Therapeutic plasma exchange as a treatment for Central Pontine Myelinolysis in a 41-year-Old male with Chronic Renal Insufficiency: A case report

@#Therapeutic plasma exchange (TPE) has been reported as a possible treatment for osmotic demyelination syndrome – central pontine myelinolysis (ODS-CPM), a degeneration of myelin within the central nervous system related to rapid hyponatremia correction, which though uncommon, has significant morbidity, and has no established specific treatment. We present our experience with a 41-year-old male with chronic kidney disease, maintained on steroids, who presented with lethargy and behavioral changes. Initial metabolic panel showed severe hyponatremia (Na 109 mEq/L). Despite cautious sodium correction, the patient’s sensorium decreased further and was intubated. Involuntary movements of the left face and arm were later seen. T2/FLAIR hyperintensities in the brainstem and thalami affirmed the diagnosis of ODS. A total of nine cycles (one cycle every two to three days) of TPE were completed. The patient was discharged with improved sensorium, from E2VxM4 to E4VxM6, and with no indication for hemodialysis due to improved creatinine. One year later, the patient has no remaining neurologic deficits. Our experience supports other case reports that TPE is a viable therapy for ODS-CPM.

Síndrome de desmielinización osmótica, a propósito de un caso / Osmotic demyelination syndrome, a case report

El síndrome de desmielinización osmótica, antes conocido como mielinólisis central pontina, es una enfermedad que ocurre en pacientes con hiponatremia grave en los cuales se realiza una corrección rápida del sodio. Clínicamente, se presenta como una parálisis seudobulbar que consiste en tetraparesia, encefalopatía, rigidez, ataxia y movimientos anormales. El síndrome de desmielinización osmótica es una enfermedad desmielinizante no inflamatoria, secundaria a edema neuronal intenso que se produce en la protuberancia y otras regiones fuera de la protuberancia. Es una patología muy poco frecuente. Sin embargo, tiene muy mal pronóstico, y la rehabilitación es el único tratamiento eficaz. Se presenta el caso de un paciente de 51 años de edad con cuadro de déficit neurológico altamente progresivo después de la corrección rápida de una hiponatremia, con tetraparesia, encefalopatía y rigidez en los días siguientes. El paciente requirió intubación orotraqueal debido al deterioro clínico. Fue diagnosticado mediante una tomografía axial computarizada (TAC) cerebral y se confirmó el diagnóstico mediante resonancia magnética (RM).

Osmotic demyelination syndrome, previously known as central pontine myelinolysis, is a known disorder in patients with severe hyponatremia in whom rapid sodium correction is performed. It is clinically described as a pseudobulbar palsy, comprised of tetraparesis, encephalopathy, rigidity, ataxia and abnormal movements. It consists of a non-inflammatory demyelination secondary to severe neuronal edema at the pons and other extrapontine locations. It is a very rare pathology, with a poor prognosis and whose only treatment is rehabilitation. A case of a 51-year-old man with fast progressive neurological deficit following rapid correction of severe hyponatremia is presented. The patient required orotracheal intubation due to clinical deteroriation and was diagnosed by computed tomography (CT) and confirmed by magnetic resonance imaging (MRI)

Status Epilepticus in a Patient with Extrapontine Myelinolysis

Extrapontine myelinolysis is a demyelinating disorder usually caused by rapid correction of chronic hyponatremia. We present an 82-year-old female patient with general weakness, and severe hyponatremia (103 mEq/L) which was corrected 14 mEq/L during 21 hours. She was stuporous and presented status epilepticus. Brain T2-weighted image showed high signal intensities of bilateral thalami and electroencephalogram monitoring indicated right hemisphere lateralized periodic discharges, maximal in the right frontal region. This case shows that status epilepticus can occur in a patient of extrapontine myelinolysis involved bilateral thalami without pontine lesions.

