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From the Pediatric Epilepsy Center (Drs. Kossoff and Freeman), Departments of Neurology and Pediatrics, The Johns Hopkins Medical Institutions, Baltimore, MD; and the Sturge–Weber Foundation (C. Buck), Mt. Freedom, NJ.
Address correspondence and reprint requests to Dr. Eric H. Kossoff, Department of Pediatric Neurology, The Johns Hopkins Hospital, 600 N. Wolfe St., Jefferson 128, Baltimore, MD 21287-1000; e-mail: ekossoff{at}jhmi.edu
Background: Epilepsy affects 80% of patients with Sturge–Weber syndrome; the majority of seizures begin before the age of 1. When seizures are intractable to medications and unihemispheric, hemispherectomy is often advised.
Objective: To examine the natural history of patients who underwent hemispherectomy and identify the outcomes in terms of seizure reduction, cognition, and motor deficits.
Methods: A questionnaire was mailed to the parents of patients identified by the Sturge–Weber Foundation as having had a hemispherectomy between 1979 and 2001. Forty-six percent (32/70) of the parents responded.
Results: The mean age at onset of seizures was 4 months, and the median age at surgery was 1.2 years. Children had failed to respond to 3.7 anticonvulsants prior to surgery and averaged 387 seizures/month. Forty-seven percent had complications (e.g., hemorrhage and hypertension) in the perioperative period; however, 81% are currently seizure-free, with 53% off anticonvulsants. Hemispherectomy type (anatomic versus functional versus hemidecortication) did not influence outcome. Age at onset of seizures did not predict seizure freedom; however, an older age at hemispherectomy was positively correlated. Postoperative hemiparesis was not more severe than before surgery. Cognitive outcome was not related to the age at operation, side of operation, or seizure freedom.
Conclusions: Children undergoing hemispherectomy presented at a young age and had frequent seizures for approximately 1 year but are now mostly seizure-free. Age at surgery did not have an adverse effect on either seizure or cognitive outcomes.
This article has been cited by other articles:
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  V. Udani, S. Pujar, P. Munot, S. Maheshwari, and N. Mehta Natural History and Magnetic Resonance Imaging Follow-up in 9 Sturge-Weber Syndrome Patients and Clinical Correlation J Child Neurol, April 1, 2007; 22(4): 479 - 483. [Abstract] [PDF]
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T. M. Kelley, L. A. Hatfield, D. D. M. Lin, and A. M. Comi Quantitative Analysis of Cerebral Cortical Atrophy and Correlation With Clinical Severity in Unilateral Sturge-Weber Syndrome J Child Neurol, November 1, 2005; 20(11): 867 - 870. [Abstract] [PDF]
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L. A. Hatfield and E. H. Kossoff Comorbidity of Epilepsy and Headache in Patients With Sturge-Weber Syndrome J Child Neurol, August 1, 2005; 20(8): 678 - 682. [Abstract] [PDF]
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 R. Jonas, S. Nguyen, B. Hu, R. F. Asarnow, C. LoPresti, S. Curtiss, S. de Bode, S. Yudovin, W. D. Shields, H. V. Vinters, et al. Cerebral hemispherectomy: Hospital course, seizure, developmental, language, and motor outcomes Neurology, May 25, 2004; 62(10): 1712 - 1721. [Abstract] [Full Text] [PDF]
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E. H. Kossoff, E. P.G. Vining, D. J. Pillas, P. L. Pyzik, A. M. Avellino, B. S. Carson, and J. M. Freeman Hemispherectomy for intractable unihemispheric epilepsy Etiology vs outcome Neurology, October 14, 2003; 61(7): 887 - 890. [Abstract] [Full Text] [PDF]
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A. M. Comi Topical Review: Pathophysiology of Sturge-Weber Syndrome J Child Neurol, August 1, 2003; 18(8): 509 - 516. [Abstract] [PDF]
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