Section 18
Metabolic & Nutritional Neurology
Wilson's Disease, Vitamin & Cofactor Therapies
7 AGENTS
Penicillamine
Cuprimine / DepenCopper chelator
- Forms
- Cap: 250mg | Tab: 250mg
- Dose
- Wilson's disease: 250mg QID initially; adjust to urinary copper excretion 0.5-1mg/day; max 2g/day (750mg/day in pregnancy)
- MOA
- Chelates copper, promotes urinary excretion
Trientine hydrochloride
SyprineCopper chelator
- Forms
- Cap: 250mg
- Dose
- Wilson's disease (penicillamine-intolerant): adults 750-1250mg/day in divided doses (max 2000mg/day); children 500-750mg/day divided
- MOA
- Chelates copper, promotes urinary excretion
Trientine tetrahydrochloride
CuvriorCopper chelator
- Forms
- Tab: 300mg (functionally scored)
- Dose
- Adults, stable de-coppered Wilson's disease tolerant of penicillamine: divided BID, max 3000mg/day; use label conversion table when switching; not mg-for-mg with other trientine products
- MOA
- Chelates copper, promotes urinary excretion
Zinc acetate
GalzinCopper absorption blocker
- Forms
- Cap: 25, 50mg elemental zinc
- Dose
- Wilson's disease: 50mg elemental zinc TID on empty stomach (adults); maintenance therapy per label
- MOA
- Induces intestinal metallothionein, blocking copper absorption
Thiamine
Vitamin B1Vitamin (cofactor)
- Forms
- Inj: 100mg/mL | Tab: 50, 100mg
- Dose
- Wernicke's encephalopathy: 500mg IV TID for 2-3 days, then 250mg IV daily for 3-5 days; give before glucose
- MOA
- Cofactor for carbohydrate metabolism enzymes
Cyanocobalamin
Vitamin B12Vitamin
- Forms
- Inj: 1000mcg/mL | Tab: 100-5000mcg
- Dose
- B12 deficiency with neurologic involvement: 1000mcg IM daily x1 week, weekly x4 weeks, then monthly; oral high-dose option for maintenance
- MOA
- Cofactor for methionine synthase and methylmalonyl-CoA mutase
Folic acid
FolateVitamin
- Forms
- Tab: 0.4, 0.8, 1mg
- Dose
- Women of childbearing age on antiepileptics: 0.4-4mg/day (higher doses per specialist/obstetric guidance)
- MOA
- Cofactor for nucleotide synthesis, ↓neural tube defect risk