Desmielinización osmótica pontina y extrapontina durante un puerperio complicado / Osmotic demyelination pontine and extrapontine during complicated puerperium

La mielinolisis pontina central es una rara alteración neurológica de etiología desconocida. La causa más frecuente es el tratamiento de una hiponatremia cuando los niveles de sodio se elevan demasiado rápido. La lesión básica consiste en la destrucción de las vainas de mielina, que afecta parte o toda la base de la protuberancia en forma simétrica (desmielinización pontina). Los pacientes con esta enfermedad suelen presentar tetraparesia progresiva subaguda acompañada de parálisis seudobulbar con disartria e imposibilidad de protruir la lengua. Se presenta un estudio con el objetivo de describir el comportamiento de un caso de desmielinización osmótica en una paciente como parte de un puerperio complicado. Se trata de una paciente femenina, mestiza, de 33 años de edad, con embarazo complicado, secundario a cesárea por óbito fetal, tras la cual lleva ingreso en terapia intensiva. Luego de varias alteraciones hidroelectrolíticas se detecta cuadriplejia fláccida, con síndrome de enclaustramiento, con diagnóstico por neuroimagen de una desmielinización osmótica, con evolución favorable(AU)

Central pontine myelinolysis is a rare neurological disorder of unknown etiology. The most frequent cause is the treatment of hyponatremia when sodium levels rise too fast. The destruction of the myelin sheaths is the basic lesion, which affects part or the entire base of the protuberance symmetrically (pontine demyelination). Patients with this disease usually present subacute progressive tetraparesis accompanied by pseudobulbar paralysis with dysarthria and the inability to protrude their tongue. A study is presented with the objective of describing the behavior of a case of osmotic demyelination in a patient as part of complicated puerperium. This is a 33-year-old female patient, with complicated pregnancy, secondary to cesarean section due to fetal death, after which she is admitted to intensive care. After several hydroelectrolyte alterations, flaccid quadriplegia was detected, with Locked-in Syndrome, with neuroimaging diagnosis of osmotic demyelination, with a favorable evolution(AU)

Neonatal Seizures with Diffuse Cerebral White Matter Lesions on Magnetic Resonance Imaging Associated with Rotavirus Infection: A Report of Three Cases

Rotavirus is the major cause of gastroenteritis in children under the age of 5. Rotavirus infection may lead to several neurological complications as meningitis, encephalitis, convulsion, encephalopathy, hemorrhagic shock, central pontine myelinolysis, Guillain-Barre syndrome, and Reye's syndrome. Further, some reports have described diffuse cerebral white matter lesions on diffusion-weighted magnetic resonance imaging (MRI) in neonates with rotavirus induced seizures. Here, we report on three neonates with rotavirus induced seizures with cerebral white matter abnormalities on MRI.

Mielinólise Extrapontina em Adolescente com Diabetes Insípidus Secundário a Disgerminoma do Sistema Nervoso Central: Relato de Caso / Extra-Pontine Myelinolysis in a Patient with Diabetes Insipidus Secondary to Disgerminoma of the Central Nervous System: Case Report / mielinólise Extrapontina en Paciente con diabetes Insípida Secundário a disgerminoma del Sistema Nervioso Central: Relato de Caso

Introdução: a síndrome de desmielinização osmótica é uma condição neurológica rara causada pelo dano à bainha de mielina dos neurônios, com difícil manejo do distúrbio do sódio em paciente com diabetes insípidus. Relato do caso: adolescente do sexo feminino, 14 anos, com diabetes insípidus secundária a disgerminoma do sistema nervoso central, com hiponatremia grave (sódio 103 meq/l). cinco dias após a correção rápida do sódio, apresentou coma (escala de Glasgow:11), disfagia, mutismo e tetraparesia. os achados na ressonância nuclear magnética craniana foram compatíveis com diagnóstico de mielinólise extrapontina. Vinte e cinco dias após a internação no centro de tratamento intensivo, a paciente encontrava-se lúcida, orientada, deambulando sem dificuldade, alimentando-se por via oral, sem engasgos, ainda com discreta diminuição de força nos membros superiores e comunicação verbal pouco lentificada. a ressonância nuclear magnética após três meses mostrou atrofia dos núcleos da base, comprovando lesão celular grave. Conclusão: a desmielinização osmótica pode apresentar-se com formas clínicas leves ou assintomáticas, até sequelas motoras graves e morte. não existe tratamento específico, o que ressalta a importância do diagnóstico precoce e do manejo adequado do distúrbio do sódio, assim como controle rigoroso dos seus níveis séricos.

