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From the Divisions of Pediatric Neurology (Drs. Jonas and Shields, and S. Yudovin), Neurosurgery (S. Nguyen and Dr. Mathern), and Neuropathology (Dr. Vinters), and Departments of Psychiatry & Biobehavioral Sciences (Drs. Asarnow and LoPresti), Linguistics (Dr. Curtiss), and Neurology (Drs. de Bode, Shields, and Vinters), The Mental Retardation Research Center (Drs. Asarnow, Shields, Vinters, and Mathern), and The Brain Research Institute (Drs. Shields, Vinters, and Mathern), David Geffen School of Medicine, University of California, Los Angeles; and Neurosurgery Department (Dr. Hu), 2nd Affiliated Hospital of Guangzhou Medical College, Guangzhou, China.
Address correspondence and reprint requests to Dr. Gary W. Mathern, Reed Neurologic Research Center, 710 Westwood Plaza, Rm 2123, Los Angeles, CA 90095-1769; e-mail: gmathern{at}ucla.edu
Objective: To compare hemispherectomy patients with different pathologic substrates for hospital course, seizure, developmental, language, and motor outcomes.
Methods: The authors compared hemispherectomy patients (n = 115) with hemimegalencephaly (HME; n = 16), hemispheric cortical dysplasia (hemi CD; n = 39), Rasmussen encephalitis (RE; n = 21), infarct/ischemia (n = 27), and other/miscellaneous (n = 12) for differences in operative management, postsurgery seizure control, and antiepilepsy drug (AED) usage. In addition, Vineland Adaptive Behavior Scale (VABS) developmental quotients (DQ), language, and motor assessments were performed pre- or postsurgery, or both.
Results: Surgically, HME patients had the greatest perioperative blood loss, and the longest surgery time. Fewer HME patients were seizure free or not taking AEDs 1 to 5 years postsurgery, but the differences between pathologic groups were not significant. Postsurgery, 66% of HME patients had little or no language and worse motor scores in the paretic limbs. By contrast, 40 to 50% of hemi CD children showed near normal language and motor assessments, similar to RE and infarct/ischemia cases. VABS DQ scores showed +5 points or more improvement postsurgery in 57% of patients, and hemi CD (+12.7) and HME (+9.1) children showed the most progress compared with RE (+4.6) and infarct/ischemia (–0.6) cases. Postsurgery VABS DQ scores correlated with seizure duration, seizure control, and presurgery DQ scores.
Conclusions: The pathologic substrate predicted pre- and postsurgery differences in outcomes, with hemimegalencephaly (but not hemispheric cortical dysplasia) patients doing worse in several domains. Furthermore, shorter seizure durations, seizure control, and greater presurgery developmental quotients predicted better postsurgery developmental quotients in all patients, irrespective of pathology.
Received June 10, 2003. Accepted in final form March 4, 2004.
Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the May 25 issue to find the title link for this article.
See also page 1664
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