Search

Background : Laryngomalacia is a congenital abnormality of the larynx that commonly occurs in children and rarely in adults. We report the first case of acquired laryngomalacia mainly due to postoperative seizure and central pontine myelinolysis after scheduled craniotomy. Case presentation : A 69-year-old man was admitted to the hospital for elective craniotomy for craniopharyngioma. After the surgery, he developed refractory seizure and required intubation and mechanical ventilation in the intensive-care unit (ICU). After treatment for the seizure, he was extubated. However, immediately after extubation, he developed stridor and respiratory retraction. We performed fiberoptic laryngoscopy and confirmed that the epiglottis had collapsed into the posterior wall of the pharynx during inspiration, which was suspected to be laryngomalacia. He received invasive mechanical ventilation for two days following re-extubation. After the second extubation, he developed stridor again due to acquired laryngomalacia. Six days later, his respiratory condition had worsened, and he received re-intubation and tracheostomy. After ICU discharge, central pontine myelinolysis was diagnosed by magnetic resonance imaging. Conclusions : Adult-onset laryngomalacia is a rare cause of upper airway obstruction but should be considered as a cause of postoperative extubation failure. We should not delay performing fiberoptic laryngoscopy to evaluate this pathology and provide optimal treatment. J. Med. Invest. 69 : 316-319, August, 2022.

Central pontine myelinolysis is most commonly associated with rapid correction of hyponatremia and has historically been associated with alcoholism. In this case report, 2 deaths with gross findings of central pontine lesions led to the possibility that CPM may have been a potential mechanism of death. Subsequent analysis revealed that these lesions were incidental findings. This case report discusses the importance of appropriate microscopic and immunohistochemical analysis of suspected CPM cases.

Background Osmotic demyelination syndrome commonly occurs after rapid correction of hyponatremia. But it has also been reported after graded correction of hyponatremia in the presence of other risk factors like chronic alcoholism, malnutrition, liver disease, and hypokalemia. Case Report We report a case of a 67-year-old man who presented with dysphagia and nasal regurgitation and had features suggestive of bulbar palsy on neurological examination. He had spontaneous rapid correction of hyponatremia from a serum sodium level of 122 mEq/L to 132 mEq/L after discharge from our hospital. Neuroimaging was suggestive of extrapontine myelinolysis involving the basal ganglia. Why Should an Emergency Physician Be Aware of This? An emergency physician should be aware of this because osmotic demyelination syndrome should also be considered in the differential diagnosis of patients presenting with bulbar palsy to the emergency department.

Osmotic demyelination syndrome (ODS) and neuroleptic malignant syndrome (NMS) lead to severe neurological sequalae. Though currently thought to be different syndromes, literature suggests a relation between the two. We present the case of a 45-year-old male who was found to have chronic severe hyponatremia and underwent rapid correction of sodium and developed parkinsonism features. Magnetic resonance imaging (MRI) confirmed extrapontine myelinolysis (a type of ODS). The patient received haloperidol for agitated behavior and developed new features of rigidity, fever, tachycardia and elevated creatine phosphokinase (CPK) levels and thus neuroleptic malignant syndrome was suspected to overlap with ODS. We report this case highlighting the difficulty in differentiating the between ODS and NMS and their relationship.

A 76-year-old male presented with a recurrent depressive episode, an unsteady gait and cognitive impairment. Extensive blood tests, including hemogram, biochemical tests, folic acid, vitamin B12, and thyroid hormone, showed normal results. With the exception of the unsteady gait, neurological examination was negative. Brian magnetic resonance imaging (MRI) showed the typical feature of central pontine myelinolysis (CPM); however, there was no history of alcoholism, liver transplantation, malnutrition or rapid correction of hyponatremia. The patient had taken venlafaxine to treat major depressive disorder for more than 20 years. After discontinuation of venlafaxine, the unsteady gait gradually resolved, and subsequent MRI revealed reduction of the lesions over 6 months. We discuss herein the possible correlation between chronic use of venlafaxine and CPM.

