Osmotic Demyelination Syndrome Resulting from an Unexpected Response to Tolvaptan in a Patient with Heart Failure / 대한내과학회지

Hyponatremia is commonly encountered in patients with heart failure and has a poor prognosis. Tolvaptan, a novel selective vasopressin V2 receptor blocker, has received attention as an effective drug for treating the syndrome of inappropriate antidiuretic hormone secretion and hypervolemic hyponatremia. However, the safety of tolvaptan in the treatment of hyponatremia is not clear. We experienced a 78-year-old woman with a history of heart failure, atrial fibrillation, and hyponatremia who developed osmotic demyelination syndrome as an unexpected response to treatment with tolvaptan.

A Case of Osmotic Demyelination Presenting with Severe Hypernatremia

Osmotic demyelination syndrome is a demyelinating disorder associated with rapid correction of hyponatremia. But, it rarely occurs in acute hypernatremia, and it leads to permanent neurologic symptoms and is associated with high mortality. A 44-year-old woman treated with alternative medicine was admitted with a history of drowsy mental status. Severe hypernatremia (197mEq/L) with hyperosmolality (415mOsm/kgH2O) was evident initially and magnetic resonance imaging revealed a high signal intensity lesion in the pons, consistent with central pontine myelinolysis. She was treated with 0.45% saline and 5% dextrose water and intravenous corticosteroids. Serum sodium normalized and her clinical course gradually improved. Brain lesion of myelinolysis also improved in a follow-up imaging study. This is the first report of a successful treatment of hypernatremia caused by iatrogenic salt intake, and it confirms the importance of adequate fluid supplementation in severe hypernatremia.

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)

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.

A Case of Osmotic Demyelination Syndrome in a Patient with Severe Hyponatremia Complicated by Rhabdomyolysis / 영남의대학술지

Hyponatremia, the most common electrolyte disorder, has been rarely reported as causing rhabdomyolysis. Osmotic demyelination syndrome (ODS), a demyelinating disease of the central pons and/or other areas of the brain, is infrequently reported as associated with rapid correction of hyponatremia. This paper reports a case of ODS after correction of severe hyponatremia complicated by rhabdomyolysis. A 47-year-old female with a history of chronic alcoholism presented herself at the hospital with altered consciousness after three days of nausea and vomiting. She was on a thiazide diuretic for essential hypertension. Her blood tests upon her hospital admission showed hyponatremia (Na+ 98 mEq/L), hypokalemia (K+ 3.0 mEq/L), and elevation of her serum creatine phosphokinase (3,370 IU/L) with an increase in her serum myoglobin level 11,267 ng/mL). She was treated with intravenous fluid therapy that included isotonic and hypertonic salines along with potassium chloride. She became more alert, and her neurological condition gradually improved after the first five days of her therapy. On the ninth day after her admission, she developed progressive quadiaresis associated with dysarthria, dysphagia, and dystonia despite the resolution of her hyponatremia. Magnetic resonance imaging of her brain on 16th day revealed symmetrical areas of signal hyperintensity in her central pons, basal ganglia, and precentral gyrus in T2-weighted images, which are consistent with ODS. Her neurological symptoms steadily improved after six weeks with only supportive treatment and rehabilitation.

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.

A Case of Osmotic Demyelination Syndrome after Hemodialysis Treatment in a Uremic Patient with Severe Hyperosmolality / 대한신장학회잡지

Osmotic demyelination syndrome (ODS) is a distinctive clinical syndrome with characteristic MR features in the central pons (central pontine myelinolysis) and in other brain locations (extrapontine myelinolysis). Rapid correction of hyponatremia is associated with ODS, but hyperosmolality rarely causes it. Here we report a case of ODS developed in a hemodialysis patient with severe uremia and concomitant hyperosmolality. A 67-year-old male was presented with general weakness and dyspnea. Laboratory findings showed severe uremia (blood urea nitrogen, 167 mg/dL; serum creatinine, 15 mg/dL), hyperosmolality (336 mOsm/kg H2O), and normal range of sodium (145 mEq/L). After the first hemodialysis treatment, his consciousness was changed to lethargic state. We initially suspected dialysis disequilibrium syndrome, but the symptoms were aggravated. MRI showed edema in central pons and symmetrical extrapotine lesions in the subcortical white matter, lateral thalamus, and posterior capsule, consistent with ODS. In spite of aggressive measures including continuous renal replacement therapy, he died of sepsis at the 26th hospital day.

Cortical Laminar Necrosis associated with Osmotic Demyelination Syndrome

Cortical laminar necrosis has been rarely observed in osmotic demyelination syndrome. We report a 32-year-old female patient who became comatose after the rapid correction of hyponatremia. There were high signal intensities in the pons and bilateral deep gray nuclei on T2-weighted MRI images, and linear hyperintensities along the cerebral cortices on T1-weighted images with a diffuse gyriform enhancement. MR spectroscopic findings showed a decrease of the N-acetyl aspartate peak and an increase in those of the lipid and lactate complex. The case demonstrates that a severe form of osmotic demyelination syndrome accompanying cortical laminar necrosis can result from the rapid correction of hyponatremia.

Initial Transient Neurologic Recovery Followed by Delayed Deterioration of Osmotic Demyelination Syndrome: A Case Report / 대한신장학회잡지

In literatures, most of the studies of severe hyponatremia during or following its treatment has been concentrated with special references to the rate of correction and its neurologic outcomes. But, there is relatively few ones analyzing the diverse clinical manifestations of neurologic symptorns or complications during the course of treating severe hyponatremia. We experienced a catastrophic course related to hyponatremia in a 51 year woman with severe rheumatoid arthritis, who underwent knee joint replacement, and this case revealed the initial transient neurologic recovery for 3 days by the initial rapid correction of hyponatremia, then followed by delayed deterioration of osmotic demyelination syndrome leading to locked-in syndrome. Reported cases with similar clinical course (biphasic course) in the world lituratures were reviewed with special interests in the initial maximum rate of correction of hyponatremia and radiologic findings. This review suggests that clinicians treating the patients with severe symptomatic hyponatremia should be aware of the possibility of delayed neurologic sequelae despite the recovery of neurologic status as well as the degree of hyponatremia in the early treatment course of hyponatremia.