What is Extrapontine Myelinolysis?

What is Extrapontine Myelinolysis?

Central pontine myelinolysis (CPM) is a demyelinating disorder characterized by the loss of myelin in the center of the basis pontis, usually caused by rapid correction of chronic hyponatremia. On rare occasions, demyelination occurs outside the pons and is termed extrapontine myelinolysis (EPM).

What are symptoms of osmotic demyelination syndrome?

Symptoms may include any of the following:

• Confusion, delirium, hallucinations.
• Balance problems, tremor.
• Problem swallowing.
• Reduced alertness, drowsiness or sleepiness, lethargy, poor responses.
• Slurred speech.
• Weakness in the face, arms, or legs, usually affecting both sides of the body.

Is osmotic demyelination syndrome same as central pontine Myelinolysis?

The neurologic manifestations associated with overly rapid correction have been called the osmotic demyelination syndrome (ODS; formerly called central pontine myelinolysis or CPM). As will be described below, almost all patients who develop ODS present with a serum sodium concentration of 120 mEq/L or less.

Can you recover from central pontine Myelinolysis?

CPM was originally believed to have a mortality rate over 50 percent , but early diagnosis has improved the outlook. Although some people still die as a result of the brain damage sustained, many people with CPM are able to recover. Recent research puts the survival rate at around 94 percent .

What condition can be Iatrogenically caused by reversing hyponatremia too rapidly?

Too-rapid correction of sodium can cause osmotic demyelination syndrome (ODS), a form of brain damage.

Can hyponatremia cause pontine Myelinolysis?

Central pontine myelinolysis is a rare neurologic condition most frequently caused by the rapid correction of hyponatremia.

What is osmotic Myelinolysis?

Osmotic myelinolysis (OM) is an acute, non-inflammatory demyelinating disease that can develop following rapid correction of hyponatremia from any cause.

Is osmotic demyelination syndrome fatal?

Osmotic demyelination syndrome (ODS) is a rare, preventable, and potentially fatal complication of rapid correction of hyponatremia.

What is locked-in syndrome?

Locked-in syndrome is a rare neurological disorder in which there is complete paralysis of all voluntary muscles except for the ones that control the movements of the eyes.

Is pontine Myelinolysis curable?

The disorder can’t be cured, but its symptoms can be treated. CPM is one of the two types of osmotic demyelination syndrome (ODS). The other type, known as extrapontine myelinosis (EPM), occurs when myelin is destroyed in areas of the brain that aren’t in the brain stem.

What happens if you correct hyponatremia too quickly?

But new evidence shows that when patients with hyponatremia get admitted to the hospital, their impatient treatment teams often correct sodium levels too quickly, increasing the risk for dangerous complications. Too-rapid correction of sodium can cause osmotic demyelination syndrome (ODS), a form of brain damage.

What is the prognosis of extrapontine myelinolysis?

Extrapontine myelinolysis could make a complete recovery with symptomatic support alone. During hyponatraemia correction, rapid osmotic shifts of fluid that cause hypernatremia, causes myelinolysis rather than absolute serum sodium level.

What is extrapontine myelinolysis with Parkinsonism like features?

Extrapontine myelinolysis presenting with parkinsonism like features may be a challenging clinical syndrome with wide differential including neurodegenerative disease, vascular and metabolic conditions and exposure to toxin (see table 1 ).

What are the signs and symptoms of central pontine myelinolysis (CPM)?

Central pontine myelinolysis (CPM) may follow correction of hyponatraemia and generally presents with tetraparesis and brain stem dysfunction that may include pontine dysfunction, pseudobulbar palsy and a ‘locked-in’ syndrome.

Does hyponatraemia cause myelinolysis?

Even gradual correction of hyponatraemia can produce myelinolysis, especially with pre-existing malnourishment, alcoholism, drug misuse, Addison’s disease and immuno-suppression. Pallidial sparing is typical of EPM in MRI scans.