BACKGROUND Central pontine myelinolysis (CPM) includes symmetric demyelination of the central pons. CPM is a rare neurological disorder that generally develops after rapid correction of hyponatremia in individuals having underlying conditions, such as malnutrition, alcoholism, and severe burns. It can cause severe long-term disabilities. However, there is currently no pharmacotherapy capable of promoting remyelination, a process crucial for recovery from CPM. We present the case of a patient with alcoholism and malnutrition-related CPM, which developed following rapid correction of hyponatremia but then improved remarkably with supportive physical therapy. CASE REPORT A 44-year-old alcoholic and malnourished man was admitted to an emergency hospital for disorientation due to overdrinking, but later developed bulbar palsy after hyponatremia was unexpectedly, but rapidly, corrected. Axial scans of the diffusion-weighted brain MRI revealed a characteristic lesion known as a piglet sign in the central pons. Based on his underlying conditions, present episode of sodium correction, and MRI finding, the patient was diagnosed as having CPM, which progressively worsened, resulting in locked-in syndrome after 12 days. The patient was then transferred to a long-term care unit and received simple motion exercise daily, but no specific medication. His symptoms gradually improved, achieving discontinuation of tube feeding on day 21, independent walking on day 110, and discharge after 6 months. CONCLUSIONS This report highlights the importance of physical therapy, the potential of which is often underestimated despite its broad benefits for human health, as a readily applicable intervention for patients with CPM. Further understanding of mechanisms underlying exercise-induced myelination should contribute to establishing novel therapies for a wide spectrum of brain disorders.

Background Variable neurological manifestations and imaging findings have been described in children with severe hypernatremia. We aimed to describe the spectrum of neuroimaging changes in infants with severe hypernatremia. Methods This retrospective study included infants with severe hypernatremia (serum sodium >160 mEq/L), abnormal neurological examination, and an abnormal magnetic resonance imaging (MRI) of the brain over a period of 2 years in a tertiary care hospital. Relevant clinical data, including the feeding practices, clinical features, complications, and biochemical and radiological parameters, were entered in a structured pro forma. MRI findings were classified as vascular (hemorrhages and cerebral sinus venous thrombosis), osmotic demyelination syndrome (pontine and extrapontine myelinolyses), and white matter changes. Results The common clinical features in the neonates were poor feeding (n = 4) and decreased urine output (n = 4); the older infants presented with gastrointestinal losses (n = 5). All cases had dehydration with encephalopathy. The patterns of radiological injury were vascular (hemorrhages, n = 5 and venous thrombosis, n = 3), osmotic demyelination (n = 8), and white matter changes (n = 7). Coagulopathy was correlated with the vascular complications (r = 0.8, p Conclusion Characteristic neuroimaging findings are vascular changes in the form of venous thrombosis and hemorrhages, osmotic demyelination and white matter tract injury, and/or mostly combinations of these findings. Severe hypernatremia and resulting hyperosmolarity frequently cause neurological sequelae in neonates and infants.

Central pontine myelinolysis (CPM) is a neurological disorder characterized by damage to the myelin and oligodendrocytes in the pons. This review focuses on the history of CPM and the discovery of its association with the treatment of hyponatremia. The author reviewed original publications regarding CPM, hyponatremia, and the treatment of hyponatremia. The author interviewed Dr. Robert Laureno who was a pioneer in CPM research with his animal work in dogs. Animal models demonstrated the role of the rapid correction of hyponatremia as causative of pontine and extrapontine myelinolytic lesions. Nevertheless, the importance of the speed of correction was widely denied. There were years of debates and only slow changes in expert guidelines. CPM occurs as a consequence of a rapid rise in serum sodium in individuals with chronic hyponatremia. It is recommended to increase plasma sodium concentration by no more than 8 to 10 mmol/L per 24 h in chronic hyponatremia.

Medulla oblongata myelinolysis is an extremely rare manifestation of extrapontine myelinolysis (EPM). Herein, we report a case of a 34-year-old man with a history of gout who presented repeated vomiting and diarrhea after ingesting 15 colchicine pills. A hyponatremia diagnosis was given and after an intensive treatment, his serum sodium level increased from 118 to 129 mmol/L within 24 hours. Brain magnetic resonance imaging (MRI) revealed a lesion in the medulla oblongata that appeared as a hypointense area in T1-weighted images and a hyperintense area in T2-weighted images. A diagnosis of medulla oblongata myelinolysis and colchicine poisoning was then given, and methylprednisolone therapy was initiated. Seventeen days later, the patient achieved a good outcome with methylprednisolone therapy. However, his medulla oblongata lesion remained detectable with MRI. Medulla oblongata myelinolysis is an extremely rare manifestation of EPM, and unique for being colchicine-induced. This case shows that colchicine poisoning can lead to hyponatremia, which in turn can induce myelinolysis if not treated correctly. As exemplified by our patient's case, desirable treatment outcomes are possible in such cases, although these outcomes may not be associated with a visible reduction of the brain lesions in MRI scans.

