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Central pontine myelinolysis

Osmotic demyelination syndrome

 

Central pontine myelinolysis is brain cell dysfunction. It is caused by the destruction of the layer (myelin sheath) covering nerve cells in the middle of the brainstem (pons).

Causes

 

When the myelin sheath that covers nerve cells is destroyed, signals from one nerve to another aren't properly transmitted. Although the brainstem is mainly affected, other areas of the brain can also be involved.

The most common cause of this disorder is a quick change in the body's sodium levels. This most often occurs when someone is being treated for low blood sodium (hyponatremia) and the sodium is replaced too fast. Sometimes, it occurs when a high level of sodium in the body (hypernatremia) is corrected too quickly.

This condition does not usually occur on its own. Most often, it's a complication of treatment for other problems, or from the other problems themselves.

Risks include:

  • Alcohol use
  • Liver disease
  • Malnutrition from serious illnesses

 

Symptoms

 

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

 

Exams and Tests

 

The health care provider will perform a physical exam and ask about the symptoms.

A head MRI scan may reveal a problem in the brainstem (pons) or other parts of the brain. This is the main diagnostic test.

Other tests may include:

  • Blood sodium level and other blood tests
  • Brainstem auditory evoked response (BAER)

 

Treatment

 

This is an emergency disorder that needs to be treated in the hospital though most people with this condition are already in the hospital for another problem.

There is no known cure for central pontine myelinolysis. Treatment is focused on relieving symptoms.

Physical therapy may help maintain muscle strength, mobility, and function in weakened arms and legs.

 

Outlook (Prognosis)

 

The nerve damage caused by central pontine myelinolysis is often long-lasting. The disorder can cause serious long-term (chronic) disability.

 

Possible Complications

 

Complications may include:

  • Decreased ability to interact with others
  • Decreased ability to work or care for self
  • Inability to move, other than to blink eyes ("locked in" syndrome)
  • Permanent nervous system damage

 

When to Contact a Medical Professional

 

There is no real guideline on when to seek medical attention, because this condition is rare in the general community.

 

Prevention

 

In the hospital, slow, controlled treatment of a low sodium level may reduce the risk for nerve damage in the pons. Being aware of how some medicines can change sodium levels can prevent the level from changing too quickly.

 

 

References

Pfennig CL, Slovis CM. Electrolyte disorders. In: Marx JA, Hockberger RS, Walls RM, et al, eds. Rosen's Emergency Medicine: Concepts and Clinical Practice. 8th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 125.

Weissenborn K, Lockwood AH. Toxic and metabolic encephalopathies. In: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. Bradley's Neurology in Clinical Practice. 7th ed. Philadelphia, PA: Elsevier; 2016:chap 84.

 
  • Central nervous system

    Central nervous system - illustration

    The central nervous system is comprised of the brain and spinal cord. The peripheral nervous system includes all peripheral nerves.

    Central nervous system

    illustration

    • Central nervous system

      Central nervous system - illustration

      The central nervous system is comprised of the brain and spinal cord. The peripheral nervous system includes all peripheral nerves.

      Central nervous system

      illustration

    Self Care

     

      Tests for Central pontine myelinolysis

       

         

        Review Date: 5/30/2016

        Reviewed By: Amit M. Shelat, DO, FACP, Attending Neurologist and Assistant Professor of Clinical Neurology, SUNY Stony Brook, School of Medicine, Stony Brook, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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