Myelinolysis, central pontine definition of myelinolysis. Central pontine myelinolysis is characterised by focal osmotic demyelination within the pons. Extrapontine myelinolysis epm as well as central pontine myelinolysis cpm can occur due to changes in serum osmolality and are referred to as osmotic demyelination syndrome ods. 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. Central pontine myelinolysis in a chronic alcoholic.
The first case of extra pontine myelinolysis was described in 1987 and before that the concept of central pontine myelinolysis was being widely used. We report a patient who developed cpm secondary to profound hyponatraemia and who recovered with no disability. Central pontine myelinolysis secondary to hyperglycaemia. For more information on subscription options, click below on the option that best describes you. Caution is recommended when administering intravenous dextrosecontaining solutions in patients with prolonged fasting, as it may be associated with complications such as wernickes encephalopathy and central pontine myelinolysis. Pdf central pontine and extrapontine myelinolysis researchgate. The osmotic demyelination syndrome ods consisting of central and extra pontine myelinolysis is a relatively rare complication of dka. Central pontine myelinolysis symptoms, treatment and prognosis. Conventional ct and mr imaging findings lag the clinical manifestations of cpm. Treatment with highdose intravenous thiamine only led to a partial clinical improvement. Central pontine myelinolysis, or cpm, is a neurological disorder that affects the brain. A case of osmotic demyelination syndrome occurred after.
Osmotic myelinolysis central pontine myelinolysis and extrapontine. At autopsy, an area of extensive demyelination in the center of the pons was found and a pathological diagnosis of central pontine myelinolysis was made. When extra pontine myelinolysis and central pontine myelinolysis occur simultaneously it is known as the osmotic demyelination syndrome. Cpm is frequently associated with rapid correction of hyponatremia, and it manifests as confusion, quadriplegia, pseudobulbar palsy, andor lockedin syndrome quadriplegia and anarthria with preserved consciousness that develop over several days. Central pontine myelinolysis wikipedia republished wiki 2. Myelinolysis definition of myelinolysis by the free dictionary. The part of the brain called the pons is especially sensitive and too much sodium can damage nerve fibers.
However, there are other predisposing risk factors chronic alcoholism, hypokalaemia that perpetuate the development of ods. In the background of such a history with normal mra, a diagnosis of central pontine. The patient had normal sodium and glucose levels throughout his admission and for the previous 12month period. Introduction pontine myelinolysis pm was first described in 1959 by adams et al. Central pontine myelinolysis information page national. Pdf the case is described of a 46 year old female alcoholic. Central pontine myelinosis and osmotic demyelination syndrome.
This is a demyelinating disease which was first described in the year 1959. Hyponatremia at time of admission was 104 meq, corrected to 121 meq within 24hrs. Myelinolysis definition of myelinolysis by medical. The patient, a 48yearold man, with a long history of alcoholic intake appeared in a hospital with the diagnosis of alcoholic. In patients with this condition, the myelin sheath which protects the nerves is damaged, interfering with their ability to signal to each other. Behavioral manifestations of central pontine myelinolysis.
We present a case in which restricted diffusion was identified within the central pons by using mr diffusionweighted imaging within 24 hours of onset of patient. Central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished. It is caused by the destruction of the layer myelin sheath covering nerve cells in the middle of the brainstem pons. Central pontine myelinolysis cpm is a noninflammatory, demyelinating lesion usually localised in the basis pontis. Isolated extra pontine myelinolysis presenting as acute onset.
Osmotic demyelination syndrome ods, which includes both central pontine myelinolysis cpm and. Treatment of central pontine myelinolysis with therapeutic. Sign in below to access your subscription for full content. The clinical dilemma of proper treatment of severe hyponatremia is. As such the condition is described in the osmotic demyelination syndrome article. Central pontine and extrapontine myelinolysis after alcohol. Very rarely similar lesions are seen involving extra pontine structures called extra pontine myelinolysis epm. The clinical dilemma of proper treatment of severe hyponatremia is exemplified by this. There is progressive failure of verbal articulation dysarthria and spastic paralysis in all four limbs quadriplegia.
