KISEP Original Articles J Korean Epilep Soc 6(2):104-109, 2002 중증영아형근간대성간질의임상적고찰 * Clinical Review of Severe Myoclonic Epilepsy in Infancy 강훈철 Oyungerel Dambajamts 김흥동 Hoon Chul Kang, M.D., Oyungerel Dambajamts, M.D. and Heung Dong Kim, M.D., Ph.D. 104 ABSTRACT Purpose Purpose Method Method Results Conclusion J Korean Epilep Soc 6104-109, 2002 서 - Epilepsy Center, Department of Pediatrics, Inje University Sang-gye Paik Hospital, Seoul, Korea 교신저자 론 대상및방법 J Korean Epilep Soc Volume 6 December, 2002
강훈철 Oyungerel Dambajamts 김흥동 - - 결 Table 1. Patients data MaleFemale 과 108 Age mo. 13153 meansd, 75.333.3 Past historyno. of patients PrematurityLBW 1 Family history No. of patients % Epilepsy 3 16.7 Febrile convulsion 2 11.1 Total 5 27.8 Related disease No. of patients Cytochrome IV deficiency 1 Duration of follow up mo. 10151 meansd, 69.932.6 LBWlow birth weight - Table 2. Characteristics of seizures Onset age mo. 210 meansd, 5.12.8 First seizureno. of patients % Focal clonic Focal with secondary generalized clonic Generalized clonic Febrile Afebrile Subsequent seizure No. of patients % Atonic Myoclonic Atypical absence Complex partial Partial with 2ndary generalized Generalized tonic Generalized tonic clonic Photosensitive seizure No. of patients % 09 050.0 04 022.2 05 027.8 07 038.9 11 061.1 01 005.6 18 100.0 06 033.3 16 088.9 06 033.3 01 005.6 10 055.6 04 022.2 Table 3. Characteristics of febrile and myoclonic seizures Febrile seizure Onset mo. 211 meansd, 07.803.0 With first seizure No. of patients % 07 038.9 PersistencyNo. of patients % 18 100.0 Status epilepticus No. of patients % 09 050.0 Myoclonic seizure Onset mo. 748 meansd, 26.512.1 PersistencyNo. of patients % 14 077.8 J Korean Epilep Soc Volume 6 December, 2002 105
중증영아형근간대성간질의임상적고찰 Table 4. EEG features Initial findingno. of patients Normal 18 Subsequent abnormalities *n14 Normal 03 Abnormal 11 Onset age mo. 888 meansd, 33.020.9 Duration from seizure onset mo. 686 meansd, 28.221.0 Patients who could be followed from seizure onset Table 5. Interictal EEG features 106 EEG features Background abnormality n11, 100% Slow and disorganized Polymorphic delta waves Asymmetric slowings Epileptiform discharges n9, 81.8% Multifocal sharp or spikes Focal sharp or spikes Generalized paroxysmal fast activities Generalized sharp & waves Secondary generalization Localized paroxysmal fast activities Spindle shaped fast activities No. of patients % N11 11 100.0 05 045.5 04 036.4 06 054.5 03 027.3 03 027.3 02 018.2 01 001.2 01 009.1 01 009.1 - - Table 6. EEG features in video/eeg monitoring EEG features Background abnormalities n6 No. of patients % n6 Slow and disorganized 4 66.7 Asymmetric slowings 1 16.7 Polymorphic delta waves 1 16.7 Epileptiform discharges n6 Focal sharp or spikes 1 16.7 Multifocal sharp or spikes 4 66.7 Secondary generalization 1 16.7 Generalized sharp or polyspikes & waves GSW 5 83.3 Generalized paroxysmal fast activities 1 16.7 Localized paroxysmal fast activities 2 33.3 Subclinical electrographic seizure activities 1 16.7 Ictal discharges n6 GSW with myoclonic seizure 4 66.7 3 Hz GSW with absence seizure 1 16.7 Partial onset seizure 1 16.7 Table 7. MRI findings MRI Initial finding n18 Normal Subsequent finding n16 Diffuse atrophy No. of patiets % 18 100.0 02 012.5 Table 8. Effectiveness of antiepileptic drugs Outcome No. of patients % Intractable 18 100.0 Ethosuximide for ABS Effective at 1 patient Zonisamide for MC Effective at 1 patient ABSatypical absence seizure, MCmyoclonic seizure J Korean Epilep Soc Volume 6 December, 2002
강훈철 Oyungerel Dambajamts 김흥동 고 Table 9. Effectiveness of ketogenic diet No. of patients 13 Finished 02 Stopped due to ineffectiveness 05 Ongoing 06 Duration mo. 535 meansd, 20.49.3 Outcome seizure frequency reduction rate 5090% 06 90% 01 100% 1 for 7 mo. Table 10. Developmental status of patients Onset age of DD mo.meansd 03036 13.211.7 Developmental status on last evaluationno. of patients % Age on last evaluation mo. meansd 13126 64.629.5 Normal 2 11.1 Borderline 1 05.6 Mild DD 4 22.2 Moderate DD 7 38.9 Severe DD 4 22.2 DDdelayed development 찰 - J Korean Epilep Soc Volume 6 December, 2002 107
중증영아형근간대성간질의임상적고찰 108 - J Korean Epilep Soc Volume 6 December, 2002
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