Search

Osmotic demyelination syndrome is classically associated with rapid correction of hyponatremia. However, it can occur in normonatremic patients with other electrolyte abnormalities. One must suspect osmotic demyelination syndrome in susceptible patients with other electrolyte abnormalities like hypokalemia and hypophosphatemia., (© Journal of the Association of Physicians of India 2011.)

Background: Central pontine and extrapontine myelinolysis (CPM/EPM) are severe neurologic complications after liver transplantation., Methods: The present work retrospectively evaluated single-center prevalence of CPM/EPM and associated risk factors: cause of liver disease, hepatic encephalopathy, preoperative, intraoperative, and perioperative blood components use, serum levels, and variation of Na, Cl, and K and immunosuppression were compared between CPM/EPM patients and control group of transplanted patients without neurologic complications., Results: Among 997 transplants, CPM/EPM were diagnosed in 11 patients (1.1%), of whom four were CPM, one was EPM, and six were associated CPM and EPM. Control group consisted of 44 transplanted patients. Central pontine and extrapontine myelinolysis patients experienced higher intraoperative and perioperative serum Na/24 hr variations compared to controls (16.69 ± 5.17 vs. 9.8 ± 3.4 mEq/L, P = 0.001). Maximum peak of intraoperative or perioperative serum Na was significantly higher in patients compared to controls (151.5 ± 3.3 vs. 140.8 ± 6.2 mEq/L, P ≤ 0.001), but no difference in preoperative serum Na was detected. Three patients presented hypernatremia as isolated risk factor., Conclusion: Extrapontine myelinolysis can be found isolated or associated with CPM in up to two of three liver transplanted patients with myelinolysis. A marked variation of perioperative serum Na remains the main risk factor even in patients without preexisting hyponatremia; however, isolated hypernatremia may be solely responsible in some cases.

Purpose: 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. In animal models, initiation of re-lowering within four hours of symptom onset has been associated with better outcomes than initiation within eight to ten hours of symptom onset. As there is no effective therapy for the condition we suggest a trial of re lowering of serum sodium early in its course., Case Report: A 33 years old woman who was on diuretics presented to us with delirium in the form of hypo responsiveness for two to three days after suffering from an acute gastroenteritis. On evaluation she was found to have hyponatremia that was corrected too rapidly and was followed by an initial improvement and later worsening of neurological signs. T2 weighted MRI of the brain showed hyperintense lesions in pons and in extra pontine areas including thalamus and cerebellum. The patient was diagnosed to be suffering from osmotic demyelinating syndrome. Re-lowering of the patient's serum sodium with dextrose 5% and desmopressin was tried along with aggressive supportive treatment. Patient was reassessed after regular intervals and at 6 months post treatment patient has recovered almost completely and is living an independent life., Conclusion: Based on the absence of other effective therapies, and the poor prognosis associated with ODS, it is suggested re-lowering the serum sodium to a level that is just below the maximal target value at 48 hours of less than 18 meq/L above the initial serum sodium. Re-lowering therapy should be initiated as quickly as possible after the onset of neurologic symptoms that are attributed to ODS.

Extrapontine myelinolysis is characterized by symmetric demyelination following rapid shifts in serum osmolality in the supratentorial compartment. Extrapontine myelinolysis in children is rare compared to adults. The most common underlying pathophysiology is rapid correction of hyponatremia. Only 2 cases were published after diabetic ketoacidosis without electrolyte imbalance in the English literature. This study presents an unusual and possibly the youngest case of extrapontine myelinolysis that occurred in the setting of diabetic ketoacidosis and complicated cerebral edema without electrolyte imbalance, along with a review of the literature., (© The Author(s) 2014.)

Background: Central pontine and extrapontine myelinolysis (CPEPM) is a rare but potentially fatal complication after orthotopic liver transplantation (OLT). The aim of this study was to identify risk factors for development of CPEPM after OLT and to assess patient outcome., Methods: We reviewed the clinical data of 1,378 patients who underwent OLT between 1987 and 2009 in Geneva, Switzerland and Edmonton, Canada. Nineteen patients (1.4 %) developed CPEPM. We compared their characteristics with control patients, matched by age, gender, date of OLT, and MELD score., Results: The 19 patients with CPEPM (7F, mean age 52.1 ± 2 years) had a mean MELD score of 26 ± 2.2. Before OLT, patients who develop CPEPM presented more frequently low (<130 mmol/l; p < 0.04) and very low (<125 mmol/l; p < 0.009) sodium than controls. In patients developing CPEPM, the number of platelet units and fresh frozen plasma transfused during surgery was higher (p = 0.05 and 0.047), hemorrhagic complications were more frequent after OLT (p = 0.049), and variations of sodium before and after OLT were higher (p = 0.023). The association of >2 of these conditions were strongly associated with CPEPM (p = 0.00015). Mortality at 1 year of patients developing CPEPM was higher (63 vs. 13 %, p < 0.0001)., Conclusions: High MELD score patients undergoing OLT, receiving massive perfusions of Na-rich products, experiencing surgery-related hemorrhagic complication and important fluctuations of Na are at risk of developing CPEPM. Therefore careful monitoring of natremia in the perioperative period and use of water-free perfusion in case of massive blood-products transfusion are critical points of this patient management.