Delirium tremens (DT) is a serious condition occurring in alcohol withdrawal syndrome. Alcohol consumption may also cause additional health problems, such as respiratory infections or neuropsychiatric conditions such as central pontine myelinolysis. In this clinical scenario, managing DT can be expected to be more compelling and complex. Alcohol decreases coughing and mucociliary clearance and disrupts the immunity of the respiratory system.Here we report on a middle-aged man with alcohol use disorder who had developed DT due to alcohol withdrawal and comorbid pneumonia.In this paper, DT, the relation between respiratory infections and alcohol intake, and the correlation of alcohol consumption and central pontine myelinolysis (CPM) are discussed. Also, the literature on alcohol consumption and the additional respiratory and neurologic problems resulting from it are presented.Delirium tremens (DT) ist eine schwerwiegende Erkrankung, die beim Alkoholentzugssyndrom auftritt. Alkoholkonsum kann auch zusätzliche gesundheitliche Probleme wie Infektionen der Atemwege oder neuropsychiatrische Störungen wie die zentrale pontine Myelinolyse verursachen. In diesem klinischen Szenario wird erwartet, dass die Behandlung von DT komplexer und überzeugender ist. Alkohol verringert den Husten und die mukoziliäre Clearance und stört die Immunität des Atmungssystems. FALL: In der unten aufgeführten Forschungsanalyse wurde ein Mann mittleren Alters mit einer Alkoholkonsumstörung, der aufgrund von Alkoholentzug und einer komorbiden Lungenentzündung ein Delirium tremens entwickelte, von unserem Forschungsteam beobachtet und vorgestellt.Delirium tremens und der Zusammenhang zwischen Atemwegsinfektionen und Alkoholkonsum; und der Zusammenhang zwischen Alkoholkonsum und zentraler pontiner Myelinolyse (CPM) wurden diskutiert. Auch die Literatur zu Alkohol und seinen zusätzlichen respiratorischen und neurologischen Problemen wurde entsprechend der vorliegenden Daten entlastet.

Objective To report the incidence rate of osmotic demyelination syndrome (ODS), associated risk factors, treatment, and long-term outcomes in a nationwide cohort. Methods We conducted a retrospective study of individuals diagnosed with central pontine myelinolysis (ICD-10 code G37.2) in the Swedish National Patient Register during 1997-2011. Results During the study period, we identified 83 individuals with ODS, 47 women and 36 men. Median age at diagnosis was 55 years. The incidence rate of ODS for the entire study period was 0.611 (95% CI: 0.490-0.754) per million person-years and increased during the study period from 0.271 (95% CI: 0.147-0.460) in 1997-2001 to 0.945 (95% CI: 0.677-1.234) individuals per million person-years in 2007-2011. Most cases (86.7%) were hyponatremic with a median sodium level at admission of 104 mmol/L. All hyponatremic cases were chronic. The cause of hyponatremia was multifactorial, including drugs (56.9%), polydipsia (31.9%), and vomiting or diarrhea (41.7%). A majority of patients (69.9%) were alcoholics. Hyponatremic patients were predominantly treated with isotonic saline (93.1%) and only 4.2% with hypotonic fluids. The median correction rate was 0.72 mmol/L/h. Only six patients were corrected in accordance with national guidelines (≤8 mmol/L/24/h). At three months, 7.2% had died and 60.2% were functionally independent (modified Rankin Scale 0-2). Interpretation We found an increasing incidence during the study period, which could partly be explained by increased access to magnetic resonance imaging. ODS occurs predominantly in patients with extreme chronic hyponatremia which is corrected too fast with isotonic saline. Most patients survived and became functionally independent.

Osmotic demyelination syndrome is an acute demyelination process that usually occurs several days following an osmotic stress. This syndrome is rare in adults (0.4% to 0.56%) and even more uncommon in children. We performed a review of all reported pediatric osmotic demyelination syndrome patients from 1960 to 2018. Among all 106 cases, 49 presented with isolated central pontine myelinolysis, 30 with isolated extrapontine myelinolysis, and 27 with combined central pontine myelinolysis and extrapontine myelinolysis. There was no gender preponderance, and the highest prevalence was noted between the ages one and five years. Magnetic resonance imaging remains the diagnostic modality of choice, and diffusion tensor imaging is now increasingly used for prognostication in osmotic demyelination syndrome. Sixty percent of the children had a complete neurological recovery. Current management of osmotic demyelination syndrome in children consists of supportive medical care, steroids, and intravenous immunoglobulin. Our review of the literature supports the hypothesis that steroids and immunoglobulins are potentially helpful, although additional controlled studies are needed.

