[Myoclonic astatic seizures in a child with Sturge-Weber syndrome].
BACKGROUND
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.


METHODS
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.


CONCLUSIONS
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.
Petit
F
F
Service de neurologie pédiatrique, hôpital Roger-Salengro, CHRU, boulevard Leclercq, 59037 Lille cedex , France.
Auvin
S
S
Lamblin
M-D
MD
Vallée
L
L
D000058:Accidental Falls / Q000706:statistics & numerical data; D002675:Child, Preschool; D004831:Epilepsies, Myoclonic / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D009069:Movement Disorders / Q000209:etiology; D010988:Play and Playthings; D012640:Seizures / Q000209:etiology; D013341:Sturge-Weber Syndrome / Q000175:diagnosis* / Q000503:physiopathology