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 en trasplante hepático / Central pontine myelinolysis after liver transplantation. Report of five cases

Background: Central Pontine Myelinolysis (CPM) is the most severe neurological complication after liver transplantation and apparently is not related to changes in osmolarity. Aim: To report five transplanted patients with CPM. Patients and Methods: In a series of 27 patients subjected to liver transplantation between 2005 and 2008, we found five patients who developed CPM. Results: All patients presented a severe hepatic encephalopathy. In the absence of alterations in osmolality, they developed, between the second to seventh day after transplantation, a central quadriplegia, hyperreflexia and Babinski sign, with preservation of sensorium. Magnetic resonance imaging showed demyelination of the motor pathway only in the protuberance. Motor recovery first began in the fingers and hands, followed by forearms, toes, feet, arms and finally the legs, defining a somatotopic recovery of the cortico-spinal pathway. Conclusions: This form of regaining motility shows that the selective involvement of the pyramidal tract in CPM, is according to its location in the pons and suggests a local entrapment. It is due to the structural rigidity of the protuberance that limits the expansive requirements of cytotoxic and vasogenic edema, and only affects the long fibers of cortico-spinal tracts, sensitized by encephalopathy. The entrapment syndromes generally preserve the axons, injure myelin and have no inflammatory reactions.

Central myelinolysis in a patient with hyponatraemia

We present a case of a 50-year old man who developed mutism and a flaccid quadriparesis within 48 hours of presentation to hospital with severe hyponatraemia. A diagnosis of central pontine myelinolysis was made based on the clinical features and typical appearances on 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.

Central pontine and extrapontine myelinolysis: report of a case with a tragic outcome

OBJECTIVE: To report a pediatric case of central pontine and extrapontine myelinolysis, a rare neurological disease often associated with rapid correction of hyponatremia.DESCRIPTION: A 15 year-old female adolescent developed locked-in syndrome during severe hyponatremia. Brain magnetic resonance imaging was consistent with the diagnosis of central pontine and extrapontine myelinolysis.COMMENTS: Serum sodium correction should proceed slowly and cautiously, based upon a careful calculation of sodium deficit, in order to minimize metabolic stress and avoid the occurrence of this dreadful complication, which has a tragic outcome in most cases. There is no scientifically proved effective treatment for myelinolysis, and severe cases usually have a dismal prognosis.

Mielinólisis osmótica extrapóntica / Extrapontine osmotic myelinolysis

La mielinólisis osmótica extrapóntica es una complicación rara del sistema nervioso central. Se presentan los hallazgos clínicos de un paciente de 49 años, alcohólico, que ingresó al servicio de urgencias en paro cardiorrespiratorio. Después de las maniobras de reanimación, el paciente se encontró en acidosis metabólica (pH 7,014) y fue tratado con bicarbonato de sodio. Cuarenta y ocho horas después, el sodio aumentó de 142 a 174 mEq/l. Durante el curso clínico, el paciente presentó signos de compromiso cognitivo, disartria, amaurosis bilateral, hiporreflexia y hemiparesia doble de predominio en el hemicuerpo derecho. La tomografía computarizada, tomada a las 72 horas de inicio del cuadro, evidenció hipodensidad lenticular bilateral con compromiso de las cápsulas internas y externas. Un mes después el paciente continuó con compromiso cognoscitivo, atrofia óptica bilateral, disartria residual, bradicinesia y hemiparesia doble

Mielinólise pontina e extra-pontina associada a Shoshin beribéri em paciente etilista / Central pontine and extra-pontine myelinosis in alcoholic patient with Shoshin beriberi

A mielinólise pontina está classicamente associada à rápida correção de hiponatremia crônica. Recentemente, fatores importantes adicionais tem sido descritos na patogênese dessa condição. Relatamos o caso de um paciente de 43 anos, etilista, desnutrido, que apresentou quadro agudo de insuficiência cardíaca por "Shoshin beribéri", insuficiência renal com tratamento por diálise. Evoluiu com tetraparesia e coma. Apresentou mielinólise pontina central e extra-pontina à ressonância magnética de crânio e anormalidades no potencial evocado auditivo.

Mielinólisis pontina despuÚs de un evento de hipoglucemia / Pontine myelinolysis after a hypoglycemic episode

A case of central pontine myelinolysis following hypoglycemia is reported. The case was a 26- year-old female. Diabetes mellitus was found when she was 8 years old and she has hypertension and renal failure. She suffered a severe hypoglycemia at an unknown time. After the episode she developed a vegetative state. A magnetic resonance scan showed features consistent with the presence of central pontine myelinolysis.

