Cerebral edema associated with betaine treatment in classical homocystinuria.
A child with cystathionine beta-synthase deficiency developed cerebral edema 4 to 6 weeks after starting betaine therapy. There was no evidence of intracranial thrombosis, but there was widespread edema of the white matter. He recovered fully after emergency decompressive craniotomy and withdrawal of betaine.
Devlin
A M
AM
Departments of Paediatric Neurology and Neurosurgery, Newcastle General Hospital, Newcastle-upon-Tyne, United Kingdom.
Hajipour
L
L
Gholkar
A
A
Fernandes
H
H
Ramesh
V
V
Morris
A A M
AA
D001622:Betaine / Q000008:administration & dosage / Q000009:adverse effects*; D001929:Brain Edema / Q000139:chemically induced* / Q000601:surgery; D002675:Child, Preschool; D003399:Craniotomy; D003541:Cystathionine beta-Synthase / Q000172:deficiency; D019299:Decompression, Surgical; D005765:Gastrointestinal Agents / Q000008:administration & dosage / Q000009:adverse effects*; D006712:Homocystinuria / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D008297:Male
D005765:Gastrointestinal Agents; D001622:Betaine; D003541:Cystathionine beta-Synthase