Osmotic demyelination as a complication of hyponatremia correction: a systematic review / Desmielinização osmótica como complicação da correção de hiponatremia: uma revisão sistemática

ABSTRACT Background: Rapid correction of hyponatremia, especially when severe and chronic, can result in osmotic demyelination. The latest guideline for diagnosis and treatment of hyponatremia (2014) recommends a correction limit of 10 mEq/L/day. Our aim was to summarize published cases of osmotic demyelination to assess the adequacy of this recommendation. Method: Systematic review of case reports of osmotic demyelination. We included cases confirmed by imaging or pathology exam, in people over 18 years of age, published between 1997 and 2019, in English or Portuguese. Results: We evaluated 96 cases of osmotic demyelination, 58.3% female, with a mean age of 48.2 ± 12.9 years. Median admission serum sodium was 105 mEq/L and > 90% of patients had severe hyponatremia (<120 mEq/L). Reports of gastrointestinal tract disorders (38.5%), alcoholism (31.3%) and use of diuretics (27%) were common. Correction of hyponatremia was performed mainly with isotonic (46.9%) or hypertonic (33.7%) saline solution. Correction of associated hypokalemia occurred in 18.8%. In 66.6% of cases there was correction of natremia above 10 mEq/L on the first day of hospitalization; the rate was not reported in 22.9% and in only 10.4% was it less than 10 mEq/L/day. Conclusion: The development of osmotic demyelination was predominant in women under 50 years of age, with severe hyponatremia and rapid correction. In 10.4% of cases, there was demyelination even with correction <10 mEq/L/day. These data reinforce the need for conservative targets for high-risk patients, such as 4-6 mEq/L/day, not exceeding the limit of 8 mEq/L/day.

RESUMO Antecedentes: A correção rápida da hiponatremia, principalmente quando grave e crônica, pode resultar em desmielinização osmótica. A última diretriz para diagnóstico e tratamento da hiponatremia (2014) recomenda um limite de correção de 10 mEq/L/dia. Nosso objetivo foi sumarizar os casos publicados de desmielinização osmótica para avaliar a adequação dessa recomendação. Método: Revisão sistemática de relatos de caso de desmielinização osmótica. Incluímos casos confirmados por imagem ou anatomia patológica, em maiores de 18 anos, publicados entre 1997 e 2019, nas línguas inglesa ou portuguesa. Resultados: Avaliamos 96 casos de desmielinização osmótica, sendo 58,3% do sexo feminino e com média de idade de 48,2 ± 12,9 anos. A mediana de sódio sérico admissional foi 105 mEq/L e > 90% dos pacientes apresentavam hiponatremia grave (<120 mEq/L). Foram comuns os relatos de distúrbios do trato gastrointestinal (38,5%), etilismo (31,3%) e uso de diuréticos (27%). A correção da hiponatremia foi feita principalmente com solução salina isotônica (46,9%) ou hipertônica (33,7%). Correção de hipocalemia associada ocorreu em 18,8%. Em 66,6% dos casos houve correção da natremia acima de 10 mEq/L no primeiro dia de internamento; a velocidade não foi relatada em 22,9% e em apenas 10,4% foi menor que 10 mEq/L/dia. Conclusão: O desenvolvimento da desmielinização osmótica foi predominante em mulheres, abaixo de 50 anos, com hiponatremia grave e correção rápida. Em 10,4% dos casos, houve desmielinização mesmo com correção <10 mEq/L/dia. Esses dados reforçam a necessidade de alvos conservadores para pacientes de alto risco, como 4-6 mEq/L/dia, não ultrapassando o limite de 8 mEq/L/dia.

Experience of Rehabilitation Treatment for Osmotic Demyelination Syndrome with Locked-in Syndrome / The Japanese Journal of Rehabilitation Medicine

Osmotic demyelination syndrome is a pathological condition that leads to electrolyte imbalances and rapid correction, resulting in pseudobulbar palsy, quadriplegia, and altered consciousness. Approximately 33-55％ of affected patients experience residual functional impairment. Herein, we describe a case of a patient with osmotic demyelination syndrome who developed locked-in syndrome during the disease course, underwent rehabilitation treatment, and achieved complete remission without sequelae.The patient was a 47-year-old man who was admitted to hospital A owing to weakness in the lower extremities and dysarthria. He had severe hyponatremia and received sodium correction. However, on hospital day 9, dysarthria redeveloped and involuntary finger movements were noted. Osmotic demyelination syndrome was suspected based on the findings of magnetic resonance imaging of the head and clinical course, leading to his transfer to hospital B. Steroid pulse and rehabilitation therapies were initiated at hospital B. By the 19th day of symptom onset, his limb and facial muscle paralysis progressed, leading to locked-in syndrome. Thereafter, the patient was transferred to hospital C, where he received physiotherapy, occupational therapy, and eating training, markedly improving his physical functions. He was discharged from hospital C, 4 months after the symptom onset, with limited range of motion of the fingers and weakness of the extremities and continued to receive outpatient rehabilitation treatment. His symptoms improved further, and 1 year after the onset of symptoms, he returned to work without any sequelae.

