Pontine Myelinolysis Research Articles

Central Pontine Myelinolysis Induced by Rapid Correction of Hyponatremia in a Patient With Chronic Alcohol Use Disorder: A Case Report

Central Pontine Myelinolysis Induced by Rapid Correction of Hyponatremia in a Patient With Chronic Alcohol Use Disorder: A Case Report

Evolution of radiological abnormalities in osmotic demyelination syndrome secondary to hyponatremia in LGI-1 autoimmune encephalitis.

Evolution of radiological abnormalities in osmotic demyelination syndrome secondary to hyponatremia in LGI-1 autoimmune encephalitis.

Rapid Correction of Measured Sodium Levels despite Relatively Stable Corrected Sodium Levels Can Lead to Osmotic Demyelination Syndrome

Rapid Correction of Measured Sodium Levels despite Relatively Stable Corrected Sodium Levels Can Lead to Osmotic Demyelination Syndrome

Síndrome de secreción inadecuada de hormona antidiurética

Syndrome of inappropriate antidiuretic hormone secretion (SIADH) is defined as a disorder in renal free water excretion control due to the inability to suppress the secretion of antidiuretic hormone (ADH) or vasopressin. The most frequent causes are usually acquired, notable among which are central nervous system involvement, malignancy, pulmonary diseases, or drugs. Up to 35% of admitted patients have hyponatremia, of which up to 40% can be attributed to SIADH. It is considered acute when it occurs in less than 48hours. It can trigger vomiting, convulsions, coma, and death due to cerebral herniation resulting from increased intracranial pressure. Chronic forms are more common and entail symptoms such as nausea, confusion, weakness, or agitation. In order to diagnose it, it is necessary to identify the presence of a true hypoosmolar euvolemic hyponatremia, ruling out other hormonal abnormalities or drug use. Treatment of the acute form is based on the emergency use of hypertonic saline solution, avoiding excessively rapid sodium correction that would trigger an osmotic demyelination syndrome. In the case of chronic hyponatremia, water restriction, increased osmole intake, and tolvaptan can be used in addition to acting on the triggering causes.

A recent ongoing effect: Hyponatremia in neurologically ill patients

Different variables altering the volume of extracellular fluid might result in hyponatremia, which is defined as a serum sodium content below 136 millimoles per liter. Depending on the volume status, it can be classified as hypovolemic, euvolemic, or hypervolemic. Hyponatremia is caused by a number of risk factors and drugs, such as diuretics, NSAIDs, and antidepressants. Hyponatremia is more common in patients with neurological conditions, especially those with head trauma, subarachnoid hemorrhage, or acute meningitis. Recent advancements in analysis for disorder of antidiuretic hormone syndrome (SIADH), a prevalent cause, include vasopressin-2 receptor antagonists. The intricate regulation of sodium and water balance by humoral, neurological, and renal mechanisms is a part of normal physiology. Hyponatremia may result from medications such as tramadol, sacubitril/valsartan, antidepressants, and anticonvulsants interfering with these mechanisms. Drug-induced hyponatremia is managed by stopping the offending agent, limiting fluid intake, and occasionally administering hypertonic saline. For chronic hyponatremia, the use of vaptans, like tolvaptan, in conjunction with urea therapy is growing. A risky complication of hyponatremia is osmotic demyelination syndrome, which can occur when the condition is treated too quickly. To avoid negative consequences, close observation and the right amount of fluids must be administered during treatment. To summarize, treating hyponatremia effectively entails determining the underlying causes, treating them, and making sure that serum sodium levels are carefully corrected to avoid consequences such as osmotic demyelination syndrome.

Sterile water and regional citrate anticoagulation: A simple CRRT strategy for safe correction of severe hyponatraemia.