Introduction: osmotic demyelination syndrome is a rare neurological condition caused by damage to the myelin sheath of neurons, involving difficulty in the management of sodium imbalance in patients with diabetes insipidus. Case report: Patient was a 14-year-old female with diabetes insipidus secondary to dysgerminoma of the central nervous system, with severe hyponatremia (sodium 103 meq/l). Five days after rapid correction of the sodium imbalance, the patient presented coma (Glasgow scale:11), dysphagia, mutism, and quadriparesis. cranial Mri findings were consistent with a diagnosis of extrapontine myelinolysis. twenty-five days after admission to the icu, the patient was alert, oriented, walking without difficulty, eating an oral diet without choking, although with slightly diminished strength in the upper limbs and slightly sluggish verbal communication. Three-month follow-up Mri showed atrophy of the basal nuclei, confirming severe cellular injury. Conclusion: Presentation of osmotic demyelination may range from mild or asymptomatic clinical forms to severe motor sequelae and death. There is no specific treatment, which highlights the importance of early diagnosis and adequate management of the sodium imbalance, as well as rigorous control of serum sodium levels

Introducción: la síndrome de desmielinización osmótica es una condición neurológica rara causada por el daño en lãs vainas de mielina de las neuronas, con difícil manejo del trastorno de los niveles de sódio en los pacientes con diabetes insípida. Relato del caso: adolescente do sexo feminino, 14 anos, com diabetes insípida secundária a disgerminoma del sistema nervioso central, con hiponatriemia grave (sódio103meq/l). cinco días después de la corrección rápida del sódio, presentó coma (escala de Glasgow: 11), disfagia, mutismo y tetraparesia. los hallazgos en la resonancia magnética del cráneo fueron compatibles con diagnosis de mielinólise extra-pontina. Veinticinco días después de la internaciónen el centro de tratamiento intensivo la paciente se encontraba lúcida, orientada, deambulando sin dificultad, alimentándose por vía oral sin atorarse, aúncon discreta disminución de fuerza en los miembros superiores y comunicación verbal poco lentificada. una resonancia nuclear magnética después de tres meses mostró atrofia de los núcleos de la base del cráneo, comprobando lesión celular grave. Conclusión: una síndrome de desmielinización osmótica puede presentarse con formas clínicas ligeras o asintomáticas, hasta secuelas motoras graves y muerte. no existe tratamiento específico, lo que resaltala importancia del diagnóstico precoz y del manejo adecuado del disturbio del sodio, así como control riguroso de sus niveles séricos

Central pontine myelinolysis after liver transplantation: case report / Mielinolisis Póntica Central post-trasplante hepático: Reporte de Caso

SUMMARY Central Pontine Myelinolysis (CPM) is a demyelinating disease with central pontine localization which presents in patients with chronic hepatic disease and hydroelectrolytic disturbances. In spite of pathophysiology still unknown, the patient liver transplanted has several risk factors to CPM. Its clinical manifestations are mainly motor and awareness alterations. Diagnosis requires brain images like magnetic resonance and it has a poor prognosis in critical care patients and it is very important to know about the possibility of presentation and to be aware about this pathology. The main objective to present this case report is sensitizing the medical community regarding the CPM in patients with liver transplant. The case report of a 40-year-old female patient who had previous history of biliary cirrhosis secondary to autoimmune hepatitis and liver transplantation was performed; subsequently developed alteration of awareness, hydroelectrolytic disturbances and abnormal findings in brain imaging. CPM is a neurological demyelinating disease with multifactorial origin, its treatment is supportive and prevention is the main goal in patients with risk factors like patient after liver transplantation.