Pontine myelinolysis with an uncommon manifestation of hallucination: a case report / Mielinólise pontina com manifestação incomum de alucinação: relato de caso

ABSTRACT This report aims to present an elderly woman with persistent delirium after hospitalization for lethargy secondary to hyponatremia. The diagnosis of pontine myelinolysis was made and there were no characteristic neurological manifestations such as pupillary changes or spastic tetraparesis. Hallucinations and personality changes were the clues to the diagnosis and should be considered an atypical manifestation of pontine myelinolysis.

RESUMO O objetivo deste relato é apresentar uma idosa que apresentou, após internação por letargia secundária à hiponatremia, delirium persistente depois da alta hospitalar. O diagnóstico de mielinólise pontina foi feito após a alta hospitalar e não houve manifestações neurológicas características, como alterações pupilares ou tetraparesia espástica. Alucinações e mudança de personalidade foram as pistas para o diagnóstico e devem ser consideradas como uma manifestação atípica de mielinólise pontina.

Central pontine myelinolysis syndrome as rare clinical manifestation of fatal refeeding syndrome: a case report / 中华内分泌代谢杂志

The most common neurological clinical manifestations of refeeding syndrome(RFS)are seizures and altered consciousness. This article presents a case in which central pontine myelinolysis(CPM) is a complication of RFS and describes its diagnosis and treatment process. This case highlights the importance of early cranial MRI examination to exclude CPM in patients with persistent hypoghosphatemia and altered consciousness during the course of RFS treatment.

Clinical and imaging characteristics of central pontine myelinolysis without hyponatremia / 中华神经医学杂志

Objective:To investigate the clinical and imaging characteristics of central pontine myelinolysis (CPM) without hyponatremia and explore its pathogenesis.Methods:A retrospective analysis was performed. Six CPM patients without hyponatremia, admitted to Department of Neurology, He'nan Provincial People's Hospital from March 2021 to March 2023 were chosen. Demographic information, causes, medical history, clinical presentations, and MRI features at onset, and 1 and 3 months after onset were analyzed. The prognoses were evaluated by modified Rankin Scale (mRS) scores 3 months after onset: mRS scores≤2 was classified as good prognosis, and mRS scores>2 as poor prognosis.Results:In these 6 CPM patients without hyponatremia, 4 were males and 2 females; 4 patients had dizziness, 3 headache, 4 limb weakness, 2 cognitive decline, and 2 slow reaction. Four CPM patients had a history of hypertension, 5 had a history of diabetes, and 1 had a history of alcoholic cirrhosis. Hormone therapy, nutritional support and symptomatic treatment were given; 5 patients had obvious improvement, and 1 had poor prognosis 3 months after onset. MRI showed asymmetrical abnormal signal in the basal pons and bilateral brachium pontis, with T1WI hypointensity, T2WI hyperintensity, T2-FLAIR hyperintensity, DWI hyperintensity and clear boundary, and without obvious mass effect or enhancement. DWI sequence enjoyed good diagnostic sensitivity in early stage of CPM: high signal changes could occur within 24 h of clinical symptoms, and isointensity 3 months after onset.Conclusion:Causes of CPM without hyponatremia are mostly hypokalemia, diabetes, malnutrition, and chronic alcoholism; its characteristic MRI manifestations are "pig nose sign", "bat wing sign" and "trident sign".

