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Central pontine myelinolysis
Definition
Central pontine myelinolysis is brain cell dysfunction caused by the destruction of the layer (myelin sheath) covering nerve cells in the middle of the brainstem (pons).
Alternative Names
Osmotic demyelination syndrome
Causes
The destruction of the myelin sheath that covers nerve cells prevents signals from being properly transmitted from one nerve to another.
The most common cause of central pontine myelinolysis is a quick rise in the body's sodium levels. This most often occurs when someone is being treated for low blood levels of sodium (hyponatremia) and the sodium is replaced too fast. It also can occasionally occur when high levels of sodium in the body (hypernatremia) are corrected too quickly.
This condition does not occur on its own. It is a complication of treatment for other conditions or from the other conditions themselves.
Risks include:
- Alcoholism
- Liver disease
- Malnutrition from serious illnesses
Symptoms
- Confusion, delirium
- Balance problems
- Difficulty swallowing
- Hallucinations
- Reduced alertness, drowsiness or sleepiness, lethargy, poor responses
- Speech changes, poor enunciation
- Tremor
- Weakness in the face, arms, or legs, usually affecting both sides of the body
Exams and Tests
An examination may show:
- Abnormal reflexes
- Confusion
- Involvement of all four arms and legs (spastic quadriplegia)
- Weakness of the face, arms, and legs (upper motor neuron syndromes)
A head MRI scan may reveal a problem in the brainstem (pons). This is the main diagnostic test.
Other tests may include:
- Blood sodium levels and other blood tests
- Brainstem auditory evoked response (BAER)
Treatment
This is an emergency disorder. You will need to go to a hospital for diagnosis and treatment. However, most people with this condition are already in the hospital for another condition.
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 usually long-lasting. The disorder can cause serious long-term (chronic) disability.
Possible Complications
- 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
Gradual, controlled treatment of low sodium levels may reduce the risk of nerve damage in the pons. Being aware of how some medications can change sodium levels can prevent these levels from changing too quickly.
References
Rosenberg GA. Brain edema and disorders of cerebrospinal fluid circulation. In: Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC, eds. Bradley’s Neurology in Clinical Practice. 6th ed. Philadelphia, Pa: Saunders Elsevier; 2012:chap59.
Skorecki K, Ausiello D. Disorders of sodium and water homeostasis. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 118.
Reviewed By: Luc Jasmin, MD, PhD, Department of Neurosurgery at Cedars-Sinai Medical Center, Los Angeles, and Department of Anatomy at UCSF, San Francisco, CA. Review provided by VeriMed Healthcare Network. David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.