Rapid overcorrection of chronic hyponatremia can lead to osmotic demyelination syndrome or central pontine myelinolysis (CPM), a diagnosis often triggered by observing the characteristics of neurological abnormalities developed as a result of CPM. However, anyone with chronic hyponatremia and overcorrection of serum sodium is at risk of physiological CPM despite the lack of clinical symptoms. We report an adult patient who presented as post-op delirium, had incidental finding of CPM by magnetic resonance imaging (MRI) of the head after a liver transplant. Despite his non-typical presentation, the patient had the typical risk factors of CPM such as chronic hyponatremia, rapid overcorrection of serum sodium and cirrhosis undergoing a transplant. As hyponatremia and neurological disorder such encephalopathy simultaneously affect patients with cirrhosis, CPM may be more common than once thought in the chronic liver disease population and inappropriate hyponatremia management has important medical consequences that can go unnoticed.

The Paraguayan War ended 150 years ago. Back then, there were outbreaks of combatants’ limb weakness and tingling related to "palustrian cachexia", not clearly funded at the time on nutritional deficiency, the use of native flora to feed troops, and alcoholism. We report a case of a soldier with ascending paralysis, mental confusion and finally tetraplegia with preserved oculomotricity. This would probably be a case of locked-in syndrome (LIS) due to Gayet-Wernicke's encephalopathy consequent to thiamine deficiency. The role of thiamine in the peripheral or central nervous system expression was shown decades later to be related to poor diet, or use of foods containing thiaminase or thiamine antagonists, worsened by the fact that the bodily stores of thiamine are restricted, and deficits may grow fast. RESUMO A Guerra do Paraguai terminou há 150 anos. Houve surtos de fraqueza dos membros dos combatentes e parestesias relacionadas à “caquexia palustre”, não claramente fundamentados na época sobre deficiência nutricional, o uso da flora nativa para alimentar as tropas e o alcoolismo. Referimo-nos a um caso de um militar com paralisia ascendente, confusão mental e finalmente tetraplegia com oculomotricidade preservada. Esse relato seria provavelmente devido a um caso de “síndrome do encarceramento” secundário à encefalopatia de Gayet-Wernicke consequente à deficiência de tiamina. O papel da tiamina na expressão periférica ou do SNC foi previsto décadas mais tarde em relação a uma dieta pobre, ou ao uso de alimentos contendo tiamina ou antagonistas da tiamina, agravado pelo fato de que as reservas corporais de tiamina são restritas, e os déficits podem se manifestar rapidamente.

A 38-year-old woman with uncontrolled type 1 diabetes (HbA1c 12.8%) was admitted for diabetic ketoacidosis (731 mg/dL blood glucose). Hyperglycemia was corrected within 24 hours to 129 mg/dL. Upon presentation, her sodium and potassium levels were 139 and 3.9 mmol/L, respectively, remaining stable until discharge. There was no history of malnutrition or alcohol abuse.

Central pontine myelinolysis (CPM) is demyelinating condition of pons caused by osmotic stress due to rapid correction of hyponatremia. We present a case where CPM was an incidental finding on FDG PET/CT scan. To the best of our knowledge, only 3 case reports have been published till date describing CPM on 18F-FDG PET/CT scans.

Central pontine myelinolysis (CPM) is a neurological disorder characterized by demyelination on the bottom of pons. CPM is known to be the most common clinical presentation of osmotic demyelination syndrome. Osmotic stress formed by rapid correction of hyponatremia in glia cells is thought to be important in pathogenesis. Oligodendroglias are more sensitive to dehydration and volume changes as they are tightly aligned in the pontine. Chronic alcohol use is a rare cause of osmotic demyelination. In chronic alcoholics, central pontine myelinolysis may be asymptomatic or mild symptoms may develop. We presented the case to emphasize that chronic alcoholism is a rare cause of central pontine myelinosis. Keywords: myelinolysis, central pontine, alcoholism.

Rationale: Pituitary tumor apoplexy (PTA) is a rare clinical syndrome which requires urgent diagnosis and treatment due to its life-threatening consequences. Management of undiagnosed pituitary tumor before pregnancy is a problem during pregnancy. Patient concerns: We reported a case with PTA which was not diagnosed before pregnancy presenting with vomiting associated with hyponatremia during the third trimester. After supplying the sodium the patient presented with dysarthria and hemiplegia. Diagnoses: MRI examination showed PTA accompanied with extrapontine myelinolysis (EPM). Interventions: The patient was given hydrocortisone according to the symptoms gradually to taper off dose, at the same times oral levothyroxine therapy (25μg/day) was given. Outcomes: The patient delivered a healthy baby via cesarean section at hospital at 38 + 1 week of gestation. We performed MRI examination regularly and the tumor regressed significantly 8 months postpartum. Lessons: We reported a case as PTA associated with EPM. Headache during pregnancy is often nonspecific, so careful medical history inquiry is very important.

