title: Radiologic and neurophysiologic aspects of stroke-like episodes in children with congenital disorder of glycosylation type Ia.
abstract: In an effort to shed light on the mechanism of hemiparetic stroke-like events experienced by patients with congenital disorder of glycosylation type Ia, we evaluated 3 children with this disorder by brain imaging studies and continuous electroencephalogram monitoring during such events. No evidence of ischemia or infarction was revealed on imaging studies and electrographic seizures or intermittent epileptiform activity was demonstrated on electrographic recordings. All 3 patients showed clinical and electrographic improvement after administration of antiepileptic medication. Epileptic phenomena can complicate the stroke-like events of patients with congenital disorder of glycosylation type Ia, and the cause of the hemiparesis may indeed be an active epileptic inhibitory process. As such, electroencephalogram monitoring is warranted, and treatment with anticonvulsant agents is indicated.
authors.affiliation: Cincinnati Children's Hospital Medical Center, Division of Neurology, 3333 Burnet Ave, MLC11006, Cincinnati, OH 45229, USA. argidino@yahoo.com
authors.forename: Argirios
authors.initials: A
authors.lastname: Dinopoulos
authors.forename: Ismail
authors.initials: I
authors.lastname: Mohamed
authors.forename: Blaise
authors.initials: B
authors.lastname: Jones
authors.forename: Sanjai
authors.initials: S
authors.lastname: Rao
authors.forename: David
authors.initials: D
authors.lastname: Franz
authors.forename: Ton
authors.initials: T
authors.lastname: deGrauw
mesh_terms: D000293:Adolescent; D000927:Anticonvulsants / Q000627:therapeutic use; D001921:Brain / Q000000981:diagnostic imaging; D002648:Child; D002675:Child, Preschool; D018981:Congenital Disorders of Glycosylation / Q000150:complications*; D004569:Electroencephalography; D005260:Female; D006031:Glycosylation; D006801:Humans; D011859:Radiography; D020521:Stroke / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology*; D016896:Treatment Outcome
chemical_list: D000927:Anticonvulsants