Metopic suture craniosynostosis: sodium valproate teratogenic effect. Case report

Made available in DSpace on 2019-09-12T16:56:54Z (GMT). No. of bitstreams: 0 Previous issue date: 2001Objective: the aim of this report is to warn that sodium valproate used during pregnancy can produce craniosynostosis in the newborn, particularly trigonocephaly. Method: we describe a case of trigonocephaly in a six month-old girl, daughter of a young non-smoker couple, whose mother had used phenobarbital 100 mg daily and sodium valproate 500 mg twice daily during the whole pregnancy. We also review current literature about this topic. Result: bone sclerosis over the metopic suture was confirmed during surgery. Bibliographical review yields previous reports on valproate teratogenicity, mainly determining metopic suture craniosynostosis. Conclusion: sodium valproate used during pregnancy can produce craniosynostosis by teratogenic effect, specially trigonocephaly (premature fusion of metopic suture)., Fac Med; Univ Sao Paulo, Area Concentracao Neurol, BR-05508 Sao Paulo, Brazil; Hosp Santa Isabel Cli

Cranial MRI in ataxia-telangiectasia.

We examined five males with laboratory-confirmed ataxia-telangiectasia (AT), aged 9-28 years, several times by MRI (9 examinations: 5 at 0.15 T, 3 at 0.5 T, 1 at 1.5 T). Intermediate, T1-, T2- and T2*-weighted spin-echo and gradient-echo sequences were performed. All patients showed vermian atrophy, enlarged fourth ventricle and cisterna magna; four showed cerebellar hemisphere atrophy; two enlarged infracerebellar subarachnoid spaces and four patients had sinusitis. No focal areas of abnormal signal were seen in the brain, diffuse high signal was found in the central cerebral white matter of the oldest patient. AT is an important human model of inherited cancer susceptibility and multisystem ageing; as in xeroderma pigmentosum and other "breakage syndromes", ionising radiation should be avoided. When imaging is necessary, MRI should be preferred to CT in patients known or suspected to have AT and those with undefined paediatric ataxias of nontraumatic origin. If atrophy of only the cerebellum, especially the vermis, is noted, laboratory research should be performed to confirm the diagnosis of AT