Osmotic demyelination syndrome: plasmapheresis versus intravenous immunoglobulin?

Abstract

A 63-year-old man with a past medical history of nonalcoholic steatohepatitis cirrhosis complicated by hepatic encephalopathy and non-bleeding esophageal varices presented for orthotopic liver transplantation. The patient had no acute complications in the immediate post-operative period, and was extubated on post-operative day (POD) 1. At that time, he was neurologically intact, alert and oriented and with no focal neurological deficits. On POD 3, he became lethargic and quadriplegic (Medical Research Council Scale Grade 0), and developed right-sided focal seizures with secondary generalization. His serum sodium was 128 mmol/L. He was re-intubated, and treated for his seizures with lorazepam 4 mg and levetiracetam 2 g, and then continued on levetiracetam 1 g two times a day. The following day, he was unresponsive and had no motor response to painful stimuli. His serum sodium had corrected without additional exogenous intervention to 135 mmol/L. On post symptom onset day (PSOD) 3, an MRI brain without contrast showed chronic small vessel ischemic changes but no other abnormality (Fig. 1a). The EEG did not show any seizure or epileptiform discharges. Serum chemistry and cerebrospinal fluid analysis did not show any significant abnormalities. On PSOD 13, his presentation remained the same. An MRI brain was repeated showing DWI restriction and high T2 signal in the central pons, suggestive of ODS (Fig. 1b). On PSOD 19, he was started on both IVIG and PP for a total of 5 days. Approximately 3 weeks after treatment with IVIG and Plasmapheresis, a repeat MRI showed similar prominence of T2 hyperintensity in the central pons with sparing of the periphery as compared to prior, findings consistent with central pontine myelinolysis/osmotic demyelination syndrome (Fig. 1c). Over the next 90 days the patient improved, becoming fully alert, regaining spontaneous muscle flicker in all four extremities (Medical Research Council Scale Grade 1), full eye movements and the ability to swallow.Osmotic demyelination syndrome (ODS) is a disorder characterized by the destruction of neuronal myelin sheaths in either the central area of the pons as in central pontine myelinolysis (CPM), or in other susceptible areas such as the basal ganglia, hippocampi or cerebellum known as external pontine myelinolysis (EPM). CPM can present with T2 hyperintensities on MRI in a classic trident-shape pattern. ODS usually presents as a complication of rapid correction of hyponatremia. Although no specific treatment has been described, plasmapheresis (PP) and intravenous immunoglobulin (IVIG) have been suggested as possible options for the management of ODS [1]. A clear association has been established between rapid correction of hyponatremia and the development of ODS. Although not completely understood, the pathophysiology of ODS classically described is the reduced extracellular osmolality causing brain cells to release osmotically-active substances in an attempt to achieve osmotic equilibrium. These osmotic substances cannot be reabsorbed rapidly, an...

Incidence of osmotic demyelination syndrome in Sweden: A nationwide study

“…Case reports have shown promising use of corticosteroids, intravenous immunoglobulins (IVIG), or plasmapheresis when symptoms of ODS have already begun to emerge. In our cohort, only one patient received IVIG treatment (none with corticosteroids or plasmapheresis).…”

Favorable Outcomes Following the Use of Triple Therapy in a Patient with Osmotic Demyelination Syndrome

“…However, these recommendations are based on small case series. Atchaneeyasakul et al 10 theorized that PP reduces the myelinotoxic products released following the osmotic stress. Five cases treated with PP resulted in TREATMENT OF OSMOTIC DEMYELINATION SYNDROME continued.…”

“…[14][15][16][17] Treatment with both PP and IVIG has been trialed as well with significant improvement in four patients. 10,18,19 The use of intravenous methylprednisolone has been described as well, however, further studies are needed to establish the true efficacy of the use of steroids in this setting. 3,20 Case reports usually describe regimens with successful outcomes.…”

Osmosinės mielinolizės sindromas (centrinė tilto ir ekstrapontininė mielinolizė): literatūros apžvalga ir atvejo pristatymas

“…Ðio gy dymo po vei kis yra su si jaes su mie lo tok si niø me dþia gø ir an timie li ni niø an ti kû nø su si da ry mo su ma þë ji mu ir re mie li niza ci jos pa lai ky mu [48]. K. At cha ne e y a sa kul su ben dra darbiais ap þvel gë dau gu mà pa skelb tø CTM at ve jø, kai gy dymui bu vo skir ta IVIG ir (ar) GA [50]. Vi sø ti ria mø jø pacien tø neu ro lo gi në bûk lë pa ge rë jo, ta èiau da lis jø li ko su neu ro lo gi niu de fi ci tu.…”

“…Dau ge liu ap ra ðy tø CTM at ve jø, sëk -min gai gy dy tø IVIG ir (ar) GA, gy dy mas bu vo pra dë tas pir mà jà simp to mø at si ra di mo sa vai tae. Kai gy dy mas bû davo ini ci juo tas pra ëjus dau giau lai ko, neu ro lo gi nis pa ge rë jimas bû da vo ne toks ryð kus [50].…”