Hyponatraemia is a prevalent electrolyte disturbance observed in critically ill patients. The rapid correction of low plasma sodium levels by continuous renal replacement therapy (CRRT) carries the risk of developing osmotic demyelination syndrome (ODS), which can be prevented by implementing an individualized CRRT method. This study aims to introduce a CRRT protocol for the safe and gradual correction of severe hyponatraemia. This retrospective case series study was conducted in an intensive care unit (ICU). All four patients with severe hyponatraemia (<125 mmol/L) and renal failure between October 1, 2022, and September 30, 2023, were treated by CRRT with sterile water and regional citrate anticoagulation (RCA). Data on patient demographics, laboratory biochemical parameters, urine outputs and CRRT-related adverse events were collected. Laboratory parameters and urine outputs were compared by paired t-tests before and after CRRT. After CRRT, sodium levels were significantly increased (112.7 ± 6.7 vs. 141.9 ± 2.8 mmol/L, p = .005). Abnormal urine outputs, potassium, creatinine and bicarbonate were corrected (p for all <.05). Safe and gradual correction of hyponatraemia and internal environmental dysregulation was achieved in all patients without any complications related to CRRT, particularly ODS. It is a novel and simple strategy to correct severe hyponatraemia effectively while ensuring the safety of patients that can be easily implemented by experienced nurse staff. The sterile water-based protocol for postfilter dilution is safe to correct severe hyponatraemia with RCA and can be easily performed by experienced critical care nurses according to the precalculated formula. CRRT-trained, experienced ICU nurses are competent to initiate and adjust sterile water infusion discretely to prevent overcorrection of hyponatraemia.

Role of clinicoradiological correlation in evaluation of various demyelinating disorders: A Systematic review

Background: There are mainly three types of white matter disorders resulting from defective myelination (dysmyelinating), destruction of myelin (demyelination) and decreased myelination (hypomyelinating). Each of these disorders require MRI and specific imaging for diagnosis. However, diagnosis of white matter disorders cannot solely rely on imaging. Objective: In this review we aim to correlate clinical presentation, history, laboratory investigations, and imaging as a tool rather than diagnostic modality to highlight the importance of clinical and radiological collaboration to diagnose the exact disease process. We would specifically discuss variants of multiple sclerosis, acute disseminated encephalomyelitis, neuromyelitis Optica spectrum disorder, progressive multifocal leuckoencephalothy, and osmotic demyelination syndrome. This study also includes specific signs of different demyelination white matter disorders on MRI which are characteristic of that disease process. However, we also highlight what clinical correlation and investigations may further aid to confirm the diagnosis, prognosis and extent of disease progression. Methods: In this review article we used Prisma guidelines we extracted studies where there was evidence of better patient outcome in terms of clinicoradiological collaboration in diagnosing various demyelinating white matter disorders. Results: We found that timely diagnosis and better patient outcomes can be achieved if clinicians also take in accord their own clinical judgement and based on that order relevant radiological investigations resulting better clinician to clinician communication, judicious use of hospital resources and overall better outcome in disease process. Conclusion: Our study concludes that solid clinical judgement, laboratory investigations along with radiological features of disease process would enhance clinical outcomes in terms of timely diagnosis, specific treatment and tracking disease prognosis.

Cavitating Osmotic Demyelination Syndrome Following Correction of Chronic Hyponatremia in Sheehan’s Syndrome: A Novel Case Report

Introduction Rapid correction of hyponatremia can result in osmotic demyelination syndrome (ODS). Sheehan’s syndrome, a rare pituitary disorder caused by severe postpartum hemorrhage, is a potential cause of chronic hyponatremia. This case report describes a rare progression of extrapontine myelinolysis to central pontine myelinolysis, ultimately leading to ODS, following the correction of chronic hyponatremia associated with Sheehan’s syndrome. Notably, this event occurred a decade after the initial postpartum hemorrhage due to placenta previa. Case Report A 40-year-old woman from rural West Bengal, India, presented in a comatose state after five years of progressively worsening symptoms, including fatigue, gastrointestinal disturbances, cold intolerance, hair loss, and severe apathy, which had been misdiagnosed as psychogenic and treated with selective serotonin reuptake inhibitors. Two days before her admission to our hospital, she was diagnosed with a lower respiratory tract infection, dehydration, and severe hyponatremia (118 mEq/L) at a local private healthcare facility. Despite treatment with 3% sodium chloride and intravenous antibiotics, her condition deteriorated, prompting her transfer. At the time of hospitalization, the patient was diagnosed with chronic hyponatremia and hypopituitarism consistent with Sheehan’s syndrome. This condition was attributed to a severe postpartum hemorrhage that occurred a decade prior, resulting from placenta previa. Initial MRI revealed extrapontine myelinolysis, and the correction of her “compensated” hyponatremia was identified as the cause of her neurological decline. Follow-up MRIs at 7 and 14 weeks confirmed the development of cavitating ODS. Discussion This case highlights several key points: First, even a relatively gradual correction of hyponatremia can precipitate ODS, especially in patients with chronic conditions like Sheehan’s syndrome. Second, it underscores the importance of meticulous management of chronic hyponatremia to prevent severe neurological outcomes. Third, it illustrates the diagnostic challenges of differentiating Sheehan’s syndrome from primary psychiatric disorders, particularly in low-resource settings where the syndrome remains prevalent. The case also emphasizes the need for awareness among healthcare providers about the potential for severe complications arising from even minor corrections in serum sodium levels in such patients.

