Osmotic demyelination syndrome: Difference between revisions

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==Background==
==Background==
Osmotic demyelination syndrome (ODS; also formerly called central pontine myelinolysis or CPM) is a neurologic condition caused by rapid correction of hyponatremia, and is characterized by dysarthria, dysphagia, lethargy, paraparesis or quadriparesis, seizures, coma, and death.(2) Osmotic demyelination syndrome was first described in a 1959 paper by Adams, Victor, and Mancall, who described rapidly evolving quadriplegia and pseudobulbar palsy in 3 alcoholic men.  
*Formerly called "central pontine myelinolysis"
*A neurologic condition caused by rapid correction of hyponatremia, with starting serum sodium normally 120 meq/L or less
*Caused by rapid correction of hyponatremia (>12 mEq/L/24 h), as water moves from cells to extracellular fluid, yielding intracellular dehydration.
*Symptoms are often irreversible or only partially reversible


===Risk Factors===
===Risk Factors===
Osmotic demyelination syndrome is caused by rapid correction of hyponatremia (>12 mEq/L/24 h) as water moves from cells to extracellular fluid, yielding intracellular dehydration. Starting serum sodium concentration is almost always 120 meq/L or less. Risk factors for osmotic demyelination syndrome include: chronic heart failure, alcoholism, cirrhosis, hypokalemia, malnutrition, and treatment with vasopressin antagonists such as tolvaptan.(1)
*Chronic [[heart failure]]
*[[Alcoholism]]
*[[Cirrhosis]]
*[[Hypokalemia]]
*[[Malnutrition]]
*Treatment with vasopressin antagonists (e.g. tolvaptan)
 
===Risk Factors for Over-correction<ref>George, J. C., Zafar, W., Bucaloiu, I. D., & Chang, A. R. (2018). Risk Factors and Outcomes of Rapid Correction of Severe Hyponatremia. Clinical Journal of the American Society of Nephrology: CJASN, 13(7), 984–992.</ref>===
*Lower initial sodium
*[[Schizophrenia]]
*Lower baseline urine sodium


==Clinical Features==
==Clinical Features==
Both men and women are equally affected.  Symptoms can be present two to six days after inappropriately rapid correction of the serum sodium concentration has occurred. Symptoms are often irreversible or only partially reversible, and include dysarthria, dysphagia, paraparesis or quadriparesis, behavioral disturbances, movement disorders, seizures, lethargy, confusion, disorientation, obtundation, and coma.  Severely affected patients may develop "locked in" syndrome. (3)
''Symptoms can be present 2-6 days after rapid correction of serum sodium''  
 
*[[Dysarthria]]
MRI can be used to visualize the pontine lesion with a characteristic "batwing" lesion of the pons appearing in typical cases.(2)
*[[Dysphagia]]
*[[Lethargy]]
*Behavioral disturbances/ confusion
*[[weakness|Paraparesis]] or quadriparesis
*[[Seizures]]
*"Locked in" syndrome
*[[Coma]] and [[death]]


==Differential Diagnosis==
==Differential Diagnosis==
 
{{AMS DDX}}


==Evaluation==
==Evaluation==
*Evaluate for alternative/reversible causes of AMS or exacerbating factors
*[[brain MRI|MRI]] can be used to visualize the pontine lesion, with a characteristic "batwing" lesion of the pons appearing in typical cases


 
==Management<ref>Sterns RH and Hix JK. Overcorrection of hyponatremia is a medical emergency. Kidney International. Volume 76, Issue 6, 2 September 2009, Pages 587-589.</ref>==
==Management==
*[[Desmopressin]] at 2 mcg q6 hrs IV/SC
In patients with chronic severe hyponatremia (Na <120mEq), the correction rate of sodium should not exceed 6 mEq/24 hours for patients with other ODS risk factors, or 12 mEq/24 hours for those without other risk factors (1).  Hypertonic (3%) saline should be given at a low infusion rate, 0.5 to 1 mL/kg/h, with frequent serum sodium checks to ensure that the correction rate does not exceed the above limits.
*6 mL/kg of 5% dextrose in water, repeated until serum sodium rise back below 9 mEq in 24 hrs
*If concerns for iatrogenic rapid increase in serum Na concentration (IV crystalloid bolus before Na lab comes back):
**Stop IV fluids, send urine osmolality, repeat serum Na
**If uOsm is < 100 mOsm/L, this suggests free water diuresis
**Or if sodium appears to be on a trajectory to increase >8mEq/L in 24hrs
**Administer DDAVP 1 mcg x1


==Disposition==
==Disposition==
*Admit
*Admit
==Prevention==
See [[hyponatremia]] for safe correction rate


==See Also==
==See Also==
 
*[[Hyponatremia]]
*[[Hypernatremia]]


==References==
==References==

Latest revision as of 23:19, 27 August 2021

Background

  • Formerly called "central pontine myelinolysis"
  • A neurologic condition caused by rapid correction of hyponatremia, with starting serum sodium normally 120 meq/L or less
  • Caused by rapid correction of hyponatremia (>12 mEq/L/24 h), as water moves from cells to extracellular fluid, yielding intracellular dehydration.
  • Symptoms are often irreversible or only partially reversible

Risk Factors

Risk Factors for Over-correction[1]

Clinical Features

Symptoms can be present 2-6 days after rapid correction of serum sodium

Differential Diagnosis

Altered mental status

Diffuse brain dysfunction

Primary CNS disease or trauma

Psychiatric

Evaluation

  • Evaluate for alternative/reversible causes of AMS or exacerbating factors
  • MRI can be used to visualize the pontine lesion, with a characteristic "batwing" lesion of the pons appearing in typical cases

Management[2]

  • Desmopressin at 2 mcg q6 hrs IV/SC
  • 6 mL/kg of 5% dextrose in water, repeated until serum sodium rise back below 9 mEq in 24 hrs
  • If concerns for iatrogenic rapid increase in serum Na concentration (IV crystalloid bolus before Na lab comes back):
    • Stop IV fluids, send urine osmolality, repeat serum Na
    • If uOsm is < 100 mOsm/L, this suggests free water diuresis
    • Or if sodium appears to be on a trajectory to increase >8mEq/L in 24hrs
    • Administer DDAVP 1 mcg x1

Disposition

  • Admit

Prevention

See hyponatremia for safe correction rate

See Also

References

  1. George, J. C., Zafar, W., Bucaloiu, I. D., & Chang, A. R. (2018). Risk Factors and Outcomes of Rapid Correction of Severe Hyponatremia. Clinical Journal of the American Society of Nephrology: CJASN, 13(7), 984–992.
  2. Sterns RH and Hix JK. Overcorrection of hyponatremia is a medical emergency. Kidney International. Volume 76, Issue 6, 2 September 2009, Pages 587-589.