Central pontine myelinolysis associated with acquired folate depletion.
After long-standing malnutrition a 15-month-old boy with signs of kwashiorkor was admitted in a moribund state with serious hyponatraemic dehydration, hypothermia, somnolence, and signs of a pontine disconnection syndrome. Folic acid levels were below the detection level in the presence of normal cobalamin levels. MRI of the brain showed global volume loss and signal abnormalities on the T2-weighted images suggestive for central pontine myelinolysis (CPM). Brainstem acoustic evoked responses have remained normal. The serious metabolic and nutritional derangements required substitution of folic acid, vitamins and trace elements as well as slow correction of hyponatraemic dehydration with return of the sodium level over a period of four days. This therapeutic regimen resulted in complete neurological recovery. Follow-up MRI documented normalisation of the initial pathologic findings. The hypothesis was put forward linking the pathogenesis of CPM with the combination of folate depletion and superimposed hyponatraemic dehydration. The previously acquired folate depletion could affect normal appositional function of myelin basic protein molecules due to insufficient methylation of arginine in position 107. The subsequent development of intramyelinic edema and CPM will then be triggered by the superimposed hyponatraemic dehydration. The verification of this hypothesis requires further investigations.
Ramaekers
V T
VT
Division of Paediatric Neurology Kinderklinik, RWTH Aachen, Germany.
Reul
J
J
Kusenbach
G
G
Thron
A
A
Heimann
G
G
D003681:Dehydration / Q000150:complications*; D005494:Folic Acid Deficiency / Q000150:complications*; D006801:Humans; D007010:Hyponatremia / Q000150:complications*; D007223:Infant; D007228:Infant Nutrition Disorders / Q000150:complications*; D007732:Kwashiorkor / Q000150:complications; D008297:Male; D017590:Myelinolysis, Central Pontine / Q000175:diagnosis / Q000209:etiology*