KISEP Reviews J Korean Epilep Soc 42:94-100, 2000 Therapeutic Strategies in Malformations of Cortical DevelopmentOverview 고태성 Tae Sung Ko, M.D. 94 서 Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea 론 J Korean Epilep Soc Volume 4 December, 2000
고태성 Table 1. Classification of malformations of cortical development I. Malformations due to abnormal neuronal and glial proflieration A. Generalized 1. Proliferation-microlissencephaly B. Focal or multifocal 1. Abnormal proliferation abnormal cell types a. Nonneoplastic 1 Tuberous sclerosis complex 2 Focal cortical dysplasia with balloon cell 3 Hemimegalencephaly b. Neoplastic 1 DNET 2 Ganglioglioma 3 Gangliocytoma II. Malformations due to abnormal neuronal migration A. Generalized 1. Classical type 1 lissencephaly agyria-pachygyria spectrum 2. Cobblestone type 2 lissencephaly 3. Lissencephaly-not otherwise classified 4. Heterotopia-subependymal SE, subcortical SC B. Focal or multifocal 1. Focal agyria/pachygyria partial lissencephaly 2. Focal or multifocal heterotopia-se, SC 3. Focal or multifocal heterotopia with organizational abnormality of the cortex-se, SC 4. Excessive single ectopic white matter neurons III. Malformations due to abnormal cortical organization A. Generalized 1. Polymicrogyria PMG B. Focal or multifocal 1. Polymicrogyria/schizencephaly 2. Focal or mutifocal cortical dysplasia without balloon cells 3. Microdysgenesis IV. Malformations of cortical development, not otherwise classified Adapted from Barkovich AJ 2000. 2) 1. Epilepsy 임상증상별치료 1) Drugs J Korean Epilep Soc Volume 4 December, 2000 95
대뇌피질발달기형의치료 2) Surgery 96 J Korean Epilep Soc Volume 4 December, 2000
고태성 3) Alternative therapy 2. Genetic counseling about syndromes J Korean Epilep Soc Volume 4 December, 2000 97
대뇌피질발달기형의치료 Table 2. Syndromes associated with malformations of cortical development 1. Lissencephalic Miller-Dieker syndrome Walker-Warburg syndrome Fukuyama congenital muscular dystrophy Myotonic dystrophy 2. Nonlissencephalic Neurocutaneous Tuberous sclerosis complex Neurofibromatosis-1 Hypomelanosis of Ito Incontinentia pigmenti Epidermal nevus syndrome Metabolic Zellweger syndrome Menkes syndrome Neonatal adrenoleukodystrophy Other Aicardi s syndrome Angelman s syndrome Smith-Lemli-Opitz syndrome Waardenburg s syndrome Noonan s syndrome Ehlers-Danlos syndrome Adapted from Whiting S and Duchowny M 1999. 27) 3. Developmental delay 4. Focal neurologic deficit 5. Mental retardation 6. Other associated anomalies 7. General supportive cares 98 J Korean Epilep Soc Volume 4 December, 2000
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대뇌피질발달기형의치료 ciated with band heterotopia. Epilepsia 1993;34:79-83. 21) Kuzniecky R, Andermann F, Guerrini R, CBPS Multicenter Collaborative Study. The congenital bilateral perisylvian syndrome, in Guerrini R, Andermann F, Canapicchi R, Roger J, Zifkin BG, Pfanner P (eds): Dysplasias of Cerebral Cortex and Epilepsy. Philadelphia, Lippincott-Raven, 1996:271-7. 22) Kim Y, Kim H, Palmini A, Woo Y, Lee M. Resective surgery for intractable epilepsy in patients with congenital bilateral perisylvian syndrome. Proceedings of International Bethel-Cleve- land Clinic Epilepsy Symposium [Abstract], 1995:95. 23) Guerreiro MM, Andermann F, Andermann E, et al. Surgical treatment of epilepsy in tuberous sclerosis: strategies and results in 18 patients. Neurology 1998;51:1263-9. 24) Vossler DG, Lee JK, Ko TS. Treatment of seizures in subcortical laminar heterotopia with corpus callosotomy and lamotrigine. J Child Neurol 1999;14: 282-8. 25) Kinsman SL, Vining EPG, Quaskey SA, Mellits D, Freeman JM. Efficacy of the ketogenic diet for intractable seizure disorder: Review of 58 cases. Epilepsia 1992;33:1132-6. 26) McLachlan RS. Vagus nerve stimulation for intractable epilepsy: A review. J Clin Neurophysiol 1997;14:358-68. 27) Whiting S, Duchowny M. Clinical spectrum of cortical dysplasia in childhood: Diagnosis and Treatment Issues. J Child Neurol 1999;14:759-71. 28) Dobyns W, Andermann E, Andermann F, et al. X-linked malformations of neuronal mirgation. Neurology 1996; 47:331-9. 100 J Korean Epilep Soc Volume 4 December, 2000