Diagnosis and treatment of common central nervous system complications.
| Complications | Diagnosis | Treatment |
|---|---|---|
| Wernicke’s encephalopathy | Clinical | Intravenous 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 diphosphate | Oral 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 urine | Correction of metabolic acidosis |
| High an ion gap metabolic acidosis | Carbohydrate restriction | |
| Antibiotics43 | ||
| Hyperammonemic encephalopathy (HAE) | Measurement of plasma ammonia, zinc, glutamine and serum albumin level along with genetic testing for OTC enzyme deficiency | Dietary protein restriction Parenteral glucose and lipid infusion |
| Repletion of zinc, other micronutrients and amino acids. | ||
| Hemodialysis44 | ||
| Reversal of surgical procedure49 | ||
| Myelopathy secondary to vitamin B12 deficiency | Serum B12, methylmalonic and plasma homocysteine levels | Intramuscular 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 deficiency | Serum and urinary copper | Parenteral; intravenous 2 mg daily of elemental copper for 5 days |
| Serum ceruloplasmin activity | Oral; 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 deficiency | RBCs folate | Parenteral; 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