Central pontin myelinolysis has since the 1970s been correlated with hyponatraemia. A 56-year-old woman was admitted to hospital after a few days of diminished capacity and acute disorientation and respiratory distress. Initially, her alcohol overconsumption was not recognised. She presented with only a light hyponatraemia and sepsis. Following fluid resuscitation she developed hypernatraemia and was later diagnosed with osmotic demyelination syndrome. Despite several weeks of therapy the outcome was fatal.

Objective: To characterize clinical and radiologic features of patients with central pontine myelinolysis (CPM) and identify variables that predict outcome., Patients and Methods: We retrospectively studied patients diagnosed as having CPM identified by a search of Mayo Clinic medical records from January 1, 1999, through December 31, 2010. Diagnosis was made by clinical and radiologic features. Favorable outcome was defined by a modified Rankin Scale score of 2 or lower. Volume of signal abnormality on brain magnetic resonance imaging (MRI) was quantified by a neuroradiologist blinded to outcomes. Wilcoxon rank sum tests were used to assess association between volume of signal abnormality and outcomes at discharge and last follow-up., Results: Of 24 patients, 14 (58%) had only CPM, and 10 (42%) had extrapontine involvement. Hyponatremia was documented in 18 patients (75%), with median sodium nadir of 114 mmol/L. Eighteen patients (75%) had alcoholism, and malnutrition was documented in 12 (50%). Presenting symptoms included encephalopathy (n=18 [75%]), ataxia (n=11 [46%]), dysarthria (n=7 [29%]), eye movement abnormalities (n=6 [25%]), and seizures (n=5 [21%]). Favorable outcome was seen in 15 patients (63%) at last follow-up. Findings on initial brain MRI were normal in 5 patients, but all MRI scans were abnormal with serial imaging. The volume of radiologic signal abnormality was not associated with outcome at discharge or last follow-up (P=.67 and P=.37, respectively)., Conclusion: Clinical outcome in patients with CPM is not predicted by the volume of radiologic T2 signal abnormality on MRI or the severity of hyponatremia. Serial brain imaging is of value because a substantial proportion of patients have normal findings on initial MRI.

A 55-year-old woman with a history of excess alcohol intake presented to the acute medical unit following concerns regarding her electrolyte disturbances. During correction of the electrolytes, the patient developed central pontine myelinolysis. The unusual features in the case were the absence of hyponatraemia which is usually associated with central pontine myelinolysis and also the good recovery that the patient made. Looking at the electrolyte changes, we suspect there may be a link to the rapid osmotic shifts occurring during refeeding and central pontine myelinolysis.

Central pontine myelinolysis (CPM) may be more prevalent after liver transplantation (OLT). Central pontine and extrapontine myelinolysis (CPEM) is rare. The occurence of CPM may be associated with hyponatremia, a rapid rise in serum sodium concentrations, postoperatively increased plasma osmolality, and the duration of the operation. Only 1 patient had abnormal sodium levels before LT. No abnormalities were detected in immunosuppressive drug blood levels. The aim of this paper was to report our experience with CPEM among LT patients., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Central pontine myelinolysis is a rare but devastating cause of morbidity and mortality after orthotopic liver transplant. The exact cause of central pontine myelinolysis is uncertain. However, rapid correction of hyponatremia has been described as a major factor. We describe a patient with central pontine myelinolysis after orthotopic liver transplant in the absence of significant hyponatremia. Although rapid correction of hypernatremia has been reported in association with central pontine myelinolysis, to our knowledge, in this case, where the serum sodium went from normal to hypernatremic, later diagnosis of central pontine myelinolysis in a postliver transplant setting is unique. We also discuss factors that may contribute to the development of central pontine myelinolysis after orthotopic liver transplant and its pathophysiology.

