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Central pontine myelinolysis
Classification and external resources

Pons labeled at bottom left
ICD-10 G37.2
DiseasesDB 2198
MedlinePlus 000775
eMedicine neuro/50
MeSH D017590

Central pontine myelinolysis is a neurologic disease caused by severe damage of the myelin sheath of nerve cells in the brainstem, more precisely in the area termed the pons.

It can also occur outside the pons.[1] The term "osmotic demyelinization syndrome" is similar to "central pontine myelinolysis", but also includes areas outside the pons.[2]

Contents

Causes

The most common cause is the rapid correction of low blood sodium levels (hyponatremia).[3]

It has been observed following hematopoietic stem cell transplantation.[4]

It is frequently associated with alcoholism. When alcoholics are treated for hyponatremia, an overly rapid rate of correction can lead to CPM.[5]

It can be associated with Hyperemesis gravidarum.

Diagnosis

It can be difficult to identify using conventional imaging techniques.[6]

Imaging by MRI demonstrates an area of high signal return on T2 weighted images.

Symptoms

Frequently observed symptoms in this disorder are sudden para- or quadraparesis, dysphagia, dysarthria, diplopia and loss of consciousness. The patient may experience locked-in syndrome where cognitive function is intact, but all muscles are paralyzed with the exception of eye blinking.

Osmotic demyelination syndrome occurs with rapid correction of hyponatremia. It is characterized by acute paralysis, dysarthria, dysphagia and other neurological symptoms.

Treatment

Treatment is supportive only.

Correct hyponatremia at a rate not in excess of 10mmol/L/24hr; diligently avoid hypernatremia. Details concerning the etiology and correction of electrolyte disorders are discussed extensively in general medicine texts. Alcoholic patients should receive vitamin supplementation. Formally evaluate their nutritional status. [7][8]

Additional images

References

  1. ^ Gocht A, Colmant HJ (1987). "Central pontine and extrapontine myelinolysis: a report of 58 cases". Clin. Neuropathol. 6 (6): 262–70. PMID 3322623.  
  2. ^ Lampl C, Yazdi K (2002). "Central pontine myelinolysis". Eur. Neurol. 47 (1): 3–10. PMID 11803185. http://content.karger.com/produktedb/produkte.asp?typ=fulltext&file=ene47003.  
  3. ^ Bernsen HJ, Prick MJ (September 1999). "Improvement of central pontine myelinolysis as demonstrated by repeated magnetic resonance imaging in a patient without evidence of hyponatremia". Acta Neurol Belg 99 (3): 189–93. PMID 10544728.  
  4. ^ Lim KH, Kim S, Lee YS, et al. (April 2008). "Central pontine myelinolysis in a patient with acute lymphoblastic leukemia after hematopoietic stem cell transplantation: a case report". J. Korean Med. Sci. 23 (2): 324–7. doi:10.3346/jkms.2008.23.2.324. PMID 18437020. PMC 2526450. http://jkms.org/contents/jkms.php?pubyear=2008&vol=23&fpage=324.  
  5. ^ Menger H, Jörg J (August 1999). "Outcome of central pontine and extrapontine myelinolysis (n = 44)". J. Neurol. 246 (8): 700–5. PMID 10460448. http://link.springer.de/link/service/journals/00415/bibs/9246008/92460700.htm.  
  6. ^ Ruzek KA, Campeau NG, Miller GM (February 2004). "Early diagnosis of central pontine myelinolysis with diffusion-weighted imaging". AJNR Am J Neuroradiol 25 (2): 210–3. PMID 14970019. http://www.ajnr.org/cgi/pmidlookup?view=long&pmid=14970019.  
  7. ^ Kleinschmidt-DeMasters BK, Norenberg MD. Rapid correction of hyponatremia causes demyelination: relation to central pontine myelinolysis. Science. 1981;211(4486):1068-70. PMID 7466381
  8. ^ Laureno R. Experimental pontine and extrapontine myelinolysis. Trans Am Neurol Assoc. 1980;105:354-8. PMID 7348981

External links

  • MedPix Images of Osmotic Myelinolysis
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