Mielinolisis central pontina e hiponatremia, un problema no resuelto: caso clínico / Central pontine myelinolysis and hyponatremia: report of 1 case

There is a controversy wheter central pontine myelinolysis can complicate either hyponatremia or its rapid correction. We report a 69 years old woman with a history of one month of vertigo, nausea, vomiting and diarrhea. She was admitted dehydrated ad stuporous, and initial laboratory values showed a serum sodium of 96 mEq/L She was treated with dextrose 5 percent and 3 percent NaCI. Serum sodium raised to 120 mEq/L at the next day and the level of consciousness improved. At the 4th day of admission, the patient was again stuporous and with spastic quadriplegia. A magnetic resonance imaging showed a central and symmetrical pontine lesion on T1 and T2 weighed images. Thereafter, the patient experienced a progressive improvement of her neurological condition and was discharged three months later, moving her lower limbs. Nine month later she was able to walk

Mielinolisis central pontina por corrección de hiponatremia dilucional y trastornos neurológicos afines / Central pontine myelinolysis due to dilutional treatment of hyponatremia and related neurological disorders

La mielinosis Central Pontina es una enfermedad caracterizada por la pérdida de mielina, con relativa buena reserva del cuerpo neuronal, que afecta a la base pontina y otras áreas encefálicas y que se vincula a la rápida corrección de una hiponatremia crónica. Se presentan tres casos, dos con lesiones óculomotoras irreversibles y una muerte. También se incluyen otros dos casos que sobreviven en buenas condiciones, con trastornos ocasionados por disionías (y su tratamiento) que caen en el diagnóstico diferencial de la Mielinolisis Central Pontina, tal como la Encefalopatía de Wernicke. Se resalta que todos los casos fueron del sexo femenino

Mielinolisis central pontina inducida por hiperemesis gravidica / Central pontine myelinolysis induced by hyperemesis gravidarum

Presentamos a un paciente de sexo feminino, de 18 años, primigesta, con hiperemesis gravídica, deshidratada, que no presentaba hiponatremia. La paciente desarolló nistagmus multidireccional, desorientación temporoesdpacial, apatía y marcha atáxica luego de la admisión al hospital; cuadro compatible con encefalopatía de wernicke. El laboratorio demostraba hipocaliemia, hipernatremia y aumento de las aminotransferasas. La osmolaridad plasmática calculada fue 319 mOsm/Kg y el déficit de agua de 2.73 I. Evolucionó con pérdida de la fuerza en cuatro miembros, hipotoncidad, reflejos tendinosos ausentes y signo de Babinski bilateral. En la resonancia magnética nuclear de cerebro se observaba, en cortes sagitales, una imagen hiperintensa en T2 a nivel pontino y en cortes axiales una banda central hipointensa en T1 sugestivas de mielinolisis central pontina. En el caso de nuestra paciente la mielinosis central pontina probablemente resulta secundaria a la asociación de: hipernatremia, hipersomolaridad de hipocaliemia.

Probable mielinolisis central pontina asociada a intoxicación por órganofosforados: hipótesis sobre una nueva etiología / Central pontine myelinolysis associated to organophosphorous insecticide

La mielinolisis central pontina (MCP) es un cuadro de alteración neurológica aguda y progresiva patológicamente caracterizada por desmielinización protuberancial y/o extraprotuberancial, etiológicamente asociada a una inadecuada corrección de la hiponatremia, por lo que también se denomina Síndrome de desmielinización osmótica. Presentamos una paciente intoxicada accidentalmente con un compuesto órganofosforado (OF), que presentó inicialmente un cuadro clínico y de laboratorio secundario a la inhibición y recuperación aguda de la actividad plasmática de la acetilcolinesterasa (intoxicación de Tipo 1). La evolución neurológica posterior resultó compatible con lesión de tallo cerebral. No se registraron alteraciones de la temperatura, la natremia, el pH ni los gases en sangre. En estadio agudo las imágenes por Resonancia Magnática (RM), mostraron lesión centroprotuberancial única y ovoide, hipointensa en T1 e hiperintensa en T2, sin edema periférico ni efecto de masa, sin signos hemorrágicos y sin cambios con el gadolinio, hallazgos estrechamente correlacionados con probable MCP. No se evidenciaron lesiones extrapontinas. Un examen neurológico de control a los 90 días no mostró anormalidades, mientras que en las imágenes por RM la lesión protuberancial mostraba signos involutivos; ambos resultados son coincidentes con los de trabajos previos sobre MCP en su evolución tardía. Se descartaron otras causas de lesión protuberancial en base a los antecedentes, la forma de presentación y la evolución aguda y crónica, tanto clínica como en RM. Se discuten los mecanismos de acción patogénica de los compuestos OF sobre la mielina, que apoyan la hipótesis etiológica propuesta. Concluimos que la relación causal, la evolución neurológica y los hallazgos en RM en una paciente normonatrémica, permiten proponer a la intoxicación por compuestos OF como una probable nueva etiología de MCP

Mielinolisis póntica central con síndrome de enclaustramiento

Tha case of a 55 year old man who presented with severe global headache 8 hours after having been drinking heavily, and who over a two hour period developed deep coma and flaccid paralysis of all four extremities is reported. A CT scan of the brain was normal; however, on Magnetic Resonance Imaging (MRI) extensive hyperintense areas in the brain stem, mainly in the pons, were easily identified. Based upon the clinical and MRI findings a diagnosis of Central Pontine Myelinolysis was made. Following several months of supportive therapy the patient partially recovered and was able to leave the hospital.