Biomaterial Database

Epilepsy surgery in bilateral Sturge-Weber syndrome. 2002

Ingrid E B Tuxhorn, and Heinz W Pannek

Pediatric Epilepsy Surgery Unit, Klinik Mara, Epilepsy Center Bethel, Bielefeld, Germany.

Two infants with severe drug refractory focal epilepsy caused by Sturge-Weber syndrome and extensive cerebral leptomeningeal angiomatosis were referred for preoperative video-electroencephalographic evaluation. Brain imaging with computed tomography and gadolinium-enhanced magnetic resonance imaging demonstrated bilateral disease in both children with a predominance of involvement of one hemisphere. Clinical examination and neurophysiology with ictal video recording demonstrated epileptogenesis from one hemisphere. Successful surgical treatment with functional hemispherectomy was followed by good long-term seizure control in both patients. The dramatic seizure control was accompanied by markedly improved quality of life for the family and children. These cases indicate that the spectrum of children that may benefit from epilepsy surgery should not be viewed too restrictively, and subsets of children with localization related epilepsy caused by extensive lesions may be resective surgical candidates with a good seizure outcome prognosis.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D004828	Epilepsies, Partial	Conditions characterized by recurrent paroxysmal neuronal discharges which arise from a focal region of the brain. Partial seizures are divided into simple and complex, depending on whether consciousness is unaltered (simple partial seizure) or disturbed (complex partial seizure). Both types may feature a wide variety of motor, sensory, and autonomic symptoms. Partial seizures may be classified by associated clinical features or anatomic location of the seizure focus. A secondary generalized seizure refers to a partial seizure that spreads to involve the brain diffusely. (From Adams et al., Principles of Neurology, 6th ed, pp317)	Abdominal Epilepsy,Digestive Epilepsy,Epilepsy, Focal,Epilepsy, Simple Partial,Focal Seizure Disorder,Gelastic Epilepsy,Partial Epilepsy,Partial Seizure Disorder,Seizure Disorder, Partial,Simple Partial Seizures,Amygdalo-Hippocampal Epilepsy,Benign Focal Epilepsy, Childhood,Benign Occipital Epilepsy,Benign Occipital Epilepsy, Childhood,Childhood Benign Focal Epilepsy,Childhood Benign Occipital Epilepsy,Epilepsy, Benign Occipital,Epilepsy, Localization-Related,Epilepsy, Partial,Occipital Lobe Epilepsy,Panayiotopoulos Syndrome,Partial Seizures, Simple, Consciousness Preserved,Rhinencephalic Epilepsy,Seizure Disorder, Focal,Subclinical Seizure,Uncinate Seizures,Abdominal Epilepsies,Amygdalo-Hippocampal Epilepsies,Benign Occipital Epilepsies,Digestive Epilepsies,Disorders, Focal Seizure,Disorders, Partial Seizure,Epilepsies, Abdominal,Epilepsies, Amygdalo-Hippocampal,Epilepsies, Benign Occipital,Epilepsies, Digestive,Epilepsies, Focal,Epilepsies, Gelastic,Epilepsies, Localization-Related,Epilepsies, Occipital Lobe,Epilepsies, Rhinencephalic,Epilepsies, Simple Partial,Epilepsy, Abdominal,Focal Epilepsies,Focal Epilepsy,Focal Seizure Disorders,Gelastic Epilepsies,Lobe Epilepsy, Occipital,Localization-Related Epilepsies,Localization-Related Epilepsy,Occipital Epilepsies, Benign,Occipital Epilepsy, Benign,Occipital Lobe Epilepsies,Partial Epilepsies,Partial Epilepsies, Simple,Partial Seizure Disorders,Partial Seizures, Simple,Rhinencephalic Epilepsies,Seizure Disorders, Focal,Seizure Disorders, Partial,Seizure, Subclinical,Seizure, Uncinate,Seizures, Simple Partial,Seizures, Subclinical,Seizures, Uncinate,Simple Partial Epilepsies,Subclinical Seizures,Uncinate Seizure
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013341	Sturge-Weber Syndrome	A non-inherited congenital condition with vascular and neurological abnormalities. It is characterized by facial vascular nevi (PORT-WINE STAIN), and capillary angiomatosis of intracranial membranes (MENINGES; CHOROID). Neurological features include EPILEPSY; cognitive deficits; GLAUCOMA; and visual defects.	Neuroretinoangiomatosis,Phakomatosis, Sturge-Weber,Angiomatosis Oculoorbital-Thalamic Syndrome,Encephalofacial Hemangiomatosis Syndrome,Meningo-Oculo-Facial Angiomatosis,Meningofacial Angiomatosis-Cerebral Calcification Syndrome,Parkes Weber Syndrome,Parkes-Weber Syndrome,Sturge Disease,Sturge Syndrome,Sturge's Syndrome,Sturge-Kalischer-Weber Syndrome,Sturge-Weber-Dimitri Syndrome,Sturge-Weber-Krabbe Syndrome,Angiomatosis, Meningo-Oculo-Facial,Hemangiomatosis Syndrome, Encephalofacial,Meningo Oculo Facial Angiomatosis,Phakomatosis, Sturge Weber,Sturge Kalischer Weber Syndrome,Sturge Weber Dimitri Syndrome,Sturge Weber Krabbe Syndrome,Sturge Weber Syndrome,Sturge-Weber Phakomatosis,Syndrome, Encephalofacial Hemangiomatosis,Syndrome, Parkes Weber,Syndrome, Parkes-Weber,Syndrome, Sturge,Syndrome, Sturge's,Syndrome, Sturge-Kalischer-Weber,Syndrome, Sturge-Weber,Syndrome, Sturge-Weber-Dimitri,Syndrome, Sturge-Weber-Krabbe