Secondary parkinsonism associated with focal brain lesions.

Focal imaging abnormalities in patients with parkinsonism suggest secondary etiology and require a distinctive clinical approach to diagnosis and treatment. We review different entities presenting as secondary parkinsonism associated with structural brain lesions, with emphasis on the clinical course and neuroimaging findings. Secondary parkinsonism may be due to vascular causes, hydrocephalus, space-occupying lesions, metabolic causes (including acquired hepatocerebral degeneration, diabetic uremic encephalopathy, basal ganglia calcifications, osmotic demyelination syndrome), hypoxic-ischaemic brain injury, intoxications (including methanol, carbon monoxide, cyanide, carbon disulfide, manganese poisoning and illicit drugs), infections and immune causes. The onset can vary from acute to chronic. Both uni-and bilateral presentations are possible. Rigidity, bradykinesia and gait abnormalities are more common than rest tremor. Coexisting other movement disorders and additional associated neurological signs may point to the underlying diagnosis. Neuroimaging studies are an essential part in the diagnostic work-up of secondary parkinsonism and may point directly to the underlying etiology. We focus primarily on magnetic resonance imaging to illustrate how structural imaging combined with neurological assessment can lead to diagnosis. It is crucial that typical imaging abnormalities are recognized within the relevant clinical context. Many forms of secondary parkinsonism are reversible with elimination of the specific cause, while some may benefit from symptomatic treatment. This heterogeneous group of acquired disorders has also helped shape our knowledge of Parkinson's disease and basal ganglia pathophysiology, while more recent findings in the field garner support for the network perspective on brain function and neurological disorders.

Prolonged extracellular low sodium concentrations and subsequent their rapid correction modulate nitric oxide production dependent on NFAT5 in microglia

Hyponatremia is the most common clinical electrolyte disorder. Chronic hyponatremia has been recently reported to be associated with falls, fracture, osteoporosis, neurocognitive impairment, and mental manifestations. In the treatment of chronic hyponatremia, overly rapid correction of hyponatremia can cause osmotic demyelination syndrome (ODS), a central demyelinating disease that is also associated with neurological morbidity and mortality. Using a rat model, we have previously shown that microglia play a critical role in the pathogenesis of ODS. However, the direct effect of rapid correction of hyponatremia on microglia is unknown. Furthermore, the effect of chronic hyponatremia on microglia remains elusive. Using microglial cell lines BV-2 and 6-3, we show here that low extracellular sodium concentrations (36 mmol/L decrease; LS) suppress Nos2 mRNA expression and nitric oxide (NO) production of microglia. On rapid correction of low sodium concentrations, NO production was significantly increased in both cells, suggesting that acute correction of hyponatremia partly directly contributes to increased Nos2 mRNA expression and NO release in ODS pathophysiology. LS also suppressed expression and nuclear translocation of nuclear factor of activated T cells-5 (NFAT5), a transcription factor that regulates the expression of genes involved in osmotic stress. Furthermore, overexpression of NFAT5 significantly increased Nos2 mRNA expression and NO production in BV-2 cells. Expressions of Nos2 and Nfat5 mRNA were also modulated in microglia isolated from cerebral cortex in chronic hyponatremia model mice. These data indicate that LS modulates microglial NO production dependent on NFAT5 and suggest that microglia contribute to hyponatremia-induced neuronal dysfunctions.

Unveiling the Patterns of Water Diuresis in Profound Hyponatremia Management in Intensive Care Unit Settings.

