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MRI of the Head. Hyperdense image on T2 (axial) and hypodense in T1 (sagittal), triangular and without capture, at the geometric center of the pons. Bat-wing shape seen on axial view.
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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 ponti...
Context in source publication
Context 1
... followed movements with horizontal eye movements, but she did not followed commands, and Babinski sign was positive bila- terally. After 24 hours, and magnetic ressoance imaging (MRI) of the head showed central pontine demyelination (Figure 1), confirming the diagnosis of CPM. The patient recovered in a few weeks, being discharged from the hospital with mild dysarthria and dysmetria. ...
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Citations
... ODS can develop either due to hyperglycemia per se or during correction of hyperglycemia using insulin [6] .CPM following orthotopic liver transplant varies from 5 to 10 % [7,8]. ODS can occur in patients with low, high or normal sodium levels particularly in liver transplant patients [9].CPM is multifactorial in etiology partly due to glial / neuronal stress and partly to deficiency in generation of organic osmolytes [10]. In liver transplant patients there is deficiency of organic osmolytes like myo inositol making them vulnerable to CPM [11] . ...
Objective: To highlight the occurrence of osmotic demyelination syndrome (ODS) in settings other than the classical ODS induced by rapid correction of hyponatremic states. The background, clinico- radiological features, treatment and outcome of eight ODS patients are discussed here. We encountered eight patients with ODS in Materials and Methods: uncommon clinical settings at the department of neurology, Government Stanley medical college hospital, Chennai between April 2017 to October 2018. Patients were evaluated, investigated, treated and outcome was assessed. Results: Eight patients in the age group 22 to 60 years had ODS. The clinical presentations were diverse. Akinetic mutism was the commonest presenting feature of ODS. Four out of eight patients had hyperglycemia out of which three had diabetic ketoacidosis (DKA) and one was in hyperglycemic hyperosmolar state (HHS). Two patients with chronic kidney disease (CKD) developed myelinolysis following hemodialysis. One patient each in post liver transplant state and following alcohol binge were diagnosed with ODS. Serum sodium levels were in normal range and there was no undue fluctuation in all. Four had central pontine myelinolysis (CPM), three had Extrapontine myelinolysis (EPM) and one had both in Magnetic Resonance Imaging (MRI) of Brain. Background illnesses were addressed. Five patients were independent with mRS of 1 and one patient had mRS of 2 at the end of 3 months and two CKD patients succumbed due to disease per se. ODS commonly occurs in the setting of rapid correction of hyponatremia especially in chronic Conclusion: alcoholics and debilitated individuals. We have described myelinolysis in diabetic ketoacidosis, hyperglycemic hyperosmolar state, Renal failure following dialysis, post liver transplant and alcohol binge drinking where there were no undue fluctuation in sodium levels. The prognosis is variable and also depends on presence of secondary complications like deep venous thrombosis, sepsis and aspiration pneumonitis.
... The involvement of cortex suggests irreversible damage and poor prognosis of WE [129]. The MRI results also help in the diagnosis of extra-pontine and CPM also referred to as osmotic demyelination syndromes, which are associated with WE and rapid correction of severe hyponatremia in pregnant women [130][131][132][133][134][135]. Necropsy findings may help further in the diagnosis of WE and WKS but then it is too late [117]. ...
Background: Hyperemesis gravidarum tends to rapidly progress into Wernicke encephalopathy and Korsakoff syndrome and, therefore, needs to be recognized early and managed promptly with targeted multimodal therapies. Objective: This study critically reviewed the relevant literature on clinical perspectives of hyperemesis gravidarum, Wernicke encephalopathy and Korsakoff syndrome. The secondary objective of this study was to improve the awareness, emphasis on early diagnosis and immediate intervention concerning these sequential syndromes in pregnant women across the board. Methods: Electronic searches (since inception-2019) of PubMed/MEDLINE, Google Scholar, OvidSP, Dove Medical Press, ScienceDomain International (SDI) and Hindawi.com were conducted using keywords and Boolean Operators. Hundreds of thousands articles were retrieved which were reviewed independently by two authors and finally 144 articles retained that addressed clinical components of these sequential syndromes along with relevance of thiamine deficiency. Results: Evidently, vulnerable women in early stage of gestation tend to develop hyperemesis gravidarum characterized by persistent severe pernicious nausea and vomiting that causes Wernicke encephalopathy defined by variable oculomotor disturbances, ataxia, confusion, metabolic disturbances and Korsakoff syndrome linked with gross memory impairment, confabulation and constipation. The women with these conditions need diagnostic evaluation by means of clinical history, relevant laboratory tests, abdominal ultrasound and brain computerized tomography and magnetic resonance imaging. Most patients need emergency admission, prompt treatment with optimal doses of antiemetics, vitamin B1, and followed by fluid replenishment and electrolyte balance with follow up till the end of pregnancy. Successful maternal and fetal outcome of pregnancy depends on multiple determinants including associated systemic diseases. Conclusion: Evidently, a variety of etiological and risk factors in pregnant women determine the initiation of hyperemesis gravidarum that subsequently causes Wernicke encephalopathy and Korsakoff syndrome, and each of which needs prompt multimodal treatment in order to reduce maternal morbidity and increase successful outcome of pregnancy. Although clinical literature concerning these sequential syndromes is huge, further studies are needed to understand their underlying pathophysiological pathways across the world.
