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.

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.

A Rare Case of Acute Psychosis as an Isolated Manifestation of Extrapontine Myelinolysis.

Aims and Background: Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are recognized as osmotic demyelination syndrome (ODS). ODS is pathologically characterized by non-inflammatory demyelination of several brain structures with sparing of axons. This condition is usually associated with overzealous correction of hyponatraemia. Acute psychosis as the sole clinical manifestation is extremely rare. Presentation of Case: Hence, we report an interesting case of a middle-aged man who was diagnosed with EPM, following rapid correction of hyponatraemia and subsequently developed acute psychosis. He made a good recovery with supportive treatment alone. Discussion and Conclusion: The possibility of psychosis as a manifestation of ODS, particularly in patients with recent correction of hyponatraemia. The rate of correction of plasma sodium level is the key point for preventing ODS and its complications.

Osmotic Demyelination Syndrome: Why Should Hypernatremia be Corrected Slowly : A Case Report.

Aim: Our aim is to present a case of hypernatremia which has led on to a flaccid quadriparesis due to brain stem demyelination. Rapid correction of hypernatremia as a cause for pyramidal tract demyelination is not documented in the literature. Presentation of Case: A 53 year old male was brought to the emergency services with suspected stroke. He was treated with intravenous mannitol and oral glycerine from the primary health centre. We detected hypoglycemia (blood sugarwas 50mg/dl-Ref range: ≤70mg%) and dextrose was given intravenously. Subsequently the patient went into a hypernatremic state with serum sodium 170milli equivalents /liter which was corrected rapidly. This was corrected over 48 hours to 140milli equalents/litre. The rate of correction exceeded 0.62millimols/liter/hour (Ideal: 0.5 mmol/L/h). On the 6th day the patient developed acute quadriparesis. Magnetic resonance imaging (MRI) of brain revealed bilateral symmetric demyelination of the corticospinal tracts. Over six months the neurological deficit improved with complete resolution of the changes in previous Discussion: Osmotic Demyelination Syndrome (ODS) has been a recognized complication of rapid correction of hyponatremia. Experiments in animals and clinical experience suggest that correction of chronic hyponatremia should be kept at a slow rate to combat this complication. The characteristic sites include pons and basal ganglia. Such a complication has not been described due to rapid correction of hypernatremia.This is probably the first case report in the literature where acute onset of quadriparesis resulted from demyelination of the pyramidal tract consequent to a rapid correction of hypernatremia. We had to wait about 6 months for the patient to obtain a complete functional recovery and the neuro imaging was repeated after 6 months to confirm the disappearance of the initial findings thus implicating rapid correction of hypernatremia as the cause of his morbidity. Conclusion: This is the first time extrapontine reversible myelinolysis due to rapid correction of hypernatremia has been documented. To prevent this potentially fatal complication it will be prudent if hypernatremia is corrected slowly.

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.

Clinical Features and Prognosis of Central Pontine Myelinolysis / 中国康复理论与实践

@#Objective To analyze the clinical features and prognosis of central pontine myelinolysis. Methods 20 cases with CPM diagnosed in last 10 years were reviewed and compared with those patients of foreign studies. Results 10 patients with hemoptysis were treated with hypophysin, which resulted in severe hyponatremia and osmotic myelinolysis. The main syndrom were dysarthria and dysphagia (secondary to corticobulbar fiber involvement), flaccid quadriparesis (as corticospinal tract involved) which later became spastic. The myelinolysis distrubuted symmetrically along the myelin disruption in the center basal, as well as independently in other brain areas (extrapontine myelinolysis or EPM), including the cerebellar and neocortical white/gray junctional areas, thalamus and striatum. Conclusion Supplement of sodium should be slower and more persistent for hyponatremia, the selection of drugs should be based on the patients' specific conditions.

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.

Secondary Parkinsonism due to Extrapontine Myelinolysis without Central Pontine Myelinolysis: A case report

Parkinsonism is characterized by clinical symptoms of rigidity, bradykinesia, tremor, and gait disturbance. There are many causes of secondary parkinsonism. The present report discusses a secondary parkinsonism of extrapontine myelinolysis (EPM) without central pontine myelinolysis (CPM) after rapid correction of hyponatremia. EPM is a demyelinating process of the brain. EPM with CPM is relatively common, but EMP without CPM is rare. A 57- year-old woman with Tsutsugamushi disease had a subacuteonset of parkinsonism on the fourteenth day after a rapid correction of severe hyponatremia. Magnetic resonance imaging of the bilateral basal ganglia showed a high signal intensity on T2-weighted images, and a low signal on T1- weighted images. She was started on levodopa, and parkinsonian features slowly improved. We report an unusual case of EPM without CPM, that presented with parkinsonism.