Central pontine myelinolysis and extrapontine myelinolysis are collectively called the osmotic demyelination syndromes. Despite being described in 1959, there are several aspects of the disorder that remain an enigma. Animal models and neuroimaging techniques have allowed us to understand the condition better. From being a universally fatal disorder that was diagnosed post mortem, increased awareness, neuroimaging techniques and supportive care have enabled us to make the diagnosis ante-mortem. This has also led to a significant drop in associated mortality. The aim of this review is to highlight the clinical spectrum, neuroimaging findings, and recent developments.

Objective. To investigate the etiology, clinical as well as neuroimaging characteristics, and outcomes after proper treatment in a series of 18 patients with osmotic demyelination syndrome. Methods. Medical records, including video records, of 18 patients with osmotic demyelination syndrome were retrospectively examined. Demographic and clinical information, imaging results, plans of management, and outcomes during the follow-up period were collected and analyzed. Results. Eighteen patients, including 10 males and 8 females, were included in the present study. The mean age at diagnosis of CNS insult was 47.4 ± 13.3 years (ranged from 30 to 78 years). Etiologies included rapidly corrected hyponatremia (50%), alcoholism (27.8%), and others. Neurological manifestations included encephalopathy (61.1%), dysphonia (50%), extrapyramidal symptoms (38.9%), and seizures (22.2%). Neuroimaging results showed that 6 patients (33.3%) had central pontine myelinolysis, 5 (27.8%) had extrapontine myelinolysis, and 7 (38.9%) had both. After treatment, 12 patients showed improvement and the other 6 did not. Among these patients, those who showed symptoms of encephalopathy had a favorable outcome. The majority of those who presented with mental retardation, seizures, and no other symptoms recovered better than their counterparts who had other symptoms. Nine out of 11 patients with pseudobulbar paralysis and/or extrapyramidal symptoms showed improvement, but the other 2 did not show improvement. Five patients who did not improve after treatment during admission were followed up for 1-3 months with rehabilitation training recommended, and it was found that 3 showed significant improvement after training, and the other 2 did not respond to this training. Conclusions. Osmotic demyelination syndrome is a complex disease entity due to a variety of etiologies, manifesting with symptoms involving diverse systems of the brain. Early identification and removal/correction of conditions leading to osmotic demyelination syndrome are the key to prevent and/or manage this disease.

Bartter syndrome (BS) is a rare autosomal recessive renal tubular disorder characterized by acute electrolyte imbalance, and similarly, osmotic demyelination syndrome (ODS) is a rather rare complication occurring during electrolyte imbalance. The pathological features of ODS include central pontine myelinolysis and extrapontine myelinolysis (EPM), which consist of severe damage to the myelin sheath of neurons. ODS is very rare in children. We describe a case of a 3-month-old infant with ODS and EPM associated with undiagnosed BS. ODS developed because of a sudden change in electrolyte levels and osmolality caused by acute dehydration during a gastrointestinal infection episode. Undiagnosed, untreated, and non-balanced BS was the cause of the neurological complication. Our patient represents the first case of ODS in BS, the ninth case of ODS in an infant less than one year old, and the third case of isolated EPM in such a young patient. This case report reminds us that in rare diseases, young patients tend to have genetic components.

