KISEP Original Articles J Korean Epilep Soc 51:22-32, 2001 간질전문크리닉에서의간질증후군의진단 : 자기공명촬영의역할 Syndromic Diagnosis at the Epilepsy Clinic:Role of MRI 이병인 1 허경 1 김장성 3 김옥준 4 박선아 1 임성룡 1 김동익 2 윤평호 2 Byung In Lee, M.D. 1, Kyung Heo, M.D. 1, Jang Sung Kim, M.D. 3, Ok Joon Kim, M.D. 4, Sun Ah Park, M.D. 1, Sung Ryong Lim, M.D. 1, Dong Ik Kim, M.D. 2, Pyung Ho Yoon, M.D. 2 ABSTRACT Purpose Purpose Methods Results Results - Conclusion J Korean Epilep Soc 522-32, 2001 서 Department of Neurology, 1 and Diagnostic Radiology, 2 Yonsei University College of Medicine, Seoul, Korea Department of Neurology, Ajou University College of Medicine, Suwon, Korea Department of Neurology, Pochon Joongmoon Medical College, Sungnam, Korea 교신저자 론 - 22 J Korean Epilep Soc Volume 5 June, 2001
이병인 허경 김장성외 환자와방법 - - J Korean Epilep Soc Volume 5 June, 2001 23
간질증후군의진단 - 1. 임상적분류 - - 2. 임상과뇌파소견의연계 Table 1. Guidelines of clinical diagnosis first step diagnosis of lobar epilepsies Lobar epilepsies Temporal lobe Aura Visceral, experiential, emotional, psychic, or olfactory symptoms Descriptions of seizures Ictus Oroalimentary automatisms Nonspecific or none Blank staring oroalimentary automatisms Frontal lobe Limbic Nonspecific or none Blank staring only or asymmetric tonic spasm or hyperkinetic automatisms Conscious head version or asymmetric tonic spasm Parietal lobe Vertigo or other symptoms of spatial distortions Any Occipital lobe Elimentary visual symptoms, conscious eye pulling sensation Any Rolandic cortex None Elimentary focal or lateralized somatosensory and/or motor symptoms Temporo-parieto-occipital junction Complex visual symptoms, mixtures of auras indicating parietal, occipital, or temporal lobe origin Unlocalized Nonspecific or none Presence of any partial features Any 24 J Korean Epilep Soc Volume 5 June, 2001
이병인 허경 김장성외 Table 2. Guidelines of Clinical-EEG and Clinical-MRI correlations Clinical dx Clinical-EEG IEDs Clinical-MRI lesions C ND D C ND D Lobar epilepsy Temporal T1-2, A1-2 T3-4, Pg1-2 F7-8 N, MF/ML T5-6 G, other lobar Lesions limited to TL Lesion Frontal Fp1-2, F3-4 Fz, F7-8 N, MF/ML C3-4, Cz G, other lobar Lesions limited to FL Paretal P3-4, Pz N, MF/ML C3-4, Cz G, other lobar Lesions limited to PL Occipital O1-2 N, MF/ML T5-6 G, other lobar Lesions limited to OL Rolandic C3-4, Cz N, MF/ML F3-4, Fz P3-4, Pz G, other lobar Lesions limited to RC TPO-junction T5-6 N, MF/ML T3-4, A1-2 P3-4, O1-2 G, other lobar Lesions limited to TPO-junction Unlocalized N, Focal IEDs, MF/ML IEDs G any lesions *Multifocal or multilobar MF/ML GE idiopathic G N, F, MF/ML Negative Any Crypt/symp G N, F, MF/ML Symptomatic G N, F, MF/ML Focal, Ude Any Focal, Cconcordant NDnot-discordant Ddiscordant Nnegative Ggeneralized Ffocal MF/MLmultifocal or multilobar TLtemporal lobe FLfrontal lobe PLparietal lobe OLoccipital lobe RCrolandic cortex TPOtemporoparietooccipital F7-8either frontal or temporal according to the topography of second maximum amplitude of IEDs Multifocal or multilobarapplied only when the clinical diagnosis or second step diagnosis was unlocalized lobar epilepsy generalized epilepsy, or undetermined epilepsy - 3. 임상, 뇌파소견및 MRI의연계 결과 J Korean Epilep Soc Volume 5 June, 2001 25
