ABSTRACT
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)
Subject(s)
Humans , Female , Pregnancy , Adult , Pregnancy Complications , Myelinolysis, Central Pontine/diagnosis , Postpartum PeriodABSTRACT
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.
Subject(s)
Humans , Female , Middle Aged , Antidepressive Agents , Brain , Hyponatremia , Myelinolysis, Central Pontine/diagnosis , Myelinolysis, Central Pontine/pathology , Magnetic Resonance Imaging , Tomography, X-Ray ComputedABSTRACT
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.
Subject(s)
Adult , Female , Humans , Middle Aged , Liver Transplantation/adverse effects , Myelinolysis, Central Pontine/etiology , Brazil , Fatal Outcome , Magnetic Resonance Imaging , Myelinolysis, Central Pontine/diagnosis , Persistent Vegetative State/etiology , Quadriplegia/etiologyABSTRACT
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.
Subject(s)
Humans , Male , Middle Aged , Hyponatremia/complications , Myelinolysis, Central Pontine/etiology , Mutism/etiology , Quadriplegia/etiology , Hyponatremia/drug therapy , Hyponatremia/physiopathology , Myelinolysis, Central Pontine/diagnosis , Myelinolysis, Central Pontine/therapy , Quadriplegia/diagnosisABSTRACT
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.
Subject(s)
Humans , Female , Adolescent , Hyponatremia/diagnosis , Myelinolysis, Central Pontine/diagnosis , Cerebrum/pathology , Fatal Outcome , Hyponatremia/etiology , Hyponatremia/therapy , Magnetic Resonance Imaging , Myelinolysis, Central Pontine/etiology , Sodium/bloodABSTRACT
La mielinosis central pontima (MCP) es un sindrome neurológico que afecta a adultos jóvenes y de mediana edad y es habitualmente provocado por una corrección de la hiponatremia efectuada en forma demasiado rápida. La evolución clínica varía notablemente desde recuperación casi total a secuelas nerológicas severas e incluso la muerte. Se decribe un caso de mielinosis central pontina en un paciente con hiponatremia y alcoholista, que se presentó con un sindrome cerebeloso sin signos piramidales y en el que la velocidad de corrección del sodio sérico se realizó dentro de los límites recomendados. Se hace énfasis en que para evitar el desarrollo de la mielinosis central pontina, la corrección del sodio sérico en casos de hiponatremia no debe ser más rápida de 12mEg/L en 24 hs y no mayor de 0.5 a 1 m Eg/L/hora.
Subject(s)
Humans , Male , Myelinolysis, Central Pontine/diagnosis , Myelinolysis, Central Pontine/physiopathology , Myelinolysis, Central Pontine/therapyABSTRACT
La Mielinolisis Central Pontina es una enfermedad desmielinizante no inflamatoria infrecuente que se ha asociado a reposición de sodio en hiponatremias severas y sintomáticas. No obstante existen otros factores que pueden alterar la homeostasis y así favorecer la mielinolisis; tales factores deben ser tomados en cuenta ante el manejo de una hiponatremia. Se plantean las diferentes causas que pueden contribuir a producir una mielinolisis pontina y se discute el caso de un paciente quien posterior a politrauma presenta múltiples complicaciones médicas incluyendo hiponatremia que asociado a otros factores generan una mielinolisis pontina demostrada. Palabras claves: Mielinolisis central pontina, hiponatremia, desnutrición.
Subject(s)
Humans , Male , Aged , Accidents, Traffic/mortality , Magnetic Resonance Spectroscopy , Myelinolysis, Central Pontine/diagnosis , Myelinolysis, Central Pontine/mortality , Myelinolysis, Central Pontine , Costa RicaABSTRACT
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.
Subject(s)
Humans , Male , Adult , Alcoholism/complications , Beriberi/complications , Myelinolysis, Central Pontine/etiology , Acute Kidney Injury , Beriberi/diagnosis , Malnutrition/complications , Myelinolysis, Central Pontine/diagnosisABSTRACT
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.
Subject(s)
Humans , Female , Pregnancy , Adult , Puerperal Disorders , Persistent Vegetative State , Diabetes Mellitus, Type 1 , Hypoglycemia , Myelinolysis, Central Pontine/etiology , Apnea , Puerperal Disorders , Hypoxia, Brain , Fetal Death , Insulin , Cerebral Cortex/pathology , Cerebral Cortex , Pregnancy in Diabetics , Myelinolysis, Central Pontine/diagnosis , Myelinolysis, Central Pontine/pathology , Heart Arrest/etiology , Heart Arrest/therapyABSTRACT
Central pontine myelinosys [CPM] is a pathological and clinical syndrome due to destruction of medullated sheaths with relative sparing of the axis cylinders and nerve cells in the pons. Clinicaly it is characterised by rapidly evolving quadriplegia and pseudobulbar palsy. In children, the most common etiology is the rapid correction of hyponatremia, and rarely other disorders like adrenal insufficiency, hyperglycaemic coma, liver disease and leukemia have been associated with CPM. The condition is often fatal and survivors have been reported to suffer from permanent neurological deficit in the form of 'locked in' stated. We report a girl who developed steroid resistant nephrotic syndrome, and during the course of her illness became hyponatremic also. Over enthusiastic correction of the latter, she developed CPM but recovered totally in a few days
Subject(s)
Humans , Female , Myelinolysis, Central Pontine/diagnosis , HyponatremiaABSTRACT
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.
Subject(s)
Female , Humans , Pregnancy , Adolescent , Hyperemesis Gravidarum/complications , Myelinolysis, Central Pontine/etiology , Hypernatremia/complications , Hypernatremia/diagnosis , Magnetic Resonance Spectroscopy , Myelinolysis, Central Pontine/diagnosis , Myelinolysis, Central Pontine/drug therapy , Osmolar ConcentrationABSTRACT
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
Subject(s)
Humans , Female , Insecticides, Organophosphate/toxicity , Myelinolysis, Central Pontine/etiology , Magnetic Resonance Spectroscopy , Calcium/adverse effects , Calmodulin , Diagnostic Imaging , Cholinergic Fibers/pathology , Reticular Formation/pathology , Magnetic Resonance Imaging , Insecticides, Organophosphate/pharmacokinetics , Myelinolysis, Central Pontine/diagnosis , Myelinolysis, Central Pontine/physiopathology , Pesticides/poisoningABSTRACT
A 9 year old boy presented with fever, drowsiness, quadriparesis and facial myokymia. MRI showed demyelination in the pontine region. A diagnosis of central pontine myelinolysis was made. Literature review revealed the rarity of uneventful recovery as has been seen in our case.
Subject(s)
Child , Down Syndrome/diagnosis , Humans , Magnetic Resonance Imaging , Male , Myelinolysis, Central Pontine/diagnosis , Neurologic Examination , Prognosis , Tomography, X-Ray ComputedABSTRACT
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.