Central pontine myelinolysis is a rare but severe disease that often occurs in alcohol-dependent and malnourished patients. One pathological mechanism is the rapid correction of chronic hyponatremia, even though the disease can occur independently of decreased serum sodium levels. Here, we present a patient suffering from malnutrition, alcohol dependency, and a severe depressive disorder, who presented himself to our clinic wishing for qualified withdrawal treatment. Because the patient reported significant weight loss and nocturnal sweating without fever, we performed different diagnostic investigations and examinations. Cranial MRI revealed the presence of a central pontine myelinolysis. In the clinical neurological examination, the patient only showed slight gait ataxia. The depressive symptoms had improved while the patient now showed problems in his short-term memory. At presentation, only slight hyponatremia was present, while no rapid correction occurred throughout treatment. The presented case reveals the importance of considering osmotic demyelination disorders as a differential diagnosis in patients suffering from neurological symptoms during alcohol withdrawal therapy. This is important independently of hyponatremia.Die zentrale pontine Myelinolyse (CPM) ist eine seltene, aber oft schwerwiegende Erkrankung, die gehäuft in Patienten mit einer Mangelernährung und einer Alkoholkonsumstörung auftritt. Ursächlich wird häufig der zu schnelle Ausgleich einer chronischen Hyponatriämie angesehen, wobei das Krankheitsbild auch unabhängig einer Hyponatriämie auftreten kann. In diesem Fallbericht präsentieren wir einen alkoholabhängigen Patienten mit einer starken Mangelernährung und klinisch schweren Depression, der zur qualifizierten Entgiftung stationär aufgenommen wurde. Aufgrund des Verdachts auf B‑Symptomatik bei Vorhandensein einer starken Gewichtsabnahme und Nachtschweiß ohne Fieber führten wir eine Reihe an Untersuchungen durch. Eine MRT-Untersuchung zeigte das Bild einer zentralen pontinen Myelinolyse, wobei der Patient neurologisch bis auf eine leichte Ataxie und Veränderungen im Affekt und Verhalten unauffällig war. Die initiale depressive Symptomatik besserte sich im Verlauf, der Patient zeigte jedoch neue Auffälligkeiten im Kurzzeitgedächtnis. Bei Aufnahme bestand eine leichte Hyponatriämie (130 mmol/l), die ohne starke Schwankungen ausgeglichen wurde (140 mmol/l bei Entlassung). Der präsentierte Fall zeigt, dass bei Vorhandensein von neurologischen Symptomen während der qualifizierten Entzugsbehandlung möglichst früh die Differentialdiagnose einer CPM in Betracht gezogen werden muss. Dies gilt auch unabhängig davon, ob eine Hyponatriämie vorliegt.

To analyze the clinical presentation and the treatment process of one case of colchicine poisoning complicated with extra pontine myelinolysis and discuss its pathogenesis. Increasing the attention of hyponatremia caused by colchicine poisoning is of great significance for improving the prognosis and quality of life of patients.分析1例秋水仙碱中毒并发脑桥外髓鞘溶解症患者的临床表现及诊治过程，探讨其发病机制。提高对秋水仙碱中毒引起低钠血症的重视，对于改善患者的预后及生活质量有着重要意义。.

Patient: Male, 38-year-old Final Diagnosis: Osmotic demyelination syndrome Symptoms: Seizure Medication: — Clinical Procedure: — Specialty: Nuclear Medicine • Radiology Objective: Challenging differential diagnosis Background: Osmotic demyelination syndrome (ODS) is an uncommon neurological disorder. Until the mid-1980s, the mortality rate was 90–100%, but more than half of patients now have a good prognosis. Early suspicion of ODS is important. However, radiologic findings of ODS are variable and scintigraphy findings have not been reported. Case Report: A 38-year-old man with alcohol abuse history was admitted due to electrolyte imbalance. On the 10th day of his hospital stay, he had a generalized tonic-clonic seizure. Brain perfusion SPECT showed asymmetrically hyper-perfused and hypoperfused lesions. Brain MRI revealed diffuse T2 hyperintensity with mild diffusion restriction in the pons and hyperperfused lesions on brain SPECT. He was treated based on the diagnosis of hyponatremia and osmotic demyelination. After treatment, the asymmetric hyperperfusion was decreased. MRI showed that the cortical hyperintensity had resolved, with encephalomalacic change shown in the pons. Conclusions: To the best of our knowledge, this is the first report showing changes in brain perfusion SPECT and MRI in an ODS patient with a seizure. This case report may be helpful to neurologists, radiologists, and nuclear physicians.

Osmotic demyelination syndrome (ODS) is a very rare condition in childhood occurring usually secondary to the rapid increase of serum sodium levels. This situation occurring secondary to the rapid correction of hyponatremia can be seen more rarely in the form of extrapontine myelinolysis and even the coexistence of these two conditions besides central pontine demyelinolysis. However, osmotic demyelination syndrome due to the rapid correction of hyponatremia in chronic renal failure (CRF) patients is very rare depending on existing uremia. In this article, we present an extremely rare case of pontine and extrapontine myelinolysis, which occurred in a pediatric patient with chronic renal failure, secondary to the rapid correction of hyponatremia. In the diffusion and cranial magnetic resonance imaging (MRI), bilateral symmetrical caudate, putamen, and thalamus involvements and hyperintense linear lesions at the pons, cortical, and subcortical areas were revealed. It was evaluated as pontine and extrapontine myelinolysis. This clinical situation presents that the presence of severe hyponatremia and extremely rapid correction of it can develop pontine and extrapontine myelinolysis even though it is very rare in uremic patients.