간질증후군의진단 Table 3. Results of stepwise classification of epileptic syndromes Clinical Clin-EEG Clin-EEG-MRI 1. LRE 190 76% 199 80% 208 84% 1.1. Ldiopathic 1.1.1. Benign epilepsy of centrotemporal spikes 0 3 3 3 1.1.3. Primary reading epilepsy 1 1 1 1.2. Symptomatic 1.2.2. Sensory evoked 1 1 1 1.2.3. Lobar epilepsy 1.2.3.1. Localized 28 34 13 40 8 Temporal lobe 6 14 8 14 1 Frontal lobe 4 8 5 12 4 Parietal lobe 3 2 4 2 Occipital lobe 2 1 0 Rolandic area 10 8 8 TPO-junction 3 1 2 1 1.2.3.2. Unlocalized* 27 18 5 9 2 1.2.3.3. MF/ML 0 5 5 11 6 1.3. Cryptogenic 1.3.2. Sensory evoked 1 1 1 1.3.3. Lobar epilepsy 1.3.3.1. Localized 78 86 20 91 8 Temporal lobe 27 36 9 37 3 Frontal lobe 10 16 8 20 4 Parietal lobe 9 7 1 8 1 Occipital lobe 9 7 1 7 Rolandic area 14 12 12 TPO-junction 9 8 1 7 1.3.3.2. Unlocalized* 54 43 8 41 3 1.3.3.3. MF/ML 0 7 7 10 3 2. GE 24 10% 20 8% 20 8% 2.1. Idiopathic 58% 2.1.4. Childhood absence epilepsy 2 1 1 2.1.5. Juvenile absence epilepsy 1 2 1 2 2.1.6. Juvenile myoclonic epilepsy 4 4 1 4 2.1.7. GTC at awakening 10 1 1 1 2.1.8. GEF plus 1 1 1 2.1.9. Nonspecific GTC only* 6 1 1 1 2.2. Crypt/sympt 29% 2.2.2. Lennox-Gastaut syndrome 6 6 2 6 2.2.3. Myoclonic astatic epilepsy 1 1 1 2.3. Symptomatic 13% 2.3.1. Non-specific etiology* 1 1 1 1 2.3.2. Specific diseases 2 2 2 3. UDE* 34 14% 29 12% 20 8% 3.1. Both F and G 24% 3.1.5. Others 8 10 6 7 3.2. Without unequivocal F or G 76% 26 19 5 13 4. S.S 1 0.4% 1 0.4% 1 0.4% 4.1.3. Alcohol related 1 1 1 Total 249 249 79 249 30 non-specific categories, Ffocal, Ggeneralized, number of patients who changed their diagnostic categories TPOtemporo-parieto-occipital, GEFgeneralized epilepsy with fever 26 J Korean Epilep Soc Volume 5 June, 2001
이병인 허경 김장성외 1. 임상적분류 - - 2. 임상과뇌파소견의연계 (clinical-eeg correlations) - Table 4. Results of EEG and MRI in clinically diagnosed epileptic syndromes EEG MRI EEG/MRI Clinical diagnosis N F MF/ML G N F NF N/N P/N N/P P/P 1. LRE 1.1. Idiopathic 1.1.1. BECTS 0 1.1.3. PRM 1 0 0 0 1 1 0 0 0 1 0 0 1.2. Symptomatic 1.2.2. SSMP 1 1 0 0 0 0 1 0 0 0 1 0 1.2.3.1. LLE 28 10 13 3 2 6 14 8 4 2 6 16 1.2.3.2. LLE 27 13 11 3 0 7 17 3 5 2 8 12 1.3. Cryptogenic 1.3.2. SSMP 1 1 0 0 0 1 0 0 1 0 0 0 1.3.3.1. LLE 78 41 27 6 4 41 29 8 23 18 18 19 1.3.3.2. ULE 54 35 11 7 1 42 7 5 30 12 5 7 2. GE 2.1. Idiopathic 2.1.1-8. Specific 8 0 2 0 6 8 0 0 0 8 0 0 2.1.1.9. Others 6 4 2 0 0 4 2 0 2 2 2 0 2.2. Crypto/sympt 2.2.2-3. Specific 7 0 0 2 5 6 0 1 0 6 0 1 2.3. Symptomatic 2.3.1. Other 1 1 0 0 0 1 0 0 1 0 0 0 2.3.2. Specific 2 0 1 0 1 0 0 2 0 0 0 2 3. UDE 3.1.5. Both F/G 8 4 0 0 4 5 1 2 2 3 2 1 3.2. Uncertain 26 14 7 3* 2 20 4 2 9 11 5 1 4. Special syndrome 1 1 0 0 0 1 0 0 1 0 0 0 Total 249 125 50% 74 30% 24 10% 26 10% 143 57% 75 30% 31 13% 78 31% 65 26% 47 19% LRElocalization related epilepsy GEgeneralized epilepsy UDEundetermined epilepsy EEG patterns characteristic of benign epilepsy of centrotemporal spikes Ffocal MF/MLmultifocal or multilobal Ggeneralized NFnon-focal Nnegative Ppositive BECTShenign epilepsy of centrotemporal spikes PRMprimary