Description

Myelinolysis is a cerebral disorder that features myelin degeneration following correction of hyponatremia. It is distinct from demyelination and may affect neuronal elements to a lesser degree. Most cases affect the pons but other areas of the brain may be affected. Prevention and early recognition can help reduce development of serious complications.


 

Features:

(1) history of hyponatremia, often with rapid sodium replacement

(2) clinical findings compatible with the site of involvement

(3) onset several days after correction of the hyponatremia

(4) MRI shows an increased signal in the area of involvement, usually symmetrical (CT scans are often negative)

 

Sites of involvement:

(1) pons: central pontine myelinolysis

(2) medulla oblongata, midbrain, pontine tegmentum: extrapontine myelinolysis

 

Symptoms with pontine involvement:

(1) mutism and dysarthria

(2) lethargy and affective change

(3) spastic quadriparesis

(4) pseudobulbar palsy

 

Symptoms in extrapontine myelinolysis:

(1) pupillary and oculomotor abnormalities (midbrain)

(2) depressed level of consciousness and cranial nerve signs (pontine tegmentum)

(3) ataxia

(4) movement disorders (parkisonian, dystonia, other)

 

Affected patients may die, suffer late sequelae, or make a complete recovery.

 

Differential diagnosis:

(1) psychiatric illness

(2) encephalitis associated with hyponatremia

(3) alcoholic withdrawal

 


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