Osmotic demyelination syndrome in a normonatremic patient of chronic kidney disease

Abstract

Osmotic Demyelination Syndrome (ODS) is associated with rapid correction of hyponatremia or fluid shifts, and is characterized by neurological involvement related to pons, brainstem or other areas of the brain. All possible measures should be taken to prevent this serious disorder. Diagnosing this condition early is very important and requires a high index of suspicion. The treatment is purely supportive and most patients may show dramatic recovery. ODS occurring in normonatremic and hypernatremic patients is very rare. We report a case of an 18-year-old boy of end-stage renal disease who presented with an episode of acute gastroenteritis. He was managed with aggressive intravenous fluids, hemodialysis and other supportive therapy. But, he developed altered sensorium and seizures that progressed to features of spastic quadriparesis and lower cranial nerve palsy. Neuroimaging showed hyperintensities in pons and midbrain suggestive of ODS. The patient had normal sodium levels at all times and had no evidence of hyponatremia. The patient was managed with hemodialysis, physiotherapy and other conservative measures and had a gradual clinical and radiological recovery.

The osmotic demyelination syndrome: the resilience of thalamic neurons is verified with transmission electron microscopy

“…95 ) and some authors who have even entitled 'ultrastructure' in their reports only illustrated with light microscopy (i.e. 54,94,96 ) while others showed morpho-functional interconnections. [97][98][99][100]…”

“…29 In the old and current literature, number of reports of regional CPM and EPM ODS defects showed as consequences of abrupt adjustment of a temporary or chronic deficiency of the homeostatic sodium gradient. Its etiology can be found in a series of diverse afflictions, 30 such as in these exemplary list of clinical studies: alcoholism and/or combined with malnutrition, 1,19,[31][32][33][34][35][36][37][38][39][40] craniofacial and neurosurgeries, 41,42 diarrhea with AIDS, 43 excessive vomitus alone 44 or associated with pregnancy, 45,46 hyper glycemia, 47 pediatric diabetes, 48 diabetes insipidus due to cancer treatments, 49 folate deficiency, and kidney defect, 50 Grave's disease, 51 diuretic unbalance, and heart failure, [52][53][54][55] heat exhausting exercise, 56,57 liver cirrhosis, 58,59 or liver transplantation. 29,60,61 Disorders of sodium and osmotic homeostasis have been found in diverse small mammals.…”

Osmotic Demyelination Syndrome - Evolution of Extrapontine Before Pontine Myelinolysis on Magnetic Resonance Imaging

“…[1011] It also occurred with gradual correction of hyponatremia as well as in normonatremic and hypernatremic patients. [12131415] Several conditions predisposing patients to its development were also found. These include renal failure patients on dialysis, liver failure, and transplantation, malignancies such as lymphoma, carcinoma, cachexia, sepsis, acute pancreatitis, pellagra, and chronic alcoholism.…”

Tongue fasciculations with denervation pattern in osmotic demyelination syndrome: a case report of diagnostic dilemma

“…Our patient had the typical MRI findings [ 10 , 26 ] of ODS and so the clinical and radiological evidence was supportive of our diagnosis of ODS. Even though our patient did not have a documented hyponatremia, it is not a must in ODS/CPM [ 4 , 5 , 8 , 27 – 32 ]. In a systematic review of studies on central pontine and extra-pontine myelinolysis around one quarter of patients with ODS/CPM had sodium of more than 135 mmol/L [ 10 ].…”

“…Even though it usually occurs after rapid correction of hyponatremia, it can occur even in normonatremic patients, especially who have risk factors like alcoholism, malnutrition, liver disease, liver transplantation, diabetes mellitus, hypokalemia, pituitary surgery, lithium toxicity, hypophosphatemia, chemotherapy and chronic renal failure [ 4 – 9 ]. The clinical manifestations of ODS include various degrees of encephalopathy, behavioral disturbances, lethargy, confusion, disorientation, obtundation, dysarthria and dysphagia [ 10 , 11 ].…”