RESUMEN La Mielinólisis Pontica Central es una enfermedad desmielinizante con localización pontina central que se presenta especialmente en pacientes con enfermedad hepática crónica y alteraciones hidroelectrolíticas. A pesar de la fisiopatología aún desconocida, el paciente con trasplante hepático tiene varios factores de riesgo para su desarrollo. Sus manifestaciones clínicas son principalmente alteraciones motoras y de conciencia. El diagnóstico requiere imágenes cerebrales como la resonancia magnética y se asocia a mal pronóstico. En los pacientes de cuidados críticos y es muy importante conocer la posibilidad de presentación y tener conciencia de esta patología. El objetivo principal de este reporte de caso es sensibilizar a la comunidad médica respecto en pacientes posterior a trasplante hepático. Se realizó el reporte de una paciente de 40 años de edad con antecedente de cirrosis biliar secundaria a hepatitis autoinmune y trasplante de hígado; posteriormente desarrolló alteración de la conciencia, alteraciones hidroelectrolíticas y hallazgos anormales en la imagen cerebral. La Mielinolísis Póntica es una enfermedad desmielinizante neurológica de origen multifactorial, su tratamiento es de apoyo y la prevención es el objetivo principal en pacientes con factores de riesgo como el paciente tras trasplante hepático.

Alteraciones del sodio como causa de muerte en Patología Forense / Alterations of sodium as a cause of death in forensic pathology

ResumenLas alteraciones del sodio son un factor primordial o agravante que podrían condicionar o causar la muerte tanto en personas conocidas sanas como que en aquellas que presentan algún tipo de patología ya sea aguda o crónica. Dichas alteraciones en muchas ocasiones podrían pasar desapercibidas, dado su grandísima complejidad de detección, de tratamiento y en ocasiones de manejo clínico. Siendo estos factores que vuelven a dichas alteraciones un eje de la medicina clínica olvidada en muchas ocasiones por parte de los peritos encargados de realizar autopsias tanto clínicas como aquellas en las que priva algún interés de índole médico legal.

AbstractSodium alterations are a primary or aggravating factor that could cause death in both well-known and wellknown people, as well as those that present some type of pathology, either acute or chronic. These alterations in many occasions could go unnoticed, given their great complexity of detection, treatment and sometimes clinical management. These factors make these alterations an axis of clinical medicine often forgotten by experts in charge of performing clinical autopsies as well as those in which it deprives some interest of a legal medical nature

Central and Extrapontine Myelinolysis after Alcohol Withdrawal and Correction of Hypernatremia in a Chronic Alcoholic: a Case Report

Osmotic demyelination syndrome (ODS) is a demyelinating disorder related to the rapid correction of hyponatremia. It usually affects the pontine area; hence, the name central pontine myelinolysis (CPM). However, it rarely occurs with the correction of hypernatremia and hyperosmolarity and involves extrapontine areas. A 56-year-old chronic alcoholic had been admitted with a history of confusion. He had been in alcohol withdrawal for 4 days. Laboratory examinations showed severe hypernatremia and hyperosmolarity. After serum sodium level was normalized; however, his clinical course did not improve and deteriorated to semicoma progressively. Magnetic resonance imaging (MRI) revealed abnormal signal intensity in the pontine and extrapontine areas, including the basal ganglia, thalamus, and cerebral cortices. This is the first case report of combined central pontine and extrapontine demyelination after alcohol withdrawal and correction of hypernatremia in a chronic alcoholic.

Central Pontine Myelinolysis Induced by Alcohol Withdrawal: A Case Report

Central pontine myelinolysis (CPM) is a demyelinating disorder characterized by the loss of myelin in the center of the basis pons, and is mainly caused by the rapid correction of hyponatremia. We report the case of a young woman who presented with gait disturbance and alcohol withdrawal, and who was eventually diagnosed with CPM. Generally, the cause and pathogenesis of CPM in chronic alcoholics remain unclear. In this cases, the CPM may be unrelated to hyponatremia or its correction. However, it is possible that the osmotic pressure changes due to refeeding syndrome after alcohol withdrawal was the likely cause in this case. This case illustrates the need for avoiding hasty, and possibly incomplete diagnoses, and performing more intensive test procedures to ensure a correct diagnosis.