We report clinical, radiological and pathological findings in a patient with central pontine and extrapontine myelinolysis. The patient was a 61-year-old woman who had a radical mastectomy for breast cancer. Based on clinical evidence, acute hyponatremia had set in only a few days before onset of symptoms. The patient's disease progressed in two stages and became more severe during slow hyponatremia correction after 9 days from onset. Diffusion MRI provided early evidence of neurological lesions. In spite of a therapeutic attempt, the patient died unexpectedly 18 days after onset of her neurological disease due to massive pulmonary embolism. Histologically, our findings confirmed that the major features of central pontine myelinolysis in the acute stage are demyelination, the presence of large amounts of macrophages with no lymphocytic inflammatory reaction, and moderate astrocytosis. It is interesting to note that a monotypic immunological reaction persisted 19 days after radiological demonstration of parenchymal alterations.

We report a case of a 75-year-old male patient who presented to the emergency room with arterial hypotension and impaired vigilance. The patient was on lithium therapy due to mood disorder. One month earlier medication with a betablocker, a loop-diuretic and an ACE-inhibitor had been started due to heart failure. Findings at admission included renal insufficiency, pneumonia and a slightly increased serum level of lithium. Three days later his Glasgow Coma Scale Score was 7, he showed gaze deviation, increased muscle tonus and cloni. The patient fully recovered after volume substitution and normalization of his renal function. Diagnosis of chronic intoxication with lithium was made due to the clinical picture and after exclusion of neurological pathologies. The pharmacokinetic characteristics of lithium is described and the risk factors leading to lithium intoxication and treatment of intoxication are discussed.

The central pontine and extrapontine myelinolysis is an important entity in the neurology disorders in the context with alcohol abuse. Though often first based on clinical characteristics, the diagnosis can be often supported with radiologic imaging.

Background and Objectives: Critically ill patients frequently develop neurologic symptoms, which frequently become a clinical challenge. Described approximately 50 years ago, pontine neuronal demyelination is a pathologic change associated with neurologic and psychiatric problems after liver transplantation. The objective of this report was to present a case of central pontine myelinolysis diagnosed after liver transplantation and to discuss its pathophysiology., Case Report: A 29 years old female patient underwent liver transplantation for fulminant hepatic failure. Postoperatively, she developed neurologic symptoms characteristic of the Locked In Syndrome and the MRI showed changes compatible with central pontine myelinolysis. The patient did not develop dramatic changes in sodium plasma levels, which is frequently incriminated as the causal agent, and improved considerably within a few weeks., Conclusions: The etiology of central pontine myelinolysis is multifactorial, and special attention should be given to the group of patients at greater risk, such as those with sudden changes in the plasma levels of sodium, liver transplantation, chronic alcoholics, and malnourished. It is important to recognize that osmotic demyelination can develop in patients with low, normal, or elevated plasma levels of sodium, indicating the contribution of other trigger factors.

Introduction: Central pontine myelinolysis (CPM) is almost always described in association with a disturbance in sodium homeostasis, most commonly rapid correction of chronic hyponatremia. It has only rarely been described in patients with disturbances of serum osmolality in the absence of abnormal serum sodium concentrations., Methods: Case report., Results: A 93 year-old-man developed marked gait ataxia 2 days after the diagnosis and treatment of hyperosmolar hyperglycemia. MRI demonstrated a symmetric lesion in the central pons consisting of increased T2 signal intensity and restricted diffusion, consistent with CPM. Calculated serum osmolality on admission was 344 mOsm/kg and fell to 300 mOsm/Kg over 20 h. Serum sodium concentration stayed between 137 and 140 mEq/l throughout the admission. One month after admission, his ataxia had nearly completely resolved and the MRI changes had improved., Conclusion: CPM can develop in the setting of hyperosmolar hyperglycemia without abnormalities of sodium homeostasis. This supports the theory that the pathogenesis of CPM is dependent on a relatively hypertonic insult, which may occur independently of sodium abnormalities. CPM can present as isolated gait ataxia. Clinical manifestations of the disorder may show significant improvement despite a dramatic initial presentation.

Osmotic demyelination syndrome is usually associated with hyponatremia or rapid correction of this condition. The prognosis is usually fatal. We treated a 34-year-old chronic renal failure patient who did not have hyponatremia but developed severe pontine myelinolysis demonstrated with MRI. Serial MRI revealed gradual reduction of the lesions over 2 months. This case demonstrates that osmotic demyelination syndrome is not always associated with hyponatremia, and that, although the prognosis is usually poor, some patients recover.

