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{{SK}} CPM; osmotic demyelination syndrome; osmotic myelinolysis; central pontine myelinosis
{{SK}} CPM; osmotic demyelination syndrome; osmotic myelinolysis; central pontine myelinosis


==Overview==
==[[Central pontine myelinolysis overview|Overview]]==
'''Central pontine myelinolysis''' is a neurologic disease caused by severe damage of the [[myelin sheath]] of [[neuron|nerve cells]] in the [[brainstem]], more precisely in the area termed the ''[[pons]]''.  It is a complication of treatment of patients with profound, life threatening [[hyponatraemia]]. It occurs as a consequence of a rapid rise in serum tonicity following treatment in individuals with chronic severe hyponatraemia who have made intracellular adaptations to the prevailing hypotonicity. It can also occur as a complication of correcting [[hypernatremia]] too rapidly.


==Pathophysiology==
==[[Central pontine myelinolysis historical perspective|Historical Perspective]]==
Brain cells adjust their osmolarities by changing levels of certain [[osmolytes]] like [[Inositol]], [[Betaine]], and [[Glutamine]]. In [[hyponatremia]] the levels of these osmolytes fall, preventing entry of free-water into cells. The reverse is true for [[hypernatremia]]. So rapid correction of [[sodium]] in [[hyponatremia]] would cause the extra cellular fluid to be relatively hypertonic. Free-water would then move out of the cells. This leads to central pontine myelinolysis.  Rapid correction of [[hypernatremia]] causes water to move into cells, leading to multiple cerebral hemorrhages, equally catastrophic as osmotic demyelination.


==Causes==
==[[Central pontine myelinolysis pathophysiology|Pathophysiology]]==
The most common cause is the rapid correction of low blood sodium levels ([[hyponatremia]]). Over rapid correction of high levels of salt in the blood ([[hypernatremia]]) can also cause the condition.


==Risk Factors==
==[[Central pontine myelinolysis causes|Causes]]==
It has been postulated that one underlying cause may be the lack of a substance that is essential for brain activity and is lacking due to [[malnutrition]]. The fact that this condition is most frequently observed in patients with general ill health ([[alcoholism]], [[cachexia]] etc.) is in accordance with this hypothesis. <ref>Adams RA, Victor M, Mancall EL. Central pontine myelinolysis: a hitherto undescribed disease occurring in alcoholics and malnourished patients. ''Arch Neurol Psychiatry.'' 1959;81:154–72.  PMID 13616772</ref>


==Natural History, Complications, Prognosis==
==[[Central pontine myelinolysis differential diagnosis|Differentiating Central pontine myelinolysis from other Diseases]]==
The nerve damage caused by central pontine myelinolysis is usually long-lasting, and the disorder can cause serious long-term (chronic) disability.


===Complications===
==[[Central pontine myelinolysis epidemiology and demographics|Epidemiology and Demographics]]==
*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


==Diagnosis==
==[[Central pontine myelinolysis risk factors|Risk Factors]]==


===Symptoms===
==[[Central pontine myelinolysis natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
*Sudden para or [[quadraparesis]], [[dysphagia]], [[dysarthria]], [[double vision]] and [[coma|loss of consciousness]].
*[[Confusion (patient information)|Confusion]], [[Delirium (patient information)|delirium]]
*[[Balance problems]]
*[[Difficulty swallowing]]
*[[Hallucination (patient information)|Hallucinations]]
*[[Reduced alertness]], [[Drowsiness (patient information)|drowsiness]] or [[sleepiness]], [[lethargy]], poor responses
*Speech changes, poor enunciation
*[[Tremor]]
*[[Muscle weakness (patient information)|Weakness]] in the face, arms, or legs, usually affecting both sides of the body
*[[Locked-in syndrome]] where cognitive function is intact, but all muscles are paralyzed with the exception of eye blinking
 
===MRI===
Imaging by [[Magnetic resonance imaging|MRI]] demonstrates an area of high signal return on T2 weighted images.


==Diagnosis==
[[Central pontine myelinolysis history and symptoms|History and Symptoms]] | [[Central pontine myelinolysis physical examination|Physical Examination]] | [[Central pontine myelinolysis laboratory findings|Laboratory Findings]] | [[Central pontine myelinolysis CT|CT]] | [[Central pontine myelinolysis MRI|MRI]] | [[Central pontine myelinolysis echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Central pontine myelinolysis other imaging findings|Other Imaging Findings]] | [[Central pontine myelinolysis other diagnostic studies|Other Diagnostic Studies]]
==Treatment==
==Treatment==
To avoid myelinolysis, the correction of hyponatremia should not exceed 1 mEq/L per hour. <ref>Kleinschmidt-DeMasters BK, Norenberg MD. Rapid correction of hyponatremia causes demyelination: relation to central pontine myelinolysis. ''Science.'' 1981;211(4486):1068-70. PMID 7466381</ref><ref>Laureno R. Experimental pontine and extrapontine myelinolysis. ''Trans Am Neurol Assoc.'' 1980;105:354-8. PMID 7348981</ref>  There is no specific treatment and the syndrome is associated with high mortality and morbidity. This being a potentially avoidable disaster, following recommendations may be adhered to while maintaining sodium levels:
[[Central pontine myelinolysis medical therapy|Medical Therapy]] | [[Central pontine myelinolysis prevention|Prevention]] | [[Central pontine myelinolysis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Central pontine myelinolysis future or investigational therapies|Future or Investigational Therapies]]
 
==Case Studies==
===Hyponatremia===
[[Central pontine myelinolysis case study one|Case #1]]
The rate of correction of [[hyponatremia]] should be 0.5-1.0meq/L/hr, with not more than a 12 meq/l correction in 24 hrs. If the patient has ongoing [[seizures]] (or [Na<sup>+</sup>]<115 meq/li), correction can be attempted at up to 2 meq/L/hr, but only while [[seizure activity]] lasts and the [Na<sup>+</sup>] exceeds 125-130 meq/Li.
 
===Hypernatremia===
The rate of correction of [[hypernatremia]] should be at 0.5meq/L/hr and should not exceed 12 meq/Li/24hrs.


== References ==
{{reflist|2}}


{{Diseases of the nervous system}}
{{Diseases of the nervous system}}

Revision as of 13:47, 28 September 2012

Central pontine myelinolysis
Pons labeled at bottom left
ICD-10 G37.2
DiseasesDB 2198
MedlinePlus 000775
MeSH D017590

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Synonyms and keywords: CPM; osmotic demyelination syndrome; osmotic myelinolysis; central pontine myelinosis

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