Seven cases of toxic encephalopathy associated with diquat poisoning / 中华急诊医学杂志

Objective:To improve the understanding of the clinical features of toxic encephalopathy associated with diquat poisoning.Methods:This study collected and analyzed the diagnosis and treatment process of 7 patients with acute diquat poisoning combined with central nervous system complications admitted to the First Affiliated Hospital of Zhengzhou University from April 2021 to April 2022. "Diquat" and "Poisoning" were used as keywords to search in CNKI, Wanfang database and PubMed database, and the literature of previous cases was reviewed for summary analysis.Results:Among the 7 patients in our hospital, there were 2 males and 5 females, with an average age of 31 years (range14-57) and an average dose of 23.14 g [(10-40)g]. During the treatment, 3 patients developed irritability and convulsions, 3 patients occurred coma, and one had generalized tonic-clonic seizures. Four patients died and 3 survived, of which 2 patients returned to normal life and study, and one remained abnormal mental behavior (currently in long-term follow-up). All three survivors developed neurological symptoms later than those who died, and were awake about 30 days after taking the drug.Conclusions:Toxic encephalopathy associated with diquat poisoning has rapid progression, poor prognosis and high mortality. This study found that the survival rate of patients with > 48 h of first onset of neurological symptoms is much higher than that of patients with ≤ 48 h of first onset of neurological symptoms, while sex, age, estimated oral dose, and type of presentation of neurological symptoms for the first time have little effect on the survival rate of hospital discharge. The earlier neurological symptoms appear, the greater the likelihood of a poor prognosis.

Probable first report of a motor deafferentation syndrome in the Paraguayan War / Provável primeiro relato de uma síndrome de desaferentação motora na Guerra do Paraguai

ABSTRACT 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.

Mielinolisis extrapontina en una paciente embarazada, con hiperémesis e hiponatremia. Reporte de un caso / Extrapontine myelinolysis in a pregnant with hyperemesis and hyponatremia. A case report

Resumen: ANTECEDENTES: La mielinolisis extrapontina forma parte del síndrome de desmielinización osmótica que lesiona los oligodendrocitos y ocasiona la pérdida de mielina en regiones del sistema nervioso central diferentes al puente del tronco encefálico. Se origina por la corrección rápida de sodio en pacientes con hiponatremia y en mujeres embarazadas se asocia con hiperémesis gravídica. CASO CLÍNICO: Paciente indígena, de 32 años, enviada a un hospital universitario de alta complejidad en Bogotá, desde un territorio de la Amazonia colombiana, en el marco de un nuevo modelo de salud, con embarazo de 15 semanas, quien tuvo un episodio convulsivo, afasia y automatismo bucal, con antecedente de hiperémesis gravídica y otro episodio de hiperémesis con hiponatremia un mes antes. Inicialmente se sospechó eclampsia y neuroinfección; se implementó tratamiento para la corrección rápida del sodio y prescripción de anticonvulsivos. La resonancia magnética cerebral fue compatible con mielinolisis extrapontina. Luego de un mes regresó a su territorio de origen para rehabilitación. Tuvo parto domiciliario a las 38.3 semanas y acudió al hospital para el alumbramiento. CONCLUSIONES: La mielinolisis extrapontina debe considerarse en el diagnóstico diferencial entre embarazo con cuadro neurológico agudo y antecedente de hiperémesis e hiponatremia. Es importante integrar, sistemáticamente, hospitales universitarios o de alta complejidad en los territorios rurales para optimizar el diagnóstico y tratamiento de estas pacientes.

Abstract: BACKGROUND: Extrapontine myelinolysis is part of the osmotic demyelination syndrome, being an acute non-inflammatory demyelinating disease caused by hyperosmotic stress that injures oligodendrocytes and causes myelin loss in regions of the central nervous system other than the pons of the brain stem. Primarily caused by a rapid sodium correction in patients with hyponatremia, in pregnant women its most frequent association is with hyperemesis gravidarum. CLINICAL CASE: A 32-year-old indigenous woman was referred to a university hospital of high complexity, in Bogotá, from a territory of the Colombian Amazon, within the framework of a new health care model, with a 15-week pregnancy, who had a convulsive episode, aphasia and oral automatism, and a recent history of hyperemesis gravidarum. Another history of hyperemesis and hyponatremia a month ago. Initially, eclampsia and neuroinfection were suspected, a rapid correction of sodium, anticonvulsant, remission and management in the intensive care unit was performed. MRI was compatible with extrapontin myelinolysis. After a month she returned to the territory of origin for rehabilitation. She had a home delivery at 38.3 weeks and went to the hospital for placental delivery. CONCLUSION: Extrapontin myelinolysis should be considered in the differential diagnosis between pregnancy with acute neurological symptoms conditions and a history of hyperemesis or hyponatremia. In case of a repeated history of acute hyponatremia in pregnant women with hyperemesis, chronicity should be considered. It is recommended to integrate university hospitals to rural territories to optimize the diagnosis and management of this type of cases.

Osmotic Demyelination Syndrome Presenting with Emotional Incontinence

No abstract available.

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.

