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. Osmotic demyelination syndrome linkedin slideshare. A holistic approach in the treatment course should be provided in order to attain recovery. Repeat imaging of the brain done 3 months after starting gluten free diet showed complete resolution of the lesion.
Myelinolysis definition of myelinolysis by the free dictionary. Hyponatremia a 48yearold man with a background of alcohol abuse was found at home by the emergency services with impaired psychomotor status. Central pontine myelinolysis lockedin syndrome youtube. The condition commonly results from a rapid increase in the sodium levels of the human body.
However, changes in sodium levels can lead to rapid movement of fluids in and out of the myelin sheath. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Reversible central pontine and extrapontine myelinolysis in a 16yearold girl. A case of osmotic demyelination syndrome occurred after the. We evaluated a 58yearold woman with pancreatic encephalopathy associated to pontine and extrapontine myelinolysis involving the brain and spinal cord. Article information, pdf download for extrapontine myelinolysis secondary to hypernatremia induced by postpartum water restriction, open. Extrapontine myelinolysis presenting with extra pyramidal features suggestive of parkinsonism may be a challenging clinical syndrome. Pdf extrapontine myelinolysisinduced parkinsonism in a. Reinduction of hyponatremia improves survival in rats with myelinolysis related neurologic symptoms. Longterm neurologic outcome in psychogenic water drinkers with severe symptomatic hyponatremia. It is predominately iatrogenic treatmentinduced, and is characterized by acute paralysis, dysphagia difficulty swallowing, dysarthria difficulty speaking, and other neurological symptoms. Plasma exchange successfully treats central pontine. On the third day, the patient had worsening of sensorium and was referred to our department for further management.
Though cpm can occur with or without epm, both share the same pathological pathways but differ in their clinical manifestations. Pancreatic encephalopathy refers to a gamut of neuropsychiatric symptoms complicating acute pancreatitis. In 1976 a link between these disorders and the rapid correction of sodium in hyponatraemic patients was suggested, and. Charness, in office practice of neurology second edition, 2003. In some cases, cpm is accompanied by extrapontine myelinolysis epm, together cpmepm. Central pontine myelinolysis pathophysiology, causes. Learn what causes it and which symptoms to watch out for. Signs of pseudobulbar palsy with dysphagia and dysarthria, paraparesis or quadriparesis are first symptoms, which usually appear within 26 days after correction of hyponatraemia. Brain mri showed pontine and extrapontine lesions typical for the osmotic demyelination syndrome figure, a. In 1976 a link between these disorders and the rapid correction of sodium in hyponatraemic patients was.
Central myelinolysis was first described in 1959 by adams as demyelinating lesions occurring in the central pons in alcoholic and undernourished patients. Central pontine myelinolysis, or cpm, is a neurological disorder that affects the brain. Central pontine myelinolysis occurs most often as a complication of treatment of patients with profound, lifethreatening hyponatremia low sodium and is a consequence of a rapid rise in serum tonicity following treatment in individuals with chronic, severe hyponatraemia who have made intracellular adaptations to the prevailing hypotonicity. 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 secondary to hypernatremia induced by. 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. A manual of 141 cerebello pontine myelinolysis pdf download rhythmic fingerwork bagpipe pdf download energy medicine pdf free download literatura brasileira ii pdf download. Extrapontine myelinolysis radiology reference article. Recommendations for treatment of symptomatic hyponatremia. Sequential mri in pontine and extrapontine myelinolysis. Osmotic demyelination syndrome ods primarily occurs after rapid correction of severe hyponatremia.
Central pontine myelinolysis cpm may follow correction of hyponatraemia and generally presents with tetraparesis and brain stem dysfunction. Cns nontumor pontine and extrapontine myelinolysis. When extra pontine myelinolysis and central pontine myelinolysis occur simultaneously it is known as the osmotic demyelination syndrome. Central pontine myelinolysis cpm is a neurological condition involving severe damage to the. A 55yearold woman presented to the emergency department with generalised weakness and lightheadedness. Although the mechanism is not fully understood and the treatment is controversial, hyponatremia is probably considered to be the main pathophysiological basis.
Central pontine myelinolysis is a disorder of cerebral white matter that usually affects alcoholics but also occurs in nonalcoholics with liver disease, including wilsons disease, malnutrition, anorexia, burns, cancer, addisons disease, and severe electrolyte disorders, such as thiazide. Central pontine myelinolysis genetic and rare diseases. There were marked perioperative rises 22 meql in the serum sodium concentration in all four patients who developed myelinolysis. In the background of such a history with normal mra, a diagnosis of central pontine.