Oromandibular dystonia and persistent psychiatric symptoms. A rather rare complication can be extrapontine myelinolysis 24, which is often associated with an overcorrection of hyponatremia. Myelinolysis definition of myelinolysis by the free. It has been suggested that in most cases central pontine myelinolysis arises because of osmotic demyelination and this is likely to occur when there is rapid 12 mmoll24 hours correction of hyponatraemia.
Central pontine myelinolysis is a rare entity of disease that is characterized by a noninflammatory demyelination of oligodendrocy tes of the basis pontis 6 mostly, central pontine myelinolysis lesions are usually symmetrical and bilateral 2. Central pontine myelinolysis or cpm is a rare neurological disorder that primarily affects the myelin sheath of our nerve cells neurons found in the brainstem. In the scenario of hypoglycaemia, 50% dextrose should be administered via a central catheter as it may cause irritation when administered peripherally. Since definitive etiology and pathogenesis of the lesion have not been established, we.
Central pontine myelinolysis, osmotic demyelination. Case report, report by journal of evolution of medical and dental sciences. It is a rare condition that has an undetermined incidence, with many cases diagnosed via autopsy. Central pontine myelinolysis cpm was originally described as a complication of rapid correction of hyponatremia, particularly in the setting of malnutrition, alcohol abuse, and liver disease 9,10. It is impossible to define a level of correction that will be absolutely free of risk.
Central pontine myelinolysis is a demyelinating disorder characterized by the loss of myelin in the center of the basis pontis usually caused by rapid correction of chronic hyponatremia. Mar 04, 2003 in a letter on central pontine myelinolysis, the middle panel of the figure was erroneously rotated 180 degrees. Central pontine myelinolysis cpm, which is a component of the osmotic demyelination syndrome ods, is a frequent neurological complication that follows rapid correction of hyponatraemia. 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. Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved.
Central pontine myelinolysis is mainly an iatrogenic disorder, and its appearance coincided with the widespread use of intravenous therapy for correction of fluid and electrolyte disorders. Central pontine myelinolysis symptoms, treatment and. Central pontine myelinolysis definition of central. Central pontine myelinolysis cpm is a rare demyelination syndrome involving the centre of the basis pontis. Wd of mcps can also occur after pontine hemorrhage and central pontine myelinolysis, not only pons infarction. The triggering factor precedes demyelination and its symptoms by 1 to 14 days. It is commonly reported in settings of hyponatraemia or its rapid correction, but in the last few years it has also been reported in patients with diabetes in the absence of electrolyte disturbances or correction of. Central pontine myelinolysis the jama network myelinolytic lesion of the pons by adams et al in 1959,1 and aptly named central pontine myelinolysis cpm, an increasing number of cases have been. Pdf central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a. An autopsy case of central pontine myelinolysis cpm was reported. Central pontine myelinosis and osmotic demyelination. Central pontine myelinolysis an overview sciencedirect topics. This is a neurological disease, with a specific location area known as the pons and iatrogenic etiology.
Central pontine myelinolysis cpm refers to the nerve damage that occurs due to destruction of myelin sheath that covers nerve cells in the pons. Central pontine myelinolysis in pediatric diabetic. A number of sites may be involved, besides the pons, and among the most. We report a case of a 39yearold woman with a history of chronic alcoholism who. Central pontine myelinolysis cpm, a neurologic disorder caused most frequently by rapid correction of hyponatremia, is characterized by demyelination that. 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 middle of the brainstem, specifically the pons region. Also known as osmotic demyelination syndrome ods, pm is subdivided into central pontine myelinolysis cpm and extrapontine myelinolysis epm 2. The rapid rise in sodium concentration is accompanied by the movement of small molecules and pulls water from brain cells.
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