Síndrome neuroléptico maligno con mielinolisis central pontina / Neuroleptic malignant syndrome with central pontine myelinolysis

RESUMEN El síndrome neuroléptico maligno (SNM) es una complicación severa de los antipsicóticos, en especial los de primera generación como el haloperidol, que fue el primero en el que se describió esta patología, caracterizada por fiebre, rigidez, alteración del estado de conciencia y disautonomías. Por otro lado, la mielinólisis central pontina (ahora llamada síndrome de desmielinización osmótica) resulta de las alteraciones agudas séricas del sodio, como las que ocurren en las reposiciones de hiponatremia, y podría poner en riesgo la vida al igual que el SNM. La asociación de estas dos patologías es inusual y hasta el momento no se conoce con claridad su relación causal, producto de los pocos casos reportados. Aunque se conoce la mortalidad del síndrome neuroléptico maligno, la compañía de la mielinolisis central pontina podría aumentar la morbimortalidad de esta entidad, por lo cual es necesario reconocerla rápidamente para prevenir la aparición de complicaciones, ya que no cuenta con un tratamiento específico. Presentamos el caso de un paciente joven que cursó con estas dos patologías, y consideramos que la causa de la mielinolisis central pontina fue el haloperidol, así como del SNM. A pesar de ello, este medicamento continúa siendo muy seguro en la práctica clínica ya que la aparición de estas complicaciones es una reacción idiosincrática por algún tipo de susceptibilidad genética desconocida.

SUMMARY Neuroleptic malignant syndrome (NMS) is a severe complication of antipsychotics, especially those of first generation such as haloperidol, which was the first in which this pathology was described, characterized by: fever, rigidity, alteration of the state of consciousness and dysautonomies. On the other side, central pontine myelinolysis (Now Called Osmotic Demyelination Syndrome), search results of acute sodium alterations of sodium, as those occurring in hyponatremia replenings and could put life at risk just like the NMS. The association of these two pathologies is unusual and until now, their causal relationship, the result of the few cases reported, is not clearly known. Although the relationship of the neurological syndrome may be limited, the company of central myelolysis could increase the morbidity of this entity, so it is necessary to be required quickly to prevent the onset of complications, which does not have a specific treatment. We present the case of a young patient who has these pathologies and we consider that the cause of centralized myelinolysis is haloperidol as well as NMS, despite this, this medication continues to be very safe in clinical practice since the appearance of These complications is an idiosyncratic reaction due to some type of unknown genetic susceptibility.

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)

Mielinolisis central pontina como complicación de hiperemesis gravídica / Central pontine myelinolysis as a complication of hyperemesis gravidarum

Central pontine myelinolysis or osmotic demyelination syndrome is a disorder consisting of non-inflammatory demyelination, involving the pons, secondary to swallowing or neuronal edema in patients with severe hyponatremia undergoing rapid sodium replacement, which is considered the main pathophysiological mechanism. The clinical manifestations are diverse and some cases have been reported in pregnant women with hyponatremia induced by severe hyperemesis gravidarum. There is no evidence regarding optimal treatment or prognosis, so it is essential to recognize hyponatremia and perform an adequate correction of sodium levels to avoid this complication. We present the case of a 21-year-old woman with a 12-week pregnancy who presented gait distrubances, polydipsia, speech disorders, dysphonia and intense vomiting with alterations in serum sodium levels. Cerebral magnetic resonance imaging revealed symmetrical hypointense lesions in the region of the pons, with no mass effect or compression of adjacent structures suggestive of osmotic demyelination.

La mielinolisis pontina central, o síndrome de desmielinización osmolar, es un trastorno que consiste en desmielinización no inflamatoria, que involucra la protuberancia secundaria a inflamación o edema neuronal en pacientes con hiponatremia grave sometidos a reemplazo rápido de sodio, el cual se considera el principal mecanismo fisiopatológico. Las manifestaciones clínicas son diversas y se han informado casos en embarazadas con hiponatremia severa inducida por hiperémesis gravídica. No existe evidencia con respecto al tratamiento o pronóstico óptimo, por lo que es esencial reconocer la hiponatremia y realizar la corrección adecuada de los niveles de sodio para evitar esta complicación. Se presenta un caso de una mujer de 21 años con embarazo de 12 semanas que presentó trastornos de la marcha, polidipsia, alteraciones del habla, disfonía y vómitos intensos junto con alteraciones de las concentraciones séricas de sodio. Las imágenes de resonancia magnética cerebral revelaron lesiones hipointensas simétricas en la región de la protuberancia, sin efecto de masa o compresión de estructuras adyacentes sugestivas de desmielinización osmótica.

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.