Neuropsychological Findings of Extrapontine Myelinolysis without Central Pontine Myelinolysis: Initial and Follow-up Evaluation

Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are well recognized syndromes related to the rapid correction of hyponatremia. Cognitive dysfunction and neuropsychological findings, however, have seldom been reported. We present a case of EPM without CPM and report the neuropsychological findings. Neuropsychological testing showed severe impairment of attention, verbal and visual memory, visuospatial functioning, and frontal/executive functioning. Language and language-related functions were normal except for naming. Follow-up neuropsychological testing at 4 months later showed slightly less impairment than the first assessment.

Complete Neurological Recovery in Two Cases of Osmotic Demyelination Syndrome Following Rapid Correction of Chronic Hyponatremia / 대한신장학회지

The osmotic demyelination syndrome (ODS) is a distinctive clinical entity with characteristic MR features in the central pons (central pontine myelinolysis, CPM) and other locations (extrapotine myelinolysis, EPM). ODS is mainly seen following rapid correction of the serum sodium level in hyponatremic patients. In the past, ODS used to be considered as fatal. However, some recent reports have described cases of survival from this syndrome, but most survivors seem to suffer irreversible neurological deficits. We report one case of 46-year-old woman who developed stupor at day 7 and the other case of 56-year-old woman with drowsiness, dysarthria and dysphagia at day 3 following the correction of hyponatremia. In both cases, the serum potassium levels were low at the time of presentation with hyponatremia. By means of brain MRI, the first case was diagnosed as CPM with EMP and the second case as isolated EPM. With conservative treatments, complete neurological recovery was achieved at 4-6 weeks after onset of ODS.

Developed Extrapotine Myelinolysis after Hysteroscopic Myomectomy: A case report / 대한마취과학회지

Transcervical resection of endometrium is under-utilized in the treatment of dysfunctional uterine bleeding, uterine myoma and menorrhagia. The procedure is similar to transurethral resection of prostate in men with a possibility of substantial absorption of irrigation fluid. Absorption of a large volume of fluid can cause excessive intravascular volume, hyponatremia, cerebral edema and death. Severe hyponatremia leading to extrapontine myelinolysis is an extremely rare complication of this procedure. So, We report a case of developed extrapontine myelinolysis after hysteroscopic myomectomy which, however, showed complete recovery.

Asymptomatic Extrapontine Myelinolysis in Diabetic Woman

Osmotic myelinolysis syndrome in central nervous system is classified into central pontine myelinolysis(CPM) and extrapontine myelinolysis(EPM). The former can cause a spastic quadriplegia, pseudobulbar palsy, and varying degree of mental disorder, but the latter a movement disorder(tremor, dystonia, parkinsonism, and chorea, etc), behavior change(mood instability, personality change, agitated delirium, and disinhibition, etc), and cognitive dysfunction. Although a few cases of asymptomatic CPM have been reported, asymptomatic EPM were rare. A 67-year-old woman with diabetes mellitus and old cerebral infarction has suffered from the common bile duct stone. She had hyponatremia(Na=126mEq/L) on admission and was corrected rapidly. Incidental findings of her brain MRI showed EPM. We experienced a case of bilateral middle cerebellar peduncle myelinolysis incidentally without neurological presentations and report a rare case of asymptomatic EPM recoverd in diabetic woman after rapid correction of hyponatremia.

A Case of Subacute Onset Choreoathetosis as Sequelae of Central Pontine and Extrapontine Myelinolysis

Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are recognized complications of hypona-tremia and its overly rapid correction. A 42-year-old woman with uterine cervical cancer had a subacute onset of choreoathetosis on the thirteenth day after a rapid correction of severe hyponatermia. A follow-up brain magnetic reso-nance image (MRI) showed abnormal signal intensity changes in the central pons, putamen, and caudate nucleus. We report an unusual case of central pontine and extrapontine myelinolysis, that presented with choreoathetosis. (J Korean Neurol Assoc 19(4):393~395, 2001)

Central Pontine and EXtrapontine Myelinolysis

The knowledge about nutritional, toxic, and metabolic causes of dementia is particularly important, because they may be reversible. Central pontine myelinolysis(CPM) is one of these causes. CPM is a well known but rare metabolic disease of unknown etiology linked to overly aggressive correction of hyponatremia. We report a 74-year-old woman who developed disorientation, memory disturbance, and behavioral problem following intensive care unit management for pneumonia. Mini-mental status examination-Korean version(MMSE-K) study revealed severe cognitive dysfunction. Brain magnetic resonance imaging showed changes consistent with CPM and extrapontine myelinolysis. After supportive care, patient's clinical status was significantly improved. We suggest that a metabolic problem such as CPM should be considered in the diagnosis of acute or subacute cognitive deterioration in elderly patients.