Cerebral edema associated with betaine treatment in classical homocystinuria

A M Devlin; L Hajipour; A Gholkar; H Fernandes; V Ramesh; A A M Morris

doi:10.1016/j.jpeds.2003.12.041

Cerebral edema associated with betaine treatment in classical homocystinuria

J Pediatr. 2004 Apr;144(4):545-8. doi: 10.1016/j.jpeds.2003.12.041.

Authors

A M Devlin¹, L Hajipour, A Gholkar, H Fernandes, V Ramesh, A A M Morris

Affiliation

¹ Departments of Paediatric Neurology and Neurosurgery, Newcastle General Hospital, Newcastle-upon-Tyne, United Kingdom.

PMID: 15069409
DOI: 10.1016/j.jpeds.2003.12.041

Abstract

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.

Publication types

Case Reports

MeSH terms

Betaine / administration & dosage
Betaine / adverse effects*
Brain Edema / chemically induced*
Brain Edema / surgery
Child, Preschool
Craniotomy
Cystathionine beta-Synthase / deficiency
Decompression, Surgical
Gastrointestinal Agents / administration & dosage
Gastrointestinal Agents / adverse effects*
Homocystinuria / complications
Homocystinuria / drug therapy*
Humans
Male

Substances

Gastrointestinal Agents
Betaine
Cystathionine beta-Synthase