[Myoclonic astatic seizures in a child with Sturge-Weber syndrome]. / Crises myoclonoastatiques chez un patient présentant un syndrome de Sturge-Weber.
Rev Neurol (Paris)
; 164(11): 953-6, 2008 Nov.
Article
em Fr
| MEDLINE
| ID: mdl-18808759
INTRODUCTION: Sturge-Weber syndrome is a neurocutaneous disease associating facial and pial angioma. Focal epilepsy is a common sign. In a few cases, generalized seizures have been reported. CASE REPORT: We report on a four-year-old girl with Sturge-Weber syndrome. The first focal seizures occurred at three years of age. She developed refractory status epilepticus. At discharge from the PICU, she was on a ketogenic diet and received three antiepileptic drugs. No seizures were observed for four months. The patient then developed several types of seizures: myoclonic seizures, focal clonic seizures, and sudden falls. We were unable to determine the etiology of the falls. Typical myoclonic astatic seizures were identified on video-electroencephalographic recordings. CONCLUSION: Seizures in Sturge-Weber syndrome are usually focal. Four patients with Sturge-Weber syndrome and myoclonoastatic seizures are reported in the literature. We discuss the pathophysiological mechanisms leading from a focal lesion to generalized myoclonoastatic seizures.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Síndrome de Sturge-Weber
/
Epilepsias Mioclônicas
Tipo de estudo:
Etiology_studies
/
Prognostic_studies
Limite:
Child, preschool
/
Female
/
Humans
Idioma:
Fr
Revista:
Rev Neurol (Paris)
Ano de publicação:
2008
Tipo de documento:
Article