Table 3

Diagnosis and treatment of common central nervous system complications.

Wernicke’s encephalopathyClinicalIntravenous thiamine 500 mg 3 times a day for 2-3 days followed by 250 mg daily for 3-5 days.32,36,38
Erythrocyte transketolase activation assay or RBC thiamine diphosphateOral maintenance dose of 50-100 mg daily for long term
Characteristic paraventricular signals on MRI
Encephalopathy associated with D-lactic acidosis:Elevated D- lactate levels in serum and urineCorrection of metabolic acidosis
High an ion gap metabolic acidosisCarbohydrate restriction
Hyperammonemic encephalopathy (HAE)Measurement of plasma ammonia, zinc, glutamine and serum albumin level along with genetic testing for OTC enzyme deficiencyDietary protein restriction Parenteral glucose and lipid infusion
Repletion of zinc, other micronutrients and amino acids.
Reversal of surgical procedure49
Myelopathy secondary to vitamin B12 deficiencySerum B12, methylmalonic and plasma homocysteine levelsIntramuscular 1000 mic gram daily for 7 days followed by once weekly and then monthly32
Abnormal signals in dorsal column and corticospinal tract on MRI
Myelopathy secondary to copper deficiencySerum and urinary copperParenteral; intravenous 2 mg daily of elemental copper for 5 days
Serum ceruloplasmin activityOral; 8 mg per day of elemental copper for 1st week, 6 mg for 2nd week, 4 mg for 3rd week and 2 mg thereafter32
MRI findings similar to B12 deficiency
Myelopathy secondary to folate deficiencyRBCs folateParenteral; 1-5 mg daily32
Oral; 1 mg 3 times a day then maintenance dose of 1 mg per day
  • RBC - red blood cells, MRI - Magnetic resonance imaging, HAE - Hyperammonemic encephalopathy, OTC - Ornithine transcarbamylase