Central pontine myelinolysis in a case of cerebral malaria. The clinical dilemma of proper treatment of severe hyponatremia is. Central pontine myelinolysis is a concentrated, frequently symmetric, noninflammatory demyelination within the central basis pontis. We present the case of a healthy young pregnant woman with hyperemesis gravidarum who developed. The clinical features vary depending on the extent of involvement. When the myelin sheath that covers nerve cells is destroyed, signals from one nerve to another arent properly transmitted.
However, it may develop in the context of normal serum sodium. Primary bilateral pontine demyelination in a cat with similarity to central pontine myelinolysis. Central pontine myelinolysis cpm is a noninflammatory, demyelinating lesion usually localised in the basis pontis. Thanks to the ninjadoc, i have been made aware of another way that a person can develop hyponatremia, which increases their risk for cpm. On rare occasions, demyelination occurs outside the pons and is termed extrapontine myelinolysis epm. Central pontine myelinolysis annals of internal medicine. We present a case of central pontine myelinolysis cpm in a patient with advanced hiv infection and miliary tuberculosis. Conventional ct and mr imaging findings lag the clinical manifestations of cpm. Central pontine myelinolysis in patient with normal serum sodium. Central pontine myelinolysis cpm is a demyelinating disease of the brainstem but rarely found in other cerebral regions. A diagnosis of central pontine myelinolysis was made.
The authors present an important clinical observation on a favorable recovery of lockedin syndrome due to central pontine myelinolysis cpm in association with liver transplantation, hyponatremia. So the other name for central pontine myelinolysis is osmotic demyelination syndrome. We describe here an unusual case of cpm occurring in a patient with cerebral malaria. 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. The main cause of central pontine myelinolysis is a rapid correction of low sodium levels in the body. Central pontine myelinolysis is caused by a rapid change in the bodys sodium levels, either from treating low sodium hyponatremia or high sodium hypernatremia in a health care setting.
Plasma exchange successfully treats central pontine. Introduction pontine myelinolysis pm was first described in 1959 by adams et al. Prognosis is not uniformly bad mri changes may be delayed mri severity is not prognostic r j martin central pontine and extrapontine myelinolysis. Central pontine myelinolysis secondary to hyperglycaemia. Central pontine myelinolysis cpm is a rare condition usually caused by rapid sodium correction in hyponatraemia after a severe neurological syndrome. Hence, the slow correction of low levels of sodium would prevent its occurrence. Magnetic resonance imaging of the brain revealed central pontine myelinolysis with a welldefined lesion in the pons of low t 1signal intensity panel a, arrow and high t 2signal intensity. We describe a case of cpm occurring secondary to nephrogenic diabetes insipidus di, which developed as a consequence of severe hypokalaemia. Changes in peroxidase activity with formation of free radicals. The part of the brain called the pons is especially sensitive and too much sodium can damage nerve fibers. Over a period of 1 year we diagnosed central pontine myelinolysis cpm in five patients all of whom survived, two of them with complete functional recovery despite extensive lesions on cranial computerized tomography and magnetic resonance imaging. We report a case of a 39yearold woman with a history of chronic alcoholism who. For more information on subscription options, click below on the option that best describes you. Central pontine myelinolysis genetic and rare diseases.
We describe the case of a 21yearold woman who was hospitalized at week 10 of gestation because of severe hyperemesis. It is characterized by acute paralysis, dysphagia difficulty swallowing, and dysarthria difficulty speaking, and other neurological symptoms. Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved. Central pontine myelinolysis with minimal hyponatremia in. Myelinolysis definition of myelinolysis by the free dictionary.
With corticobulbar pathway involvement, patients are affected by dysarthria and dysphagia 3. Pdf central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a. 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 27, 2019 central pontine myelinolysis cpm is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency hyponatremia. Central pontine myelinolysis, or cpm, is a neurological disorder that affects the brain. Central pontine myelinolysis in advanced hiv infection. Pdf central pontine myelinolysis induced by alcohol. Signs of pseudobulbar palsy with dysphagia and dysarthria, paraparesis or quadriparesis are first symptoms, which usually appear within 26 days after correction of hyponatraemia.
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. Pdf primary bilateral pontine demyelination in a cat. Treatment of central pontine myelinolysis with therapeutic. So, central pontine myelinolysis is the destruction of the myelin sheath around nerve cells that are in the pons. 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. 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 in the vast majority of cases it is associated with central pontine myelinolysis but it can also rarely occur as an isolated entity. A case of osmotic demyelination syndrome occurred after the.
