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Magnetic resonance imaging (MRI) of patient 1, showing lesions typical of central pontine myelinolysis, with high T2-weighted MRI signal (water-containing structures appear white) and diffusion restriction within the pons, sparing descending tracts. Findings are consistent with central pontine myelinolysis and are shown to illustrate the severity of the osmotic demyelination syndrome. Patients with this type of lesion are unable to receive or send impulses below the level of the midbrain, while higher cortical functions remain essentially intact ("locked-in" syndrome).
Source publication
OBJECTIVE: To report 2 cases of central pontine myelinolysis (CPM) post liver transplantation in which treatment with plasmapheresis and intravenous immune globulin improved expected neurologic outcome. CASE SUMMARY: Two patients who underwent orthotopic liver transplant developed CPM early in their postoperative course. Magnetic resonance imaging...
Context in source publication
Context 1
... immediate MRI scan documented multiple lesions with a high T2-weighted signal along with a diffu- sion restriction within the pons. While a differential diag- nosis in this setting would typically include metabolic en- cephalopathy, calcineurin inhibitor toxicity, intracranial hemorrhage, central nervous system mass lesion, or undi- agnosed seizure activity, clinical and radiographic findings were considered pathognomonic of severe central pontine myelinolysis (Figure 2). ...
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Objective:
This is a review on clinical presentation, diagnosis, and treatment of reported cases of Bickerstaff brain encephalitis.
Materials and methods:
Cases of pediatric Bickerstaff's brainstem encephalitis collected from PubMed, Cochrane Library and Scopus Web of Science databases were reviewed. The inclusion criteria of the cases were base...
Citations
... Apart from supportive therapy and experimental therapies, no definitive treatment has been established for ODS. Ludwig et al. reported 2 cases of ODS in which treatment with plasmapheresis and intravenous immune globulin improved long-term neurologic outcomes (26). Kengne (29). ...
Background
Osmotic demyelination syndrome (ODS) has a low incidence but is a life-threatening neurological disorder whose common cause is rapid overcorrection of chronic hyponatremia. Transcatheter aortic valve replacement (TAVR) is a new and important therapy for patients with aortic valve stenosis. In this article, we discuss the case of a 64-year-old woman who developed ODS after TAVR and provide a literature review.
Case Presentation
A 64-year-old female patient was admitted to the hospital with chest tightness, shortness of breath, and fatigue for 2 months, with worsening of symptoms for 3 days prior to presentation. Auscultation revealed crackles in the lung fields, and systolic murmurs could be easily heard in the aortic area. Echocardiography showed severe aortic stenosis. Chest X-ray showed pulmonary oedema. Laboratory examinations showed that her serum sodium was 135 mmol/L. The patient received a diuretic to relieve her symptoms but showed little benefit. Her symptoms worsened, and her blood pressure dropped. Then, she underwent emergency TAVR under extracorporeal membrane oxygenation (ECMO) support. After the operation, her urine output increased markedly, and serum sodium increased sharply from 140 to 172 mmol/L. An MRI scan showed multiple lesions in the pons suggestive of ODS.
Conclusion
To date, this is the first reported case of a patient who developed ODS after receiving TAVR. In current clinical practice, diuretics are often used in aortic stenosis patients because of pulmonary oedema. After a patient receives TAVR, kidney perfusion pressure quickly returns to normal, and with the residual effect of a high-dose diuretic, balances of fluid volume and electrolyte levels in this phase are quite fragile and must be carefully managed. If a patient has neurological symptoms/signs during this phase, ODS should be considered, and MRI might be necessary.
... 14-17 Treatment with both PP and IVIG has been trialed as well with significant improvement in four patients. 10,18,19 The use of intravenous methylprednisolone has been described as well, however, further studies are needed to establish the true efficacy of the use of steroids in this setting. 3,20 Case reports usually describe regimens with successful outcomes. ...
... In the AMBAR trial, IVIG is used to correct a possible immunological deficit caused by plasma depletion. In fact, this combination approach of PE with albumin plus IVIG replacement has already been used for selected neurologic diseases [60,61]. The AMBAR trial evaluates the maintenance period including combination of monthly LVPE (similar to regular plasma donation plasmaphereses) with IVIG every 4 months. ...
