Betaine anhydrous
Cystadane · ATC code A16AA06
- Key facts
- Active substance
- Betaine anhydrous
- ATC code
- A16AA06
- Authorisation
- centrally authorised across the EU (EMA)
- Dosage forms
- Cystadane 1 g oral powder
- Marketing authorisation holder
- Recordati Rare Diseases
- Information as of
- 2026-10-27
Key facts
Betaine anhydrous is a methyl-group donor that lowers homocysteine. It is used as adjunctive treatment of homocystinuria.
What it is used for
- Adjunctive treatment of homocystinuria involving deficiencies or defects in cystathionine beta-synthase (CBS), 5,10-methylene-tetrahydrofolate reductase (MTHFR) or cobalamin cofactor metabolism (cbl)
- Used as a supplement to vitamin B6, vitamin B12, folate and a specific diet
Dosing
- Children and adults: recommended total daily dose 100 mg/kg/day in 2 doses
- Titrate individually according to plasma homocysteine and methionine; some patients needed doses above 200 mg/kg/day
- Caution when up-titrating in CBS deficiency (risk of hypermethioninaemia)
- No dose adjustment needed in renal insufficiency or non-alcoholic hepatic steatosis
- Mix the powder with water, juice, milk, formula or food and take immediately; measuring spoons give 100 mg, 150 mg and 1 g
Contraindications
- Hypersensitivity to the active substance or any excipient
Important warnings
- Uncommon severe cerebral oedema associated with hypermethioninaemia in CBS deficiency; keep plasma methionine below 1000 µM
- Measure methionine at start and about annually or biannually; monitor more often above 700 µmol/L
- If signs of cerebral oedema appear (morning headache with vomiting, visual changes), interrupt treatment; discontinue indefinitely if they recur
- Leave 30 minutes between betaine and amino acid mixtures or medicines containing vigabatrin or GABA analogues
Side effects
- Very common: blood methionine increased
- Uncommon: brain oedema, anorexia, agitation, irritability
- Uncommon: diarrhoea, glossitis, nausea, stomach discomfort, vomiting
- Uncommon: hair loss, hives, abnormal skin odour, urinary incontinence
Interactions
- No interaction studies; in vitro data suggest possible interaction with amino acid mixtures and with vigabatrin and GABA analogues
Pregnancy and breastfeeding
- Limited data show no adverse effect on pregnancy or the foetus/newborn; should not be used during pregnancy unless clearly necessary
Summary of the official product information, written with AI (Claude, Anthropic) on 2026-10-28. Every number was checked automatically against the original text. Not reviewed by a physician – the full official text is authoritative.
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For patients
Package leaflet, verbatim
Authorisation and brand names by country
Medicines with exactly this active-substance composition, as listed in the national registers – alphabetical and without ranking. Whether a product is currently available is not shown here.
EU – centrally authorised (all member states)
Source: European Medicines Agency (EMA), product information
Brand names in 5 more European countries
- France
- Citrate De Betaine Cristers, Citrate De Betaïne Citron Sans Sucre Édulcoré À La Saccharine Sodique Upsa, Citrate De Betaïne Menthe Sans Sucre Édulcoré À La Saccharine Sodique Upsa
- Greece
- Alkacitrat
- Iceland
- Amversio, Cystadane
- Liechtenstein
- Amversio, Cystadane
- Norway
- Amversio, Cystadane
EMA – Article 57 product data (EU/EEA), 2026-10-28
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Another question about this substance?
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Sources
- European Medicines Agency. Cystadane – EPAR, Product Information (OF THE TEXT Detailed information on this medicinal product i). 2026-10-27. https://www.ema.europa.eu/en/documents/product-information/cystadane-epar-product-information_en.pdf
Editorial: Medelix editorial team · last updated 2026-10-27 · How these pages are made · Report an error
Source: European Medicines Agency (EMA), product information. Not advertising, not a treatment recommendation. For questions about your own treatment, ask your doctor or pharmacist.