Central pontine myelinolysis and extrapontine myelinolysis, seminars in ultrasound, ct and mri on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. The full text of this article is available as a pdf 172k. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly. Outcome of central pontine and extrapontine myelinolysis n. Distribution of demyelinating lesions in pontine and extrapontine myelinolysis three autopsy cases including one case. Central and extrapontine myelinolysis charlemagne noukoua keywords. Central and extrapontine myelinolysis affecting the brain. Extrapontine myelinolysis presenting as acute parkinsonism bmc. Central pontine and extrapontine myelinolysis after.
Central pontine myelinolysis information page national. Central pontine myelinolysis symptoms, treatment and prognosis. Pdf central pontine myelinolysis without hyponatraemia. At admission the patient appeared restless and confused and severe. On viewing the imaging findings, history of recent treatment was looked into. Of the 34 patients for whom followup data were available, 32 survived. This is a neurological disease, with a specific location area known as the pons and iatrogenic etiology. The extrapontine myelinolysis of osmotic demyelination syndrome ods is a wellknown but uncommon disorder of the central nervous system. Neuropsychological findings of extrapontine myelinolysis without.
He was admitted in a local hospital and was diagnosed as a case of hyponatremic encephalopathy serum sodium level 116 mmoll. Jul 21, 2011 central pontine myelinolysis cpm is a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly. Extrapontine myelinolysis epm, characterized by demyelination of extra pontine brain areas, often occurs in the setting of acute changes in serum osmolarity, specifically rapid correction of hyponatremia. Common clinical manifestations of myelinolysis are paralysis, dysarthria.
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. Extrapontine myelinolysis presenting as acute parkinsonism. Parkinsonism and recovery in central and extrapontine. 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. Rhythmic fingerwork bagpipe pdf download ciematame. The etiology was not known then but the authors suspected the cause to be either a toxin or a nutritional deficiency. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment.
Wed like to understand how you use our websites in order to improve them. Central pontine and extrapontine myelinolysis in children. Osmotic myelinolysis is a known complication of pancreatic encephalopathy. Extrapontine myelinolysis is rare in pediatric patients. Download fulltext pdf pontine and extrapontine myelinolysis article pdf available in acta medica portuguesa 234. Central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished adams et al. In this disease, myelin and oligodendrocytes are damaged, while the neurons and axons are relatively spared.
Central pontine myelinolysis is considered to require emergent attention once suspected or diagnosed. 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. These conditions are now grouped under the term osmotic demyelination syndrome. Central pontine myelinolysis is a concentrated, frequently symmetric, noninflammatory demyelination within the central basis pontis. Central pontine myelinolysis, paralysis, dysarthria, dysphagia, diplopia, loss of consciousness. The part of the brain called the pons is especially sensitive and too much sodium can damage nerve fibers. Central pontine myelinolysis symptoms, treatment and. Central pontine myelinolysis an overview sciencedirect topics. Clinicians should maintain their vigilance while correcting electrolyte imbalances, especially with associated comorbidity. Regions of involvement manifested increased t2 signal intensity. Alcoholism hyponatremia and central pontine myelinolysis. Pontine and extrapontine myelinolysis can occur after the rapid correction of hyponatremia. The first case of extra pontine myelinolysis was described in 1987 and before that the concept of central pontine myelinolysis was being widely used.
The lesions occur symmetrically in the thalamus, pons, striatum, cerebral cortex, cerebellum and other areas. Ongoing free water losses from diarrhea, ng suction, and extensive third spacing into the bowel delayed the rapid reversal of hypernatremia. The findings in 44 patients 42 of whom were chronic alcoholics with central pontine myelinolysis show that the outcome does not depend on the severity of neurological deficits during the acute phase of the condition or on concomitant internal diseases, including the degree of hyponatremia. Mar 27, 2019 myelinolysis has occurred in individuals undergoing renal dialysis, burn victims, people with hivaids, people overusing water loss pills diuretics, and women with eating disorders such as anorexia or bulimia. This study aimed to identify the causes and contributing factors, neurologic presentation, and outcomes of central pontine and extrapontine myelinolysis and to examine any trends in the presentation and course of these disorders over the past 50 years. Hypokalemia was found to be a predisposing factor in 7 cases of central pontine myelinolysis seen amongst 22 cases of hyponatremia in a recent study, even when rapid correction of hyponatremia and nonacuteness of hyponatremia were not found to be the risk factors. 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. There are additional reports of epm occurring in hyperglycemic hyperosmolar states,1 hypernatremia,2 and normonatremia. We describe the case of a 21yearold woman who was hospitalized at week 10 of gestation because of severe hyperemesis. Central pontine and extrapontine myelinolysis sciencedirect. 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. There was documentation of hyponatraemia na111 meql which was treated with intravenous normal saline. Download fulltext pdf central pontine myelinolysis without hyponatraemia article pdf available in the journal of the royal college of physicians of edinburgh 4. Udainiya department of neurology, sms medical college and hospital, jaipur, india central pontine myelinolysis is a demyelinating affection of central pons diagnosed on the basis of characteristic mri finding in an appropriate clinical setting.