We report the case of a 54-year-old alcoholic female patient who was hospitalized for neurologic alterations along with a severe hyponatremia (plasma Na+: 97 mEq/l). She suffered from potomania and was given, a few days before admission, a thiazide diuretic for hypertension. A careful correction of plasma Na+ levels was initiated over a 48-hour period (rate of correction < 10 mEq/l/24h) in order to avoid brain demyelination. After a 2-day period of clinical improvement, her neurologic condition started to deteriorate. By the 5th day of admission, she became tetraplegic, presented pseudobulbar palsy, ataxia, strabism, extrapyramidal stiffness and clouding of consciousness. Scintigraphic and MRI investigations demonstrated pontine and extrapontine lesions associated with Gayet-Wernicke encephalopathy. After correction of ionic disorders (hyponatremia, hypokaliemia) and vitamin B (thiamine) deficiency, the patient almost completely recovered without notable disabilities. This case illustrates that profound hyponatremia, in a paradigm of slow onset, can be compatible with life. It also demonstrates that demyelinating lesions, usually considered as a consequence of a too fast correction of hyponatremia, may occur despite the strict observance of recent guidelines. There is increasing evidence to suggest that pontine swelling and dysfunction may sometimes occur in alcoholic patients even in absence of disturbance in plasma Na+ levels. It is therefore of importance, while managing a hyponatremic alcoholic patient, to identify additional risk factors (hypokaliemia, hypophosphoremia, seizure-induced hypoxemia, malnutrition with vitamin B deficiency) for brain demyelination and to correct them appropriately.

A 24-year-old woman developed hyperemesis gravidarum, complicated by Wernicke's encephalopathy and central pontine myelinolysis. The first-was due to a deficiency of thiamine, and was hastened by intravenous dextrose without thiamine supplements. However, no changes in serum sodium or phosphorus values were observed. In this case, the pathophysiology of central pontine myelinolysis remained obscure.

Myelinolysis is a neurologic disorder that can occur after rapid correction of hyponatremia. Initially named "central pontine myelinolysis," this disease is now known to also affect extrapontine brain areas. Manifestations of myelinolysis usually evolve several days after correction of hyponatremia. Typical features are disorders of upper motor neurons, spastic quadriparesis and pseudobulbar palsy, and mental disorders ranging from mild confusion to coma. Death may occur. The motor and localizing signs of myelinolysis differ from the generalized encephalopathy that is caused by untreated hyponatremia. Experiments have duplicated the clinical and pathologic features of myelinolysis by rapidly reversing hyponatremia in animals. Myelinolysis is more likely to occur after the treatment of chronic rather than acute hyponatremia and is more likely to occur with a rapid rate of correction. The exact pathogenesis of myelinolysis has not been determined. Optimal management of hyponatremic patients involves weighing the risk for illness and death from untreated hyponatremia against the risk for myelinolysis due to correction of hyponatremia. Experiments in animals and clinical experience suggest that correction of chronic hyponatremia should be kept at a rate less than 10 mmol/L in any 24-hour period.

In a recent series of 44 liver transplants we identified both extrapontine myelinolysis (EPM) - characteristic of cyclosporine neurotoxicity - and central pontine myelinolysis (CPM) in 5 recipients posttransplant. An additional 2 recipients had EPM only posttransplant. MRIs performed in 4 asymptomatic recipients were normal. Large perioperative shifts in serum sodium, hypomagnesemia, and high cyclosporine levels may play a role in the development of these lesions, although the evidence from this study is inconclusive. In addition to supportive care, dilantin was started in patients who had seizures; aggressive magnesium replacement was initiated for hypomagnesemia, and cyclosporine levels were reduced in all patients. All patients demonstrated a slow steady recovery and all but 2 are at home at the time of writing. CPM may be more prevalent than previously appreciated following liver transplantation, although its prognosis may not be as dismal.

Central pontine myelinolysis (CPM) is a rare syndrome generally linked to hyponatremia. Autopsy and imaging studies described demyelination in the central pons, in some cases combined with thalamic, internal capsular, hemispheric and cerebellar white matter lesions. The clinical syndrome and prognosis spans from asymptomatic patients displaying only imaging lesions to coma and death. Frequent findings are pyramidal tract and pseudobulbar signs. No effective treatment is known. Alcohol abuse is often associated. The imaging pattern of CPM is unique and generally accepted as diagnostic when other causes such as ischemia, multiple sclerosis, tumors, radiation, or pharmacological effects are ruled out. However, the exact mechanism of this selective process of demyelination remains obscure. The current major controversy concerns the role of therapeutic efforts of sodium level restoration in the etiology of CPM. We present a case of CPM where no hyponatremia was found. We hypothesize that (1) iatrogenic sodium restoration may not in all cases of CPM be the putative mechanism, (2) the clinical onset of CPM may in some patients represent a delayed response to an initially asymptomatic demyelination, and (3) normal admission sodium levels do not exclude the diagnosis of CPM.

Central pontine myelinolysis can occur after orthotopic liver transplantation leading to high mortality and serious morbidity. In our case, central pontine myelinolysis was associated with wide fluctuations in cyclosporine levels during an episode of hypocholesterolaemia, which may have precipitated central pontine myelinolysis.