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'''For patient information, click [[Central pontine myelinolysis (patient information)|here]]'''
__NOTOC__
[[Image: Pontine myelinolysis.jpg|thumb|Right|PX:400|Loss of myelinated fibers at the basilar part of the pons in the brainstem (Luxol-Fast blue stain)


{{Infobox_Disease
Source: Wikipedia: Jensflorian
| Name          = Central pontine myelinolysis
| Image          = Illu pituitary pineal glands.jpg
| Caption        = Pons labeled at bottom left
| DiseasesDB    = 2198
| ICD10          = {{ICD10|G|37|2|g|35}}
| ICD9          =
| ICDO          =
| OMIM          =
| MedlinePlus    = 000775
| MeshID        = D017590
}}
{{SI}}
{{CMG}}


{{SK}} CPM; osmotic demyelination syndrome; osmotic myelinolysis; central pontine myelinosis
<nowiki>https://en.wikipedia.org/wiki/Central_pontine_myelinolysis#Pathophysiology</nowiki>]]
{{Central pontine myelinolysis}}
'''For patient information, click [[Central pontine myelinolysis(patient information)|here]]'''


==Overview==
{{CMG}}; {{AE}} {{MMJ}}
'''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==
{{SK}}
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 overview|Overview]]==
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 historical perspective|Historical Perspective]]==
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>


==Diagnosis==
==[[Central pontine myelinolysis classification|Classification]]==
 
==[[Central pontine myelinolysis pathophysiology|Pathophysiology]]==
 
==[[Central pontine myelinolysis causes|Causes]]==


===Symptoms===
==[[Central pontine myelinolysis differential diagnosis|Differentiating Central pontine myelinolysis from other Diseases]]==
*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===
==[[Central pontine myelinolysis epidemiology and demographics|Epidemiology and Demographics]]==
Imaging by [[Magnetic resonance imaging|MRI]] demonstrates an area of high signal return on T2 weighted images.


==Treatment==
==[[Central pontine myelinolysis risk factors|Risk Factors]]==
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:


===Hyponatremia===
==[[Central pontine myelinolysis screening|Screening]]==
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===
==[[Central pontine myelinolysis natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
The rate of correction of [[hypernatremia]] should be at 0.5meq/L/hr and should not exceed 12 meq/Li/24hrs.


== References ==
==Diagnosis==
{{reflist|2}}
[[Central pontine myelinolysis diagnostic study of choice|Diagnostic study of choice]] | [[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 electrocardiogram|Electrocardiogram]] | [[Central pontine myelinolysis x ray|X-Ray Findings]] | [[Central pontine myelinolysis echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Central pontine myelinolysis CT scan|CT-Scan Findings]] | [[Central pontine myelinolysis MRI|MRI Findings]] | [[Central pontine myelinolysis other imaging findings|Other Imaging Findings]] | [[Central pontine myelinolysis other diagnostic studies|Other Diagnostic Studies]]


{{Diseases of the nervous system}}
==Treatment==
[[de:Zentrale pontine Myelinolyse]]
[[Central pontine myelinolysis medical therapy|Medical Therapy]] | [[Central pontine myelinolysis interventions|Interventions]] | [[Central pontine myelinolysis surgery|Surgery]] | [[Central pontine myelinolysis primary prevention|Primary Prevention]] | [[Central pontine myelinolysis secondary prevention|Secondary Prevention]] | [[Central pontine myelinolysis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Central pontine myelinolysis future or investigational therapies|Future or Investigational Therapies]]


{{WH}}
==Case Studies==
{{WS}}
[[Central pontine myelinolysis case study one|Case #1]]


[[Category:Disease]]
[[Category: (name of the system)]]
[[Category:Overview complete]]
[[Category:Neurology]]
[[Category:Intensive care medicine]]
[[Category:Nephrology]]
[[Category:Emergency medicine]]

Revision as of 19:23, 23 December 2019

Loss of myelinated fibers at the basilar part of the pons in the brainstem (Luxol-Fast blue stain) Source: Wikipedia: Jensflorian https://en.wikipedia.org/wiki/Central_pontine_myelinolysis#Pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Central pontine myelinolysis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

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