Introduction
Preliminary studies have shown that treatment with plasma exchange (PE) plus therapeutic albumin replacement in patients with Alzheimer's disease (AD) induced mobilization of plasma and cerebrospinal fluid amyloid β protein, associated with an improvement in memory and language functions, as well as the stabilization of brain perfusion, which persisted after treatment discontinuation.
Methods
Alzheimer's Management By Albumin Replacement (AMBAR) is a multicenter, randomized, blinded and placebo-controlled, parallel-group, phase IIb/III trial enrolling patients with mild to moderate AD. The study evaluates PE with different replacement volumes of therapeutic albumin (5% and 20% Albutein®, Grifols), with or without intravenous immunoglobulin (Flebogamma® 5% DIF, Grifols). Patients are randomized to one of three active treatment groups or one control (sham PE) group (1:1:1:1). The intervention regime includes a first 6-week stage of intensive treatment, followed by a second 12-month stage of maintenance treatment. The change from the baseline to the end of treatment periods in the ADAS-Cog and ADCS-ADL scores are the coprimary efficacy variables. Secondary efficacy variables include change from the baseline in scores on cognitive, functional, behavioral, and overall progression tests; changes in plasma and cerebrospinal fluid levels of amyloid β and tau protein; and assessment of structural and functional changes in brain areas of interest. Safety and tolerability are assessed.
Results
The study has enrolled 496 patients from 41 centers (19 in Spain and 22 in the USA); 347 of these patients were randomized and underwent close to 5000 PEs, of which approximately 25% were sham PEs.
Discussion
We present an innovative approach for treating AD. The study has been designed to demonstrate clinical efficacy, defined as slow decline of the patient's cognition and brain function. The sample size has adequate power to detect differences between any of the active treatment groups and the control group, as well as between the three active treatment groups combined and the control group.
... Although no abrupt changes in sodium levels or osmolarity were detected, the patient's baseline hypothalamic dysfunction may have predisposed to central pontine myelinolysis during the acute process. After reviewing the literature available on the topic, 8,9 we decided to administer IV immunoglobulins (0.4 g/kg for 5 days). Treatment led to significant improvements over the following weeks: the patient's level of consciousness improved progressively and she displayed spontaneous mobility of all 4 limbs, started articulating speech, and was able to chew, swallow, and even walk. ...
Introduction
ROHHAD syndrome (rapid-onset obesity with hypothalamic dysregulation, hypoventilation, and autonomic dysregulation) is a rare and complex disease, presenting in previously healthy children at the age of 2-4 years. Up to 40% of cases are associated with neural crest tumours.
Development
We present the case of a 2-year-old girl with symptoms of rapidly progressing obesity, who a few months later developed hypothalamic dysfunction with severe electrolyte imbalance, behaviour disorder, hypoventilation, and severe autonomic dysregulation, among other symptoms. Although the pathophysiology of this syndrome remains unclear, an autoimmune hypothesis has been proposed for ROHHAD. Therefore, after obtaining a limited response to intravenous immunoglobulins, we decided to test the response to a high dose cyclophosphamide (low dose was not effective either). Unfortunately our patient experienced many severe complications (among them central pontine myelinolysis, from which the patient recovered, and failure to wean from the ventilator requiring tracheostomy and long term ventilation) that required a prolonged ICU stay. Although her behaviour improved, our patient unfortunately died suddenly at home at the age of 5 due to respiratory pathology.
Conclusions
ROHHAD syndrome is a rare and little-known disease which requires a multidisciplinary approach because it involves complex symptoms and multiple organ system involvement. Alveolar hypoventilation should be identified early and appropriate treatment should be started promptly for the best possible outcome. Immunomodulatory treatment with immunoglobulins, cyclophosphamide, or rituximab has previously resulted in symptom improvement in some cases. Because of the low incidence of the syndrome, multi-centre studies must be carried out in order to gather more accurate information about ROHHAD pathophysiology and design an appropriate therapeutic approach.