A 58yearold woman presented with subacute onset of tetraparesis, anarthria, and dysphagia after rapid correction of hyponatremia caused by repeated vomiting. Central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished. Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved. In the background of such a history with normal mra, a diagnosis of central pontine myelinolysis with extra pontine myelinolysis involving bilateral basal ganglia was made. To our knowledge, this is the first clinic pathological. Pathology outlines pontine and extrapontine myelinolysis. The clinical dilemma of proper treatment of severe hyponatremia is. A young alcoholic male patient was referred from another hospital with a history of loose stools and vomitings.
Myelinolysis has occurred in individuals undergoing renal dialysis, burn victims, people with hivaids, people overusing water loss pills diuretics, and women with eating disorders such as anorexia or bulimia. Central pontine myelinolysis cpm is a wellrecognized complication of treating patients with severe and prolonged hyponatremia, particularly when corrected too rapidly. Posted in central pontine myelinolysis extrapontine myelinolysis and tagged alzheimers disease, brain injury, cognition, cpmepm, diffusion tensor imaging, electroencephalography, extrapontine myelinolysis, fmri, high definition fiber tracking, magnetic resonance imaging, mri neurography, nfl, pet scan, positron emission tomography, post. Central pontine and extrapontine myelinolysis springerlink. Current concepts in pontine myelinolysis translational biomedicine. Central pontine myelinolysis cpm is a demyelinating disorder affecting mainly the ventral part of the pons. Psychogenic polydipsia excessive fluid seeking behaviour pdf.
There may be a lag between neurological symptoms and radiographic findings in this condition. Recovery from probable central pontine myelinolysis. 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. Extrapontine myelinolysis in a pediatric case of diabetic. The clinical features vary depending on the extent of involvement. Initial signs and symptoms appear within two to three days and include a depressed level of awareness, difficulty speaking, and difficulty swallowing. 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. Central and extrapontine myelinolysis 02 after alcohol.
Extrapontine myelinolysis hyponatremia and central. Hyponatremia at time of admission was 104 meq, corrected to 121 meq within 24hrs. 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. Central pontine myelinolysis an overview sciencedirect. Extrapontine myelinolysis without central pontine myelinolysis is rare and should raise a concern of associated adrenal insu. It is not clearly known how the myelin sheath damage occurs. On rare occasions, demyelination occurs outside the pons and is termed extrapontine myelinolysis epm. Plasma exchange successfully treats central pontine myelinolysis. Osmotic myelinolysis is a distinctive clinical syndrome with characteristic mr features in the central pons central pontine myelinolysis and in other locations extrapontine myelinolysis. Cpmepm is not a primary disease but develops against a background of other, usually severe conditions, apparently as a complication of the main. The osmotic demyelination syndrome ods has been identified as a complication of the rapid correction of hyponatremia for decades. Extrapontine myelinolysis of osmotic demyelination syndrome. Free author information authors from the national institute of neurologic disorders and stroke, national institutes of health, bethesda, maryland b. Central pontine and extrapontine myelinolysis despite.
My son just had his tonsils out a week ago ive been horribly upset because i had cognitive testing to see how cpmepm has impacted my cognitive abilities. Treatment of central pontine myelinolysis with therapeutic. Pontine and extrapontine myelinolysis1, brain, volume 102, issue 2, 1 june 1979, pages 3685. Case report clinical evolution of central pontine myelinolysis in a. In 1976 a link between these disorders and the rapid correction of.
Extrapontine myelinolysis hyponatremia and central pontine. Central pontine myelinolysis cpm is a demyelinating disease of the brainstem but rarely found in other cerebral regions. Extrapontine myelinolysis of osmotic demyelination. However, in recent years, a variety of other medical conditions have been associated with the development of ods, independent of changes in serum sodium which cause a rapid changes in osmolality of the interstitial extracellular compartment of the brain leading. The clinical manifestations and distribution of lesions seen on imaging demonstrate that neurologic illness following correction of hyponatremia is due to myelinolysis. A 41yearold woman presented with acute parkinsonism like features while on a holiday. Central pontine myelinolysis cpm and extrapontine myelinolysis epm are well recognized syndromes. Central pontine and extrapontine myelinolysis after alcohol. It has been decades since the disease has been identified, still development of treatment is undergoing. Resolving mr features in osmotic myelinolysis central. Osmotic demyelination syndrome ubaidur rahaman senior resident, critical care medicine sanjay gandhi post graduate institute of medical sciences, lucknow, india 2.