Background and Aim: Liver transplantation (LT) is the only curative treatment for patients with the end-stage liver disease. Amongst the complications post-LT, the neurological complications (NC) are particularly relevant. Our aim is to assess the incidence, risk factors and clinical presentation of NC in recipients after living donor liver transplantation. Methods: Between November 2011 and December 2013, 149 patients were admitted to ICU in 3 different centres in Egypt after LDLT and were evaluated by full clinical examination, laboratory investigations, neuroimaging and the NC were observed over one month. This study was approved by the ethical committee of the National Research Center. Results: 46 recipients (30.9%) developed neurological complications. The most common neurological complication was Encephalopathy (14.1%) while the least were both central pontine myelinolysis and meningoencephalitis (0.7%). In addition, 7 patients developed cerebrovascular events (either ischemic or hemorrhagic strokes). Patients were then classified into uncomplicated and complicated subgroups according to the highest percentage of neurological complication symptoms. These were encephalopathy, delirium with agitation, hallucinations, and delusions. Conclusion: A high incidence of neurological complications (30.9%) after LDLT was recorded, prolonging patient hospital stays. The most common complications were encephalopathy, delirium, hallucinations, delusions, and seizures some of which were drug related.

Acute neurovascular events, though rare, can complicate pregnancy and postpartum period. It is important to be aware of these clinical conditions for reducing maternal and fetal morbidity and mortality. In this review, we present a few important neurovascular emergencies and their imaging manifestations by various imaging modalities-computed tomography (CT), magnetic resonance imaging (MRI), and digital subtraction angiography (DSA)-which presented at our institution in the peripartum period.

Central pontine myelinolysis (CPM) develops due to acute hypernatremia from a normal baseline serum sodium in the setting of electrolyte abnormalities induced by topiramate use. Topiramate is a commonly used medication with several indications including migraines, myoclonic jerks and seizures. It has been reported to cause renal tubular acidosis and severe electrolyte abnormalities, which in turn predispose patients to neuropathology via renal concentration defects and osmotic shifts. Our patient is a 55-year-old woman with a history of multiple sclerosis and myoclonus on topiramate for several years who presented with weakness and was found to be profoundly hypokalemic. She went on to develop changes in mental status, motor deficits and evidence of CPM on MRI during her hospitalisation. Surprisingly, the patient never had hyponatremia; however, she had an acute rise in serum sodium from a normal baseline after fluid resuscitation with normal saline for hypotension during her admission.

Central pontine myelinolysis (CPM) is commonly associated with osmotic stress and rapid correction of hyponatraemia. It has rarely been reported in conjunction with malignancies. We report a case where CPM was not only associated with a new diagnosis of diffuse large B-cell lymphoma but was also a key presenting feature.

A 27-year-old man with a history of diabetes mellitus (DM) and asthma presented to the emergency department 1 month after the onset of dysarthria and ataxia. The symptoms were noted abruptly upon waking. He swayed on standing, fell easily, and noted tremor when manipulating objects. His speech was nearly unintelligible. He also had 1 month of mild distal paresthesias and a 30-pound unintentional weight loss. He denied diplopia, dysphagia, preceding illness, or other systemic symptoms. Over the course of the month, his dysarthria improved but his imbalance remained unchanged. His delay in seeking medical attention was due to lack of health insurance. The authors thank Michel J. Berg, MD, Department of Neurology, University of Rochester Medical Center, and Melissa Tsuboyama, MD, Department of Child Neurology, University of Rochester Medical Center, for reviewing the manuscript.

Hyponatremia and its rapid correction is a well-known cause of osmotic demyelination most commonly affecting the pons. We report on a case of severe hyponatremia likely due to psychogenic polydipsia resulting in hypotonic hyperhydration with resulting cortical laminar necrosis on initial imaging, mimicking hypoxic brain damage. Pontine myelinolysis became apparent on follow-up imaging, illustrating the diagnostic challenges of extrapontine manifestations of severe hyponatremia.

Central pontine myelinolysis (CPM) is a potentially-devastating complication of rapid osmolar shifts, classically attributed to overlyaggressive correction of chronic hyponatremia. Magnetic resonance imaging (MRI) allowed earlier diagnosis of CPM, but most importantly, it has revealed that the odds of good functional recovery are surprisingly high. A trident shaped pontine lesion is a typical finding in CPM (the trident sign). The "piglet sign" is a much less well-known radiologic finding in CPM. Due to the rarity of CPM, very little has been published on the evolution of these MRI findings. We present a case of CPM in an alcoholic young man, and describe the temporal evolution of both the trident and piglet signs on MRI in CPM.

