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While this maldistribution promotes astrocyte swelling, alternative evidence identifies the initial pathology as arising from disrupted astrocytic potassium buffering (Eid and Lee 2013)

Hereditary causes include genetic defects in the metabolism of amino acids, cholesterol, and fatty acids, such as propionyl-CoA carboxylase deficiency (aka propionic acidemia) and medium chain acyl-CoA dehydrogenase deficiency (36)

M., Dietrich, B., and Hiersemenzel, R

Such inherited disorders lead to a buildup of organic acids, which are subsequently removed from the body via urinary excretion of acylcarnitine esters