Mielinosis extrapontina: reporte de un caso de síndrome de desmielinización osmótica

Introducción: El síndrome de desmielinización osmótica es una entidad clínica la cual supone un reto diagnóstico por su curso en muchos casos silentes y por su cuadro clínico que se presenta típicamente de manera tardía. Se describen dos tipos de desmielinización osmótica que pueden presentarse de manera aislada o simultánea: la mielinosis central pontina y la mielinosis extrapontina. Caso clínico: femenina de 21 años sin antecedentes personales con episodio convulsivo tónico clónico por primera vez en su vida. Se le encuentra a su ingreso un nivel de sodio sérico disminuido, razón por la cual se inicia la corrección intravenosa del mismo. Cursó con cefaleas persistentes y vómitos a las 48 horas posterior a la corrección del sodio, y se realizó una resonancia magnética cerebral que evidenció datos de mielinosis extrapontina. Conclusión: La corrección rápida de los niveles de sodio se asocia a la posibilidad de desarrollar mielinosis pontina. La precaución en la corrección de la hiponatremia es la piedra angular para prevenir el desarrollo de esta condición. (provisto por Infomedic International)

Introduction: Osmotic demyelination syndrome is a clinical entity which poses a diagnostic challenge because of its often-silent course and its clinical picture which typically presents late. We describe two types of osmotic demyelination that can occur in isolation or simultaneously: central pontine myelinosis and extrapontine myelinosis. Case report: 21-year-old female with no personal history with tonic-clonic seizure episode for the first time in her life. On admission she was found to have a decreased serum sodium level, for which reason intravenous sodium correction was started. He presented with persistent headaches and vomiting 48 hours after sodium correction, and a brain MRI was performed, which showed evidence of extrapontine myelinosis. Conclusion: Rapid correction of sodium levels is associated with the possibility of developing pontine myelinosis. Caution in correcting hyponatremia is the cornerstone in preventing the development of this condition. (provided by Infomedic International)

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.

Síndrome de desmielinização osmótica: relato de caso com evolução favorável / Osmotic demyelination syndrome: report of a case with favorable outcome

Os autores relatam um caso de paciente apresentando evolução favorável após confirmado diagnóstico de síndrome de desmielinização osmótica (mielinólise pontina central e extrapontina) através de exame de ressonância magnética.

The authors report a case of a patient with favorable outcome after diagnosis of osmotic demyelination syndrome (central pontine and extrapontine myelinolysis) confirmed by magnetic resonance imaging.

Pontine and extrapontine osmotic myelinolysis after the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) associated with fluoxetine: case report

Osmotic demyelination syndrome (ODS) may be precipitated by aggressive correction of a hypo or hyper-osmolar states. We describe the case of a 53-year-old woman that was started on fluoxetine 20 mg/day for depression and nine days later was found to have fluoxetine-induced syndrome of inappropriate secretion of antidiuretic hormone. After hyponatremia correction the mental status of the patient gradually improved, but subsequently she had intermittent difficulty in speaking, naming objects, memory deficits and psychomotor slowness. Magnetic resonance revealed bilateral symmetric hyperintense lesions in the basal ganglia, temporal lobe and hippocampal formation compatible with ODS. These symptoms gradually resolved and she was discharged home without any deficits. Two months later, a new image showed lesion in pons and the other lesions had disappeared. Fluoxetine therapy had never been related with a complication like that.

A síndrome de desmielinização osmótica (SDO) pode ser precipitada pela correção agressiva de um estado hiper ou hipoosmolar. Nós descrevemos o caso de mulher de 53 anos que havia iniciado o uso de fluoxetina 20 mg/dia para depressão e que nove dias depois foi diagnosticada como tendo síndrome da secreção inapropriada de hormônio antidiurético induzida por fluoxetina. Depois da correção da hiponatremia o estado mental da paciente gradualmente melhorou, mas subsequentemente ela apresentou dificuldade intermitente para fala e para nomear objetos, déficits de memória recente e lentidão psicomotora. Ressonância magnética revelou lesões hiperintensas bilaterais e simétricas na região dos gânglios da base, lobo temporal e hipocampo compatíveis com SDO. Estes sintomas gradualmente se resolveram e a paciente foi de alta sem qualquer déficit. Dois meses mais tarde uma nova imagem cerebral mostrou lesão na ponte e ausência das lesões antigas. Até onde sabemos a terapia com fluoxetina nunca foi relacionada a uma complicação tardia como esta.