Central Pontine Myelinolysis: Difference between revisions

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== Clinically Relevant Anatomy<br>  ==
== Clinically Relevant Anatomy<br>  ==
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== Mechanism of Injury / Pathological Process<br>  ==
== Mechanism of Injury / Pathological Process<br>  ==


add text here relating to the mechanism of injury and/or pathology of the condition<br>  
Central pontine myelinolysis is a concentrated, frequently symmetric, noninflammatory demyelination within the pons. The area involved is usually confined to the basis pontis, but occasionally involves the tegmentum too.<br>
 
It is one of the demyelinating condions, and was first described by Adams et al in 1959.<ref>Adams RD, Victor M, Mancall EL. Central pontine myelinolysis: a hitherto undescribed disease occurring in alcoholic and malnourished patients. AMA Arch Neurol Psychiatry. Feb 1959;81(2):154-72</ref>&nbsp;In at least 10% of patients with central pontine myelinolysis, demyelination also occurs in extrapontine regions, including the mid brain, thalamus, basal nuclei, and cerebellum. The exact mechanism that strips the myelin sheath is unknown.
 
Central pontine myelinolysis occurs most often as a complication of treatment of patients with profound, life-threatening hyponatremia (low sodium) and is 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.&nbsp;
 
In some cases, demyelination occurs outside the pons too; cases which include extrapontine demyelination are labelled "osmotic demyelination syndrome", "extra pontine myelinosis" or "osmotic myelinosis".<ref>Martin RJ. Central pontine and extrapontine myelinolysis: the osmotic demyelination syndromes. J Neurol Neurosurg Psychiatry. Sep 2004;75 Suppl 3:iii22-8</ref>
 
Microscopically the lesion shows degeneration and loss of oligodendrocytes with preservation of axons unless the lesion is very advanced.
 
 


== Clinical Presentation  ==
== Clinical Presentation  ==


add text here relating to the clinical presentation of the condition<br>  
The classic clinical presentation of central pontine myelinolysis is progressive spastic quadriparesis with pseudobulbar palsy.<ref>Laureno R, Karp BI. Myelinolysis after correction of hyponatremia. Ann Intern Med. Jan 1 1997;126(1):57-62</ref><br>  


== Diagnostic Procedures  ==
== Diagnostic Procedures  ==

Revision as of 09:33, 25 March 2014

Original Editor - Your name will be added here if you created the original content for this page.

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Clinically Relevant Anatomy
[edit | edit source]

add text here relating to clinically relevant anatomy of the condition

Mechanism of Injury / Pathological Process
[edit | edit source]

Central pontine myelinolysis is a concentrated, frequently symmetric, noninflammatory demyelination within the pons. The area involved is usually confined to the basis pontis, but occasionally involves the tegmentum too.

It is one of the demyelinating condions, and was first described by Adams et al in 1959.[1] In at least 10% of patients with central pontine myelinolysis, demyelination also occurs in extrapontine regions, including the mid brain, thalamus, basal nuclei, and cerebellum. The exact mechanism that strips the myelin sheath is unknown.

Central pontine myelinolysis occurs most often as a complication of treatment of patients with profound, life-threatening hyponatremia (low sodium) and is 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. 

In some cases, demyelination occurs outside the pons too; cases which include extrapontine demyelination are labelled "osmotic demyelination syndrome", "extra pontine myelinosis" or "osmotic myelinosis".[2]

Microscopically the lesion shows degeneration and loss of oligodendrocytes with preservation of axons unless the lesion is very advanced.


Clinical Presentation[edit | edit source]

The classic clinical presentation of central pontine myelinolysis is progressive spastic quadriparesis with pseudobulbar palsy.[3]

Diagnostic Procedures[edit | edit source]

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Outcome Measures[edit | edit source]

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Management / Interventions
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Differential Diagnosis
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Resources
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References[edit | edit source]

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  1. Adams RD, Victor M, Mancall EL. Central pontine myelinolysis: a hitherto undescribed disease occurring in alcoholic and malnourished patients. AMA Arch Neurol Psychiatry. Feb 1959;81(2):154-72
  2. Martin RJ. Central pontine and extrapontine myelinolysis: the osmotic demyelination syndromes. J Neurol Neurosurg Psychiatry. Sep 2004;75 Suppl 3:iii22-8
  3. Laureno R, Karp BI. Myelinolysis after correction of hyponatremia. Ann Intern Med. Jan 1 1997;126(1):57-62