Achados de fala, deglutição e qualidade de vida na mielinólise extrapontina: relato de caso de uma adolescente com germinoma do sistema nervoso central / Speech, swallowing and quality of life outcomes in extrapontine myelinolysis: a case report of a teenager with central nervous system germ cell tumor

RESUMO O presente trabalho é um relato de caso que descreve os achados fonoaudiológicos e de qualidade de vida apresentados por uma adolescente com um tumor do sistema nervoso central acometida pela mielinólise extrapontina. A mielinólise extrapontina é uma doença desmielinizante aguda que pode ser causada por variações abruptas na osmolaridade sérica, como o que ocorre na rápida correção da hiponatremia. Os dados foram obtidos a partir da avaliação clínica fonoaudiológica, de questionários pediátricos sobre a qualidade de vida e dados contidos no prontuário médico. A paciente apresentou mutismo, disartria e disfagia decorrentes da mielinólise extrapontina, com impactos significativos na sua comunicação verbal, alimentação e qualidade de vida. Os escores de qualidade de vida após a mielinólise extrapontina apresentaram piora quando comparados aos do período anterior à doença. Recebeu atendimento fonoaudiológico hospitalar e ambulatorial. Foram observadas evoluções nos padrões de fala, deglutição e mobilidade das estruturas orofaciais, com reversão parcial dos déficits neurológicos. A atuação fonoaudiológica, como parte de uma equipe multiprofissional de saúde, é de grande importância na reabilitação funcional dos pacientes acometidos por esta doença.

ABSTRACT This paper is a case report describing the speech-language and quality of life outcomes presented by a teenager with a central nervous system germ cell tumor affected by extrapontine myelinolysis. The extrapontine myelinolysis is an acute demyelinating disease that can be caused by abrupt changes in serum osmolality, as in the rapid correction of hyponatremia. Data were obtained from clinical assessment protocols, pediatric inventory about quality of life and medical records information. The patient presented mutism, dysphagia and dysarthria caused by extrapontine myelinolysis, with significant impacts on their verbal communication, feeding and quality of life. The scores of quality of life after extrapontine myelinolysis decreased compared to the time before the disease. She received speech-language therapy during hospital admission and in the outpatient clinic. We observed changes in patterns of speech, swallowing and mobility of orofacial structures; with partially reverse of neurological deficits. The speech therapists, as part of a multidisciplinary team of health care, are of great importance in the functional rehabilitation of patients affected by this disease.

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.

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.

Leucopatía reversible de la protuberancia: en paciente con SIDA y tratamiento retroviral / Pontine reversible leucopathy in an AIDS patient associated with highly active antiretroviral therapy (HAART): report of one case

Posterior reversible encephalopathy (PRES) is a condition characterized by T2 and FLAIR hyperintensities in magnetic resonance imaging (MRI) studies, localized preferentially in the occipital-parietal white matter regions. Pathological MRI images located in midbrain, pons, medulla and spinal cord, that could be asymptomatic, were recently included in this entity. These images are interpreted as vasogenic edema, which is caused by arterial hypertension or eclampsia, neurotoxicity related to immunosuppressive agents or chemotherapy, among other causes. We report a 25 years old asymptomatic male with AIDS, with normal blood pressure who after initiating highly active antiretroviral therapy (HAART) reported vertigo. The MRI showed a central pontine T2 hyperintensity with diffusion restriction, which was interpreted as a central pontine myelinolysis (CPM), but the lack of motor symptoms made improbable a real demyelination of the pons. The follow-up MRI revealed complete regression of the images. To our knowledge, this case could be the second report of a reversible leucopathy of the pons in a patient with AIDS, were the MRI images also simulated a CPM. This report extends the knowledge around the variability of the pathogenic interpretation of CPM images and their association with HAART.

Primary Sjogrens Syndrome with Renal Tubular Acidosis with Central Pontine Myelinolysis – A rare case report

A middle aged female patient, a case of Primary Sjogren’s Syndrome with renal tubular acidosis as revealed by severe hypokalemia along with normal anion gap, metabolic acidosis and acidic urinary pH had brain stem lesion which presented as quadriplegia, dysphagia and dysarthria. Laboratory tests revealed that anti-nuclear antibodies (ANA) and anti Ro/SSA antibodies were positive. MRI showed hyper intense lesion in T2W images in middle pons, typical characteristic of central pontine myelenolysis. So, patient was diagnosed as Primary Sjogren’s Syndrome with renal tubular acidosis with central pontine myelinolysis. She recovered with correction of hypokalemia, intravenous methyl prednisolone and cyclophosphamide.

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.