Central pontine myelinolysis

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

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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.

Causes

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

Some scholars postulate that the real cause might 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 assumption. [1]

Diagnosis

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, double vision 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.

Treatment

To avoid myelinolysis, the correction of hyponatremia should not exceed 1 mEq/L per hour. [2][3]

References

  1. 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
  2. Kleinschmidt-DeMasters BK, Norenberg MD. Rapid correction of hyponatremia causes demyelination: relation to central pontine myelinolysis. Science. 1981;211(4486):1068-70. PMID 7466381
  3. Laureno R. Experimental pontine and extrapontine myelinolysis. Trans Am Neurol Assoc. 1980;105:354-8. PMID 7348981
  • Gocht A , Colmant HJ. Central pontine and extrapontine myelinolysis: a report of 58 cases. Clin Neuropath. 1987;6:262–70. PMID 3322623
  • Menger H , Jorg J. Outcome of central pontine and extrapontine myelinolysis (n=44). J Neurol. 1999;246:700–5. PMID 10460448

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