Hyponatremia treatment guidelines recommend avoiding excessive increases in serum sodium concentration (s[Na]) to prevent osmotic demyelination syndrome. Although an unexpected rise in s[Na] has been attributed to water diuresis during the treatment of hyponatremia, clinical courses of water diuresis are unclear. We conducted this study to investigate the clinical characteristics of water diuresis during profound hyponatremia management. In this retrospective observational study, we examined patients with profound hyponatremia (s[Na] ≤120 mEq/L) admitted to the intensive care unit of a Japanese hospital. The manifestation of water diuresis was defined as a urine volume ≥2 ml/kg/h and a urinary sodium plus potassium concentration (u[Na+K]) ≤50 mEq/L. We analyzed changes in urine volume and u[Na+K] over time for patients experiencing water diuresis. This analysis employed a mixed-effects model with spline terms for time, and the results are graphically presented. Among 47 eligible patients, 30 (64%) met the criteria for water diuresis. The etiologies of hyponatremia were drug-related hyponatremia (n=10; 33%), primary polydipsia (n=8; 27%), hypovolemic hyponatremia (n=7; 23%), syndrome of inappropriate secretion of antidiuresis (n=7; 23%), and acute heart failure (n=1; 3%). Among patients with water diuresis, 27 (90%) experienced the manifestation of water diuresis within 24 hours after the start of correction. The increased urine volume and decreased u[Na+K] levels began several hours before the peak manifestation of water diuresis. Within 6 hours after the manifestation of water diuresis, 29 patients (97%) received electrolyte-free infusions and 14 (47%) received desmopressin. One patient (3%) with water diuresis experienced overcorrection. Water diuresis is common during the treatment for profound hyponatremia and typically occurs within the first 24 hours, preceded by changes in urinary characteristics. Early detection and prompt response to water diuresis through urine monitoring during the early periods of hyponatremia treatment may be effective for managing water diuresis.

Exploring Uncommon Neurologic Complication in Young Diabetics with Osmotic Demyelination Syndrome: A Case Series and Literature Review.

Osmotic demyelination syndrome (ODS) is well known to be associated with the rapid correction of hyponatremia. However, there is limited literature on its link to persistent or corrected hyperglycemic states in diabetic patients. We report two young patients with ODS and hyperglycemia. Neuroimaging and blood tests indicated central pontine and extrapontine myelinolysis with a hyperglycemic hyperosmolar state (HHS). These unusual cases provide insight into the neurologic complications of hyperglycemia.

Multisystem Imaging Manifestations of Kidney Failure.

Kidney failure (KF) refers to a progressive decline in glomerular filtration rate to below 15 ml/min per 1.73 m2, necessitating renal replacement therapy with dialysis or renal transplant. The hemodynamic and metabolic alterations in KF combined with a proinflammatory and coagulopathic state leads to complex multisystemic complications. The imaging hallmark of systemic manifestations of KF is bone resorption caused by secondary hyperparathyroidism. Other musculoskeletal complications include brown tumor, osteosclerosis, calcinosis, soft-tissue calcification, and amyloid arthropathy. Cardiovascular complications and infections are the leading causes of death in KF. Cardiovascular complications include accelerated atherosclerosis, cardiomyopathy, pericarditis, myocardial calcinosis, and venous thromboembolism. Neurologic complications such as encephalopathy, osmotic demyelination, cerebrovascular disease, and opportunistic infections are also frequently encountered. Pulmonary complications include edema and calcifications. Radiography and CT are used in assessing musculoskeletal and thoracic complications, while MRI plays a key role in assessing neurologic and cardiovascular complications. CT iodinated contrast material is generally avoided in patients with KF except in situations where the benefit of contrast-enhanced CT outweighs the risks and in patients already undergoing maintenance dialysis. At MRI, group II gadolinium-based contrast material can be safely administered in patients with KF. The authors discuss the extrarenal systemic manifestations of KF, the choice of imaging modality in their assessment, and imaging findings of complications. ©RSNA, 2024 Supplemental material is available for this article.