Ataxic Form of Central Pontine Myelinolysis Developed during Alcohol Withdrawal in a Chronic Alcoholic

Central pontine myelinolysis (CPM) is well-recognized osmotic demyelination syndrome that is related to various conditions such as rapid correction of hyponatremia and chronic alcoholism. Acute ataxia as a sole clinical sign in CPM is rare. We report a case of a 59-year-old man with dysarthria, intention tremor, and a significant gait ataxia starting after alcohol withdrawal, with radiological evidence of CPM. CPM should be included in the differential diagnosis of alcoholic patients who develop a sudden ataxia. Chronic alcohol abuse is one of the most commonly encountered predisposing factors. Alcohol withdrawal represents an additional vulnerability factor, being responsible for electrolyte imbalances which are not always demonstrable but are certainly involved in the development of CPM.

Preoperative Extrapontine Myelinolysis with Good Outcome in a Patient with Pituitary Adenoma

Few preoperative extrapontine myelinolysis (EPM) cases with pituitary adenoma have been reported. No such case had long follow-up to see the outcome of EPM. We reported a 38-year-old man complaining of nausea, malaise and transient loss of consciousness who was found to have severe hyponatremia. Neurologic deficits including altered mental status, behavioral disturbances, dysarthria and dysphagia developed despite slow correction of hyponatremia. Endocrine and imaging studies revealed hypopituitarism, nonfunctional pituitary macroadenoma and extrapontine myelinolysis. Transsphenoidal surgery was performed after three weeks of supportive therapy, when neurological symptoms improved significantly. The patient recovered function completely 3 months after surgery. Our case indicates that outcome of EPM can be good even with prolonged periods of severe neurologic impairment.

Freezing of Gait in Extrapontine Myelinolysis

A 65-year-old female visited us due to gait disturbance. A neurological examination showed cognitive impairment, dystonia, myoclonus, bradykinesia, postural instability, and freezing of gait (FOG). She was diagnosed with extrapontine myelinolysis based on her history of hyponatremia and high signal intensities (HSIs) in both striata on T2-weighted images. Her neurological problems including FOG improved over 25 days. In a follow-up MRI 50 days after the onset, HSIs disappeared in the striata but new ones appeared in the pons. FOG may have been related to striatal dysfunction in this patient.

Reversible Cerebellar Ataxia Related to Extrapontine Myelinolysis without Hyponatremia after Cisplatin-Based Chemotherapy for Cholangiocarcinoma / Journal of the Korean Cancer Association, 대한암학회지

A 60-year-old woman presented with cerebellar signs including dysarthria and ataxia, after intravenous infusion of cisplatin-based chemotherapy. Several blood tests showed mild neutropenia, normocytic normochromic anemia, but no evidence of a marked hyponatremia. Brain magnetic resonance imaging with diffusion-weighted sequences showed hyper-intense signal abnormalities in the extrapontine region, sparing the basis pontis. Here, we report on the case of a patient with reversible cerebellar ataxia related to extrapontine myelinolysis without hyponatremia after treatment with cisplatin-based chemotherapy for cholangiocarcinoma and discuss the literature on cerebellar ataxia in patients who underwent recent chemotherapy for malignancy.

A Case of Osmotic Demyelination Presenting with Severe Hypernatremia

Osmotic demyelination syndrome is a demyelinating disorder associated with rapid correction of hyponatremia. But, it rarely occurs in acute hypernatremia, and it leads to permanent neurologic symptoms and is associated with high mortality. A 44-year-old woman treated with alternative medicine was admitted with a history of drowsy mental status. Severe hypernatremia (197mEq/L) with hyperosmolality (415mOsm/kgH2O) was evident initially and magnetic resonance imaging revealed a high signal intensity lesion in the pons, consistent with central pontine myelinolysis. She was treated with 0.45% saline and 5% dextrose water and intravenous corticosteroids. Serum sodium normalized and her clinical course gradually improved. Brain lesion of myelinolysis also improved in a follow-up imaging study. This is the first report of a successful treatment of hypernatremia caused by iatrogenic salt intake, and it confirms the importance of adequate fluid supplementation in severe hypernatremia.