... In the ICU, CPEPM occurs in malnourished, usually alcoholic patients and in those with cirrhosis. Other risk factors are chronic renal failure necessitating hemodialysis and adrenal insufficiency (Morais et al., 2009). After liver transplantation, CPEPM is seen in patients with cirrhosis with pretransplantation serum hyponatremia, where large volumes of intravenous fluids were given in the operating theatre, resulting in an increase in plasma osmolality. ...
... In a series described by Abbasoglu et al. (1998), all patients expired within 3 months. Others report a mortality rate > 50% in the first 2 weeks and 90% after 6 months (Morais et al., 2009). However, some recent reports are less pessimistic. ...
Neurologic complications of critical illness require extensive clinical and neurophysiologic evaluation to establish a reliable prognosis. Many sequelae of intensive care unit (ICU) treatment, such as delirium and ICU-acquired weakness, although highly associated with adverse outcomes, are less suitable for prognostication, but should rather prompt clinicians to seek previously unnoticed persisting underlying illnesses. Prognostication can be confounded by drug administration particularly because its clearance is abnormal in critical illness. Some neurological complications are severe, and can last for months or years after discharge from ICU. The most important ethical aspects regarding neurologic complications in critically ill patients are prevention, recognition, and identification, and prevention of self-fulfilling prophecies. This chapter summarizes the tool of prognostication of major neurological complications of critical illness.
... • The association with alcoholism was the first to be noted and continues to be particularly frequent (in up to 40% of cases). It has been suggested that alcohol itself interferes with sodium/water regulation by suppression of antidiuretic hormone, and inadequate nutrition of alcoholics is an obvious accompaniment [64][65][66]. ...
... • CPM is also a recognized complication of liver transplantation. In a 10 year retrospective series of 627 transplants it occurred in 2% of cases (and contributed to the overall neurological complication rate of 26%) [64,65]. ...
... • Radiological confirmation is necessary to exclude other diagnosis and to determine the exact extension of the demyelination. Since CT can underestimate the real extension MRI plays a pivotal role in the determination of the presence, number and extension of the lesions [65]. ...
The present exhibit aims:
1. To appraise the wide array of neurological syndromes and disorderes that can
be associated with liver cirrhosis, highlighting their most pertinent neuroimaging
manifestations and providing an up-to-date information on these relatively understudied
entities.
2. To study and illustrate perioperative neurological complications which may originate
before, during or after orthotopic liver transplantation.
... One of the worst outcomes is the 'locked-in syndrome' [1,[12][13][14] , but other severe neurological deficits, coma and death have been described. One of the most common surgical scenarios in which CPM and EPM have been reported has been after liver transplantation [15][16][17][18][19][20][21][22][23] , but it has also been described in patients who develop diabetes insipidus or panhypopituitarism following intracranial surgery [24][25][26][27][28][29][30][31] . ...
Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are dire neurological disorders, characterized by severe damage to the myelin sheath of neurons, which typically result from rapid correction or overcorrection of systemic hyponatremia. For many years, both conditions have been considered universally fatal, though survivors have been reported more recently. Pediatric cases are rare. We present a 13-year-old boy with panhypopituitarism secondary to repair of a nasofrontal encephalocele in infancy, managed on long-term corticosteroid, deamino arginine vasopressin and thyroid hormone. He presented with severe hyponatremia (116 mEq/l), which during correction rapidly and unexpectedly increased to 176 mEq/l, resulting in profoundly impaired consciousness. Brain imaging revealed multiple bilateral changes in the basal ganglia, thalamus, pons and cerebral white matter, consistent with both CPM and EPM. Malignant cerebellar edema necessitated emergent suboccipital craniectomy, with subsequent improvement in level of consciousness and imaging postoperatively. However, he succumbed to acute cardiorespiratory arrest 8 weeks later. Nine similar cases from the literature are reviewed.
Osmotic demyelination syndrome is an uncommon neurologic condition, characterized by noninflammatory demyelination involving the pons and other areas of the central nervous system. As chronic hyponatremia is frequently associated with cirrhosis, patients undergoing liver transplantation are at an increased risk for developing this condition. We report the case of a patient who developed refractory hypernatremia and osmotic demyelination syndrome after liver transplantation. The patient was a 40-year-old man, who underwent liver transplantation for the treatment of cryptogenic cirrhosis, and had a preoperative sodium level of 128 mmol/L. Although there were no intraoperative complications, the patient showed signs of mental confusion and drowsiness in the second postoperative day, and we noticed an increase to 136 mmol/L in his serum sodium. Treatment with 5% dextrose and desmopressin was initiated, but his serum sodium continued to increase steadily, while his neurologic condition gradually worsened. Serum sodium rose to 157 mmol/L, and a magnetic resonance imaging of the brain showed extensive lesions consistent with osmotic demyelination syndrome. The clinical condition of the patient continued to deteriorate until his death 17 days after the transplant. Although the occurrence of this syndrome after liver transplantation is well described, the steady increase in serum sodium despite early treatment, as described in this case, is highly unusual, and highlights the great attention that must be taken with monitoring and control of serum sodium in patients who undergo liver transplant in the context of chronic hyponatremia. This manuscript is compliant with the Helsinki Congress and the Istanbul Declaration.
Liver transplantation has made great strides over the last decades towards a standardized procedure but still remains costly and resource-intensive. Given the fact that underlying acute liver failure and end-stage liver disease affect all physiologic systems and may cause hemodynamic, hematological, metabolic, and other homeostatic abnormalities, monitoring remains a key issue and prerequisite for success of liver transplantation.
Central pontine and extrapontine myelinolysis (CPM/EPM) are severe neurologic complications after liver transplantation.
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.
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.
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.