... Once ODS has been established, care is mainly supportive, requiring the assistance of the transplant team, neurology, and physical and occupational therapy. Case reports in the post-LT setting have demonstrated significant improvement in neurologic symptoms with the use of plasmapheresis combined with intravenous immunoglobulin infusion (44). Most LT physicians seek to maintain lower calcineurin inhibitor trough levels in order to minimize their potential confounding neurologic side effects. ...
The osmotic demyelination syndrome (ODS) is a serious neurologic condition that occurs in the setting of rapid correction of hyponatremia. It presents with protean manifestations, from encephalopathy to the “locked-in” syndrome. ODS can complicate liver transplantation (LT), and its incidence may increase with the inclusion of serum sodium as a factor in the Model for End-Stage Liver Disease score. A comprehensive understanding of risk factors for the development of ODS in the setting of LT, along with recommendations to mitigate the risk of ODS, are necessary. The literature to date on ODS in the setting of LT was reviewed. Major risk factors for the development of ODS include severe pre-transplant hyponatremia (Serum sodium [SNa] < 125 mEq/L), the magnitude of change in SNa pre- vs. post-transplant, higher positive intraoperative fluid balance, and the presence of postoperative hemorrhagic complications. Strategies to reduce the risk of ODS include correcting hyponatremia pre-transplant via fluid restriction and/or ensuring an appropriate rate of increase from the preoperative SNa via close attention to fluid and electrolyte management both during and after surgery. Multidisciplinary management involving transplant hepatology, nephrology, neurology, surgery, and anesthesiology/critical care is key to performing LT safely in patients with hyponatremia. This article is protected by copyright. All rights reserved.
... При этом указания на существенную гипонатриемию отсутствовали. Массивная инфузионная или диуретическая терапия непосредственно перед клиническим ухудшением также не проводилась, а плазмаферез, по мнению некоторых авторов [56], может способствовать улучшению исходов ЦПМ. ...
A case report of central pontine and extrapontine myelinolysis in the combination with spinal cord damage is presented. The authors analyze literature data on this problem and discuss the pathogenesis and diagnostic issues of myelinolysis.
... Although no abrupt changes in sodium levels or osmolarity were detected, the patient's baseline hypothalamic dysfunction may have predisposed to central pontine myelinolysis during the acute process. After reviewing the literature available on the topic, 8,9 we decided to administer IV immunoglobulins (0.4 g/kg for 5 days). Treatment led to significant improvements over the following weeks: the patient's level of consciousness improved progressively and she displayed spontaneous mobility of all 4 limbs, started articulating speech, and was able to chew, swallow, and even walk. ...
Introduction:
ROHHAD syndrome (rapid-onset obesity with hypothalamic dysregulation, hypoventilation, and autonomic dysregulation) is a rare and complex disease, presenting in previously healthy children at the age of 2-4 years. Up to 40% of cases are associated with neural crest tumours.
Development:
We present the case of a 2-year-old girl with symptoms of rapidly progressing obesity, who a few months later developed hypothalamic dysfunction with severe electrolyte imbalance, behaviour disorder, hypoventilation, and severe autonomic dysregulation, among other symptoms. Although the pathophysiology of this syndrome remains unclear, an autoimmune hypothesis has been proposed for ROHHAD. Therefore, after obtaining a limited response to intravenous immunoglobulins, we decided to test the response to a high dose cyclophosphamide (low dose was not effective either). Unfortunately our patient experienced many severe complications (among them central pontine myelinolysis, from which the patient recovered, and failure to wean from the ventilator requiring tracheostomy and long term ventilation) that required a prolonged ICU stay. Although her behaviour improved, our patient unfortunately died suddenly at home at the age of 5 due to respiratory pathology.
Conclusions:
ROHHAD syndrome is a rare and little-known disease which requires a multidisciplinary approach because it involves complex symptoms and multiple organ system involvement. Alveolar hypoventilation should be identified early and appropriate treatment should be started promptly for the best possible outcome. Immunomodulatory treatment with immunoglobulins, cyclophosphamide, or rituximab has previously resulted in symptom improvement in some cases. Because of the low incidence of the syndrome, multi-centre studies must be carried out in order to gather more accurate information about ROHHAD pathophysiology and design an appropriate therapeutic approach.
... During OLT surgery, the liver transplant anesthesia team frequently checks the blood gas and serum sodium levels and tries to avoid an excessive amount of blood products and/or albumin infusions to prevent overcorrecting the serum sodium level, especially for patients with pretransplant hyponatremia. In terms of posttransplant management, it is important to promote high suspicion for ODS and to consider prompt initiation of plasmapheresis and immunoglobulins as a treatment for ODS [15,16]. ...
Background
Serum sodium (Na) is considered to reflect the severity of liver cirrhosis. In the last few years, much effort has been made to integrate this association into prognostic models after liver transplantation. The aim of this study was to investigate the associations between peritransplant Na and neurological complications, as well as short-term survival, after liver transplantation.
Material/Methods
A total of 306 liver transplantations between 2012 and 2015 were evaluated. Pre- and posttransplant sodium concentrations were investigated with regard to 3-month survival and incidence of posttransplant neurological complications, along with other factors present in the operative side of the recipient and donor.
Results
The 3-month survival rate was 94%. Neither hyponatremia (<130 mEq/L) nor hypernatremia (>145 mEq/L) at pretransplantion predicted 3-month survival. A large amount of intraoperative blood transfusion and a large delta Na showed a significant association with poor outcomes at 3 months. On multivariate analysis, the requirement of blood transfusion and warm ischemia time remained independent prognostic factors for 3-month mortality. Hyponatremia and a large delta Na tended to lead to the frequent development of neurological complications. These complications, secondary to rapid Na correction, were concerning and potentially led to a prolonged hospital stay and early mortality.
Conclusions
Rapid change in the sodium level might be caused by large amounts of blood transfusion products. This leads to a diminished short-term survival, as well as a higher rate of neurological complications.
... In the literature, there are separate reports on the use of high doses of corticosteroid hormones [16] and plasmapheresis for the treatment of EPM [17][18][19]. The Fig. 2 (a-d). ...
Topicality:
Central pontine and extrapontine myelinolysis is a rare and dangerous form of the demyelinating process of undefined origin, the development of which is associated with a very low sodium level in the blood (hypernatremia).
Objective:
To describe a rare case of extrapontine myelinolysis as a complication occurring in the hemorrhagic period of anterior communicating artery aneurysm rupture as well as to demonstrate that this condition can be diagnosed intravitally.
Conclusion:
Clinical vigilance of extrapontine myelinolysis may promote the timely diagnosis and treatment of this disease, which is a potential cause of death. Pulse-therapy with glucocorticoids provides the opportunity to achieve regression of neurological symptoms and to stabilize the patient's condition.
... Bibl, et al, reportaron tres casos de MCP tratados con plasmaféresis, todos con mejoría clínica en diversos periodos de tiempo. Ludwig, et al, reportaron dos casos de MCP posterior a trasplante hepático, quienes recibieron plasmaféresis e inmunoglobulinas por seis y cinco días respectivamente, mostrando mejoría clínica a las dos semanas de tratamiento y resolución clínica al año de haber recibido el tratamiento 39 . ...
La mielinolisis central pontina (MCP) es una enfermedad desmielinizante no inflamatoria caracterizada por destrucción de las vainas de mielina a nivel del puente de Varolio. Los factores predisponentes son alcoholismo crónico, estados de desnutrición y corrección rápida de una disnatremia. Reporte de caso: femenino de 54 años de edad quien presentó cuadriparesia y afasia súbitas, inicialmente manejada como evento vascular cerebral. Después presenta nistagmus vertical. Se realiza resonancia magnética de cráneo simple, la cual mostró datos compatibles con mielinolisis central pontina. La citometría hemática con anemia megaloblástica. Recibió tratamiento de soporte y rehabilitación física, sin mejoría del cuadro clínico. Conclusiones: este caso relaciona la MCP con hipovitaminosis, asociación poco frecuente en el desarrollo de una MCP. No se corroboraron niveles séricos de complejo B, lo cual nos hubiese permitido definir un diagnóstico más preciso. Hasta este momento, no existe un tratamiento efectivo para la MCP.