Central pontine myelinolysis: a rare presentation secondary to hyperglycaemia

Central pontine myelinolysis (CPM) is classically described as a demyelinating condition that results from the rapid correction of hyponatraemia. CPM has also been reported to arise from hyperglycaemia in association with concomitant acidosis, hypernatraemia and hyperosmolar syndrome. Herein, we report a rare presentation of CPM, which was purely secondary to hyperosmolar hyperglycaemia. The patient presented with ataxia and pseudobulbar affect, which evolved subacutely over a duration of two weeks. It is important to note that, in addition to acute changes in osmolality, a subacute shift secondary to hyperglycaemia may also lead to CPM.

Mielinólise pontina em paciente etilista / Pontine myelinolysis in an alcoholic patient

A Mielinólise Pontina (MP) define-se como uma lesão desmielinizante, associada a quadro de tetraparesia e incapacidade na fala, frequentemente relacionada a distúrbios eletrolíticos e observada em pacientes etilistas. Relatamos o caso de um paciente com diagnóstico de MP firmado por ressonância magnética (AU)

Pontine myelinolysis (MP) is defined as a demyelinating lesion associated with tetraparesis and disability in speech, often related to electrolyte disturbances and observed in alcoholic patients. Here we report the case of a patient diagnosed with MP through MRI (AU)

Mielinolisis Central Pontina: a proposito de un caso / Central Pontine Myelinolysis: a case report

La Mielinolisis central pontina es una lesión desmielinizante en la base de la protuberancia que en la mayoría de los casos se produce por una rápida correción de la hiponatremia. Comunicamos el caso de un paciente varón de 29 años con cuadriparesia, ataxia disartria y parálisis bilateral del sexto nervio craneal de presentación siete días después de la correción hidroelectrlítica por un estado de deshidratación secundario a un cuadro emético. Los estudios de resonancia magnética mostraron un patrón compatible con la enfermedad y su evolución en el tiempo. Este caso resalta la importancia de plantear el diagnostico de mielinolisis central pontina en pacientes con comprmiso corticoespinal, corticobulbar y otras vías nerviosas, en quienes exista el antecedente de corrección hidroelectrolítica.

Central pontine myelinolysis is a demyelinating lesion in the basis pontis which in most cases is caused by a rapid correction of hyponatremia. we report the case of a 29 years old male patient presented with quadriparesis, ataxia, dysarthria and bilateral sixth nerve palsy appeared seven days after correction of fluid and electrolyte imbalance by a state of dehydration secondary to an emetic event. MRI studies showed a pattern consistent with the disease and its evolution over time. This case highlights the importance of addressing the diagnosis of central pontine myelinolysis in patients with commitment corticospinal, corticobulbar and other neural pathways, where there is a history of fluid and electroyte management.

Recovery from a Complicated Case of Central Pontine and Extrapontine Myelinolysis by Dopaminergic Treatment: One-Year Follow-up: A Case Report

Central pontine and extrapontine myelinolysis are well-recognized osmotic demyelination syndromes related to the rapid correction of hyponatremia, chronic alcoholism, and malnutrition. They are reported to show brain stem signs and various movement disorders. A 58-year-old man with a history of chronic alcoholism was admitted for dysarthria, dysphagia, and gait disturbance that had developed five days after a right forearm cellulitis. Magnetic resonance imaging revealed demyelinating patterns in the central portion of the pons and both thalami. He showed severe extrapyramidal symptoms with truncal swaying and postural instability that resulted in severe gait disturbance. Postural instability showed little improvement after conventional physical therapy, but his symptoms markedly improved after five days of dopamine administration. Cessation of dopamine agents was attempted two times, but postural instability and gait disturbance recurred. Therefore, medication was continued for one year. The patient showed stable gait and no further deterioration of postural instability during dopamine therapy.