An 82-year-old man presented with altered mental status, withdrawal to pain, osmolality 405 mOsm/kg, and sodium >180 mEq/L, corrected at 8 mEq/L/d. Brain MRI on day 4 showed restricted diffusion within bilateral middle cerebellar peduncles and along the cortical spinal tracts (figure). Extrapontine osmotic demyelination was diagnosed. Classically described after rapid correction of hyponatremia, few cases have been reported in patients with severe hypernatremia. Extrapontine osmotic demyelination occurs after rapid increase in extracellular sodium causing cellular dehydration and cell death of astrocytes or oligodendrocytes provoking non-inflammatory demyelinating lesions in pontine or extrapontine regions. Isolated extrapontine lesions are seen in two-fifths of patients.1,2

Demyelinated lesions in human pons observed after osmotic shifts in serum have been referred to as central pontine myelinolysis (CPM). Astrocytic damage, which is prominent in neuroinflammatory diseases like neuromyelitis optica (NMO) and multiple sclerosis (MS), is considered the primary event during formation of CPM lesions. Although more data on the effects of astrocyte-derived factors on oligodendrocyte precursor cells (OPCs) and remyelination are emerging, still little is known about remyelination of lesions with primary astrocytic loss. In autopsy tissue from patients with CPM as well as in an experimental model, we were able to characterize OPC activation and differentiation. Injections of the thymidine-analogue BrdU traced the maturation of OPCs activated in early astrocyte-depleted lesions. We observed rapid activation of the parenchymal NG2+ OPC reservoir in experimental astrocyte-depleted demyelinated lesions, leading to extensive OPC proliferation. One week after lesion initiation, most parenchyma-derived OPCs expressed breast carcinoma amplified sequence-1 (BCAS1), indicating the transition into a pre-myelinating state. Cells derived from this early parenchymal response often presented a dysfunctional morphology with condensed cytoplasm and few extending processes, and were only sparsely detected among myelin-producing or mature oligodendrocytes. Correspondingly, early stages of human CPM lesions also showed reduced astrocyte numbers and non-myelinating BCAS1+ oligodendrocytes with dysfunctional morphology. In the rat model, neural stem cells (NSCs) located in the subventricular zone (SVZ) were activated while the lesion was already partially repopulated with OPCs, giving rise to nestin+ progenitors that generated oligodendroglial lineage cells in the lesion, which was successively repopulated with astrocytes and remyelinated. These nestin+ stem cell-derived progenitors were absent in human CPM cases, which may have contributed to the inefficient lesion repair. The present study points to the importance of astrocyte-oligodendrocyte interactions for remyelination, highlighting the necessity to further determine the impact of astrocyte dysfunction on remyelination inefficiency in demyelinating disorders including MS. Electronic supplementary material The online version of this article (10.1186/s40478-020-01105-2) contains supplementary material, which is available to authorized users

Background Osmotic demyelination syndrome (ODS), which embraces central pontine myelinolysis (CPM) and extrapontine myelinosis (EPM), is often underdiagnosed in clinical practice, but can be fatal. In this article, we review the etiology, patho- physiology, clinical features, diagnosis, treatment, and prognosis of ODS. Methods Pertinent publications from the years 1959 to 2018 were retrieved by a selective search in PubMed. Results The most common cause of ODS is hyponatremia; particular groups of patients, e.g., liver transplant recipients, are also at risk of developing ODS. The pathophysiology of ODS consists of cerebral apoptosis and loss of myelin due to osmotic stress. Accordingly, brain areas that are rich in oligodendrocytes and myelin tend to be the most frequently affected. Patients with ODS often have a biphasic course, the first phase reflecting the underlying predisposing illness and the second phase reflecting ODS itself, with pontine dysfunction, impaired vigilance, and movement disorders, among other neurological abnormalities. The diagnostic modality of choice is magnetic resonance imaging (MRI) of the brain, which can also be used to detect oligosymptomatic ODS. The current mainstay of management is prevention; treatment strategies for manifest ODS are still experimental. The prognosis has improved as a result of MRI-based diagnosis, but ODS can still be fatal (33% to 55% of patients either die or remain permanently dependent on nursing care). Conclusion ODS is a secondary neurological illness resulting from a foregoing primary disease. Though rare overall, it occurs with greater frequency in certain groups of patients. Clinicians of all specialties should therefore be familiar with the risk constellations, clinical presentation, and prevention of ODS. The treatment of ODS is still experimental at present, as no evidence-based treatment is yet available.