Pre-operative sodium benzoate can rapid increase sodium level and induce central pontine myelinolysis after liver transplantation: case report

Pre-operative sodium benzoate can rapid increase sodium level and induce central pontine myelinolysis after liver transplantation: case report

Hypertonic saline for ischemic stroke: a review

Background and Objective: Elevated intracranial pressure (ICP) is a critical complication of ischemic stroke. This review article summarizes existing literature pertaining to the role of hypertonic saline (HTS) in lowering ICP in ischemic stroke patients. Methods: Studies were selected using a comprehensive search of several research databases. Studies conducted in adult (aged 18 and older) human patients receiving HTS osmotherapy for elevated ICP were included, encompassing a wide range of neurological conditions and various administration methods, durations, and concentrations of HTS. Results: We found HTS to reduce ICP in ischemic stroke patients, on par effectively, and in some studies, superior to more conventional ICP-lowering methods such as mannitol. Patients with comorbid conditions such as cerebrovascular and renal diseases also tolerated HTS well. The risk of developing hypernatremia was significantly higher in patients receiving HTS osmotherapy. Neurological complications, such as central pontine myelinosis, were not observed in any of the participants; however, no clear benefit regarding the long-term neurologic outcome of these patients has been reported thus far. Conclusion: While encouraging, existing literature on the use of HTS as a treatment for elevated ICP still needs to be more conclusive and necessitates further research. Questions regarding duration, optimal concentration of intervention, and method of administration need to be addressed by future randomized controlled trials.

Clinical factors associated with hyponatremia correction during treatment with oral Urea.

Oral urea is being used more commonly to treat hyponatremia, but factors contributing to the correction rate are unknown. We hypothesized that clinically relevant factors can be identified to help guide hyponatremia correction with oral urea. Retrospective study in two university hospitals including hospitalized patients with hyponatremia (plasma sodium < 135mmol/L) treated with oral urea. Linear mixed-effects models were used to identify factors associated with hyponatremia correction. Rates of overcorrection, osmotic demyelination and treatment discontinuation were also assessed. We included 161 urea treatment episodes in 140 patients (median age 69 years, 46% females, 93% syndrome of inappropriate antidiuresis). Oral urea succeeded fluid restriction in 117 treatment episodes (73%), was combined with fluid restriction in 104 treatment episodes (65%) and was given as only treatment in 27 treatment episodes (17%). A median dose of 30 grams/day of urea for 4 days (interquartile range 2-7 days) increased plasma sodium from 127 to 134mmol/L and normalized hyponatremia in 47% of treatment episodes. Older age (ß 0.09, 95%CI 0.02 to 0.16), lower baseline plasma sodium (ß -0.65, 95%CI -0.78 to -0.62), and higher cumulative urea dose (ß 0.03, 95%CI -0.02 to -0.03) were independently associated with a greater rise in plasma sodium. Concurrent fluid restriction was associated with a greater rise in plasma sodium only during the first 48h of treatment (ß 1.81, 95%CI 0.40 to 3.08). Overcorrection occurred in 5 cases (3%), no cases of osmotic demyelination were identified, and oral urea was discontinued in 11 cases (11%) due to side-effects. During treatment with oral urea, older age, higher cumulative dose, lower baseline plasma sodium and initial fluid restriction are associated with a greater correction rate of hyponatremia. These factors may guide clinicians to achieve a gradual correction of hyponatremia with oral urea.

Lingual Fasciculation as a Point of Call for the Diagnosis of Amyotrophic Lateral Sclerosis: A Literature Review.

Dental surgeons often play a pivotal role in the initial detection of lingual fasciculations (LFs). These involuntary micro-movements of the tongue can serve as early clinical indicators of neurodegenerative diseases, with amyotrophic lateral sclerosis (ALS) being the most concerning. Therefore, it is imperative to educate dental surgeons on identifying LF and understanding the potential underlying pathologies. This study aimed to pinpoint the pathologies in which LFs could emerge as an early clinical marker. Our review focused on articles delineating patient populations exhibiting LF within broader pathological contexts, encompassing neurological and other conditions, with the aim of elucidating their etiologies. We conducted a comprehensive literature review across four databases (PubMed, Embase, Web of Science, and Scopus). Two authors independently extracted data, with consultation from a third author when necessary. Eligible articles included those describing patients with LFs, detailing the methods of detection, diagnosis, and associated pathologies. Our review identified 22 articles encompassing 153 patients with LF, with an average age of 45.8 years and a female prevalence of 43%. Electromyography and ultrasound emerged as the predominant detection methods. ALS constituted the primary diagnosis in the majority of cases (91%). Additionally, other conditions diagnosed included Machado-Joseph disease (0.046%), familial transthyretin amyloid neuropathy (0.013%), Brown-Vialetto-Van-Laere syndrome (0.006%), chronic inflammatory demyelinating polyneuropathy (0.006%), bulbospinal amyotrophy or Kennedy's disease (0.006%), and osmotic demyelination syndrome (0.006%). LF secondary to organophosphate poisoning was also documented. Symptoms associated with LF encompassed taste alterations, dysphagia, difficulty swallowing, and slurred speech. While primarily indicative of ALS, LFs may also signal diverse underlying pathologies. Healthcare practitioners should be vigilant in their detection and expedite patient referrals to facilitate early integration into care protocols.