Files are available under licenses specified on their description page. Learn what causes it and which symptoms to watch out for. All structured data from the file and property namespaces is available under the creative commons cc0 license. Most published cases are based on autopsy reports, whereas antemortem diagnosis is rare. Nov 15, 2019 how can central pontine myelinolysis be prevented. 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. At admission the patient appeared restless and confused and severe. Keywords autocorrection, central pontine myelinolysis, hyponatraemia, osmotic demyelinisation syndrome abstract central pontine myelinolysis is a rare and potentially lifethreatening complication of a sudden rise in serum osmolality. Recovery from probable central pontine myelinolysis. Osmotic demyelination syndrome ods primarily occurs after rapid correction of severe hyponatremia. Diabetic ketoacidosis, alcoholic ketoacidosis, starvation ketosis and hypoglycaemic ketoacidosis.
A 9 year old boy presented with fever, drowsiness, quadriparesis and facial myokymia. In 1976 a link between these disorders and the rapid correction of. Therapeutic effect of steroids in osmotic demyelination of infancy. Central pontine myelinolysis cpm is a noninflammatory, demyelinating condition that has been described mostly in the context of rapid correction of hyponatraemia. Although usually associated with rapid correction of hyponatremia, cpm may occur despite normonatremia, is. Central pontine myelinolysis cpm is a clinical condition characterized by myelin destruction in the rostral part of the pons. 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. Wd of mcps can also occur after pontine hemorrhage and central pontine myelinolysis, not only pons infarction.
Central pontine and extrapontine myelinolysis after alcohol. As such the condition is described in the osmotic demyelination syndrome article. The neurologists attributed it to a high blood pressure blood drug called moduretic and 48 hours of vomiting. The authors present an important clinical observation on a favorable recovery of lockedin syndrome due to central pontine myelinolysis cpm in. Decreased diffusion in central pontine myelinolysis. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly. 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. Central pontine myelinolysis cpm, a frequently fatal disorder, is often associated with hyponatremia. Two patients with central pontine myelinolysis cpm were studied with diffusionweighted mr imaging 1 week after onset of tetraplegia. Central pontine myelinolysis radiology reference article. Central pontine myelinolysis cpm occurs in the setting of rapidly corrected hyponatremia, especially in chronically debilitated patients. Central pontine myelinolysis cpm, a neurologic disorder caused most frequently by rapid correction of hyponatremia, is characterized by demyelination that affects the central portion of the base of the pons. Central pontine myelinolysis following rapid correction of.
However, there are other predisposing risk factors chronic alcoholism, hypokalaemia that perpetuate the development of ods. The payee may view, download, andor print the article for hisher personal, scholarly, research. Recovery of lockedin syndrome in central pontine myelinolysis. 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. Stegbauer, alex schneider, john mukai, and kenneth r. By continuing to use our website, you are agreeing to our use of cookies. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. While hospitalized the patient developed an unusual ataxic variant of cpm with full clinical recovery. It is caused by the destruction of the layer myelin sheath covering nerve cells in the middle of the brainstem pons. Decreased diffusion in central pontine myelinolysis steven c. Central pontine myelinolysis cpm is an acute demyelinating neurological disorder affecting primarily the central pons and is frequently associated with rapid correction of hyponatremia. Central pontine myelinolysis and the rate of correction of.
An mri scan is much recommended and considered as the main diagnostic tool for central pontine myelinolysis. Chronic alcoholism is frequently associated with this condition which may have. Central pontine myelinolysis fulltext european neurology 2009. We present a case in which the diagnosis of cpm was confirmed by computed tomography ct. Central pontine myelinolysis an overview sciencedirect. Central pontine myelinolysis mayo clinic proceedings. In mri scan results, damage to the pons is more visible compared with ct scan results. Central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished adams et al. Central pontine myelinolysis, also known as osmotic demyelination syndrome, is an uncommon disorder associated with rapid correction of severe hyponatremia. Feb 09, 2014 there may be no safe limit for the rate of rise of na. Access to this free content requires users to be registered and logged in. Central pontine myelinolysis symptoms, treatment and prognosis. However, it may develop in the context of normal serum. Laureno and karp suggest that it may be impossible to define a level of correction that is always completely free of risk.
Early diagnosis of central pontine myelinolysis with. It is predominately iatrogenic treatmentinduced, and is characterized by acute paralysis, dysphagia difficulty swallowing, dysarthria difficulty speaking, and other neurological symptoms. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency hyponatremia. In this manner, the incidence rate of cpm can also be greatly reduced. Central pontine myelinolysis cpm is a demyelinating disorder characterized by the loss of myelin in the center of the basis pons, and is mainly caused by the rapid correction of hyponatremia.
The osmotic demyelination syndrome j neurol neurosurg psychiatry 2004. The rapid rise in sodium concentration is accompanied by the movement of small molecules and pulls water from brain cells. Central pontine myelinolysis secondary to hypokalaemic. Central pontine myelinolysis cpm is a demyelinating disease of the pons and is associated with a rapid correction of. Central pontine myelinolysis cpm is heterogeneous group of demyelinating.
Central pontine myelinolysis cpm is a neurological demyelinating disease of the pons. 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. However, there are case reports of this disease in the setting of normal serum sodium or minimal hyponatremia. Primary bilateral pontine demyelination in a cat with similarity to. Central pontine myelinolysis cpm is classically attributed to overly rapid correction of profound hyponatremia. Introduction central pontine myelinolysis cpm is a syndrome that is characterised by rapid destruction of myelin sheaths of mainly. Only few cases have been reported during pregnancy, most of which were reported in patients with hyperemesis. This means that central pontine myelinolysis, or a subtype of central pontine myelinolysis, affects less than 200,000 people in the us population. Central pontine myelinolysis is characterised by focal osmotic demyelination within the pons. The patient was presumed to have hypernatremiainduced central pontine myelinolysis and extrapontine myelinolysis. The clinical dilemma of proper treatment of severe hyponatremia is exemplified by this case. 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.
Classical clinical manifestations range from a depressed level of consciousness to dysarthria and flaccid quadriplegia. 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 pathophysiology, causes. 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. In describing a patient with central pontine myelinolysis cpm and behavioral symptoms, price and mesulam1 considered the development of cpm to be related to the rapid correction of hyponatremia. 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. 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. Early diagnosis of central pontine myelinolysis with diffusionweighted imaging kimberly a. If the pons and the corticospinal and bulbar tracts are affected in cpm, patients present with frequent encephalopathies and signs of damage to the brainstem. The serum sodium concentration in this patient on admission was 114 mmoll and 17.
Central pontine myelinolysis wikipedia republished wiki 2. Central pontine myelinolysis cpm is a rare, acute, and uniformly fatal demyelinative. 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. In at least 10% of patients with central pontine myelinolysis, demyelination also occurs in extrapontine regions, including the mid brain, thalamus, basal nuclei, and cerebellum. Central pontine myelinolysis lockedin syndrome youtube. Pdf central pontine myelinolysis is a rare and potentially lifethreatening complication of a sudden rise in serum osmolality. Central pontine and extrapontine myelinolysis was experimentally produced in dogs by the rapid correction of severe, sustained, vasopressin. These cases have been hypothesized to be secondary to other metabolic disturbances such as hyperglycemia or hypophosphatemia.
Diagnostic examination for central pontine myelinolysis includes imaging tests. Extrapontine myelinolysis radiology reference article. Central pontine myelinolysis an overview sciencedirect topics. Osmotic demyelination syndrome ods is brain cell dysfunction. Pdf central pontine myelinolysis cpm commonly presents as a complication of treatment in patients with profound. There are no inflammatory changes, and blood vessels are normal. Central pontine myelinolysis cpm is a rare, acute, and uniformly fatal demyelinative process that involves the pons almost exclusively. Central pontine myelinolysis is listed as a rare disease by the office of rare diseases ord of the national institutes of health nih. In the course of our studies of the neuropathology of alcoholism, which were begun at the neurological unit, boston city hospital, and have continued in the lab.
Central pontine myelinolysis cpm is known to be almost universally fatal. Initial signs and symptoms appear within two to three days and include a depressed level of awareness, difficulty speaking, and difficulty swallowing. The ct scan and mri scan are two conventional procedures for cpm. My wife, 50, suffered central pontine myelinolysis in august 2000. This case report underlines the importance of recognising risk factors predisposing the patient to the development of central pontine myelinolysis.
Hyponatremia alone or slowly corrected hyponatremia did not produce the disease. Recovery after central pontine myelinolysis springerlink. Central pontine myelinolysis information page national. Also known as osmotic demyelination syndrome ods, pm is subdivided into central pontine myelinolysis cpm and extrapontine myelinolysis epm 2. The cause is traditionally associated with overzealous correction of hyponatraemia in patients who are malnourished, alcoholic or chronically ill. Common clinical manifestations of cpm include spastic quadriparesis, dysarthria, pseudobulbar palsy, and encephalopathy of various degrees. Its clinical presentation varies, but may include acute paralysis, dysarthria and dysphagia. This is a neurological disease, with a specific location area known as the pons and iatrogenic etiology.
To determine outcome and potential predictors of outcome in pontine and extra pontine myelinolysis, these authors retrospectively analyzed results of four types of electrophysiologic tests and brain imaging data in 44 patients treated between 1990 and 1996 at one hospital 26 patients or at other hospitals 18 patients in the german state of north rhinewestphalia. In 1976 a link between these disorders and the rapid correction of sodium in hyponatraemic patients was. Central pontine myelinolysis cpm 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. It is commonly found in association with alcoholism, rapidly corrected hyponatraemia and electrolyte abnormalities. Central pontine myelinolysis, paralysis, dysarthria, dysphagia, diplopia, loss of consciousness.
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