Wolf in sheep’s clothing

Autoimmune encephalitis (AE) is an immune-mediated disease with a varied clinical spectrum. Osmotic demyelination is a condition characterized by hypernatremia following an episode of correction for hyponatremia. Our case report is about a patient who presented with features of central diabetes insipidus and an imaging diagnosis of osmotic demyelination. Magnetic resonance imaging of the brain, which later turned out to be AE based on the positive auto-antibodies.

Rate of Sodium Correction and Osmotic Demyelination Syndrome in Severe Hyponatremia: A Meta-Analysis.

Current guidelines recommend limiting the rate of correction in patients with severe hyponatremia to avoid severe neurologic complications such as osmotic demyelination syndrome (ODS). However, published data have been conflicting. We aimed to evaluate the association between rapid sodium correction and ODS in patients with severe hyponatremia. We searched PubMed, Embase, Scopus, Web of Science, and Cochrane Central Register of Controlled Trials from inception to November 2023. The primary outcome was ODS and the secondary outcomes were in-hospital mortality and length of hospital stay. We identified 7 cohort studies involving 6,032 adult patients with severe hyponatremia. Twenty-nine patients developed ODS, resulting in an incidence rate of 0.48%. Seventeen patients (61%) had a rapid correction of serum sodium in the first or any 24-hour period of admission. Compared with a limited rate of sodium correction, a rapid rate of sodium correction was associated with an increased risk of ODS (RR, 3.91 [95% CI, 1.17 to 13.04]; I2 = 44.47%; p = 0.03). However, a rapid rate of sodium correction reduced the risk of in-hospital mortality by approximately 50% (RR, 0.51 [95% CI, 0.39 to 0.66]; I2 = 0.11%; p < 0.001) and the length of stay by 1.3 days (Mean difference, -1.32 [95% CI, -2.54 to -0.10]; I2 = 71.47%; p = 0.03). Rapid correction of serum sodium may increase the risk of ODS among patients hospitalized with severe hyponatremia. However, ODS may occur in patients regardless of the rate of serum sodium correction.

Hyperintensity in Bilateral Posterior Limbs of Internal Capsule Due to Hypernatremia

Abstract Purpose: Osmotic demyelination syndrome is usually caused by rapid correction of hyponatremia but sometimes develops from acute severe hypernatremia. Studies suggested that serum sodium increasing at the rate of &lt;6~8 mmol/L in 24 hours has a low risk of osmotic demyelination syndrome, but sometimes exceptions present. Aside from the classical sites of involvement, such as pons and basal ganglia, internal capsules are rarely affected. We report a case with acute paraparesis caused by acute hypernatremia-induced extrapontine myelinolysis involving the posterior limbs of bilateral internal capsules. Case: A 54-year-old man was admitted for aseptic encephalitis and moved to the intensive care unit due to poor consciousness and respiratory failure. Although cerebrospinal fluid pleocytosis was improved later, acute hypernatremia due to partial diabetes insipidus developed. He presented acute paraplegia two days later with a negative result on the whole spine MRI. Although the increasing rate of serum sodium did not exceed the recommended safety range, the extrapontine myelinolysis involving posterior limbs of the bilateral internal capsule, as long as the corpus callosum, still developed. The patient regained partial walking ability after one year. Conclusion: This case report extends the spectrum of classical osmotic demyelination in clinical manifestations, image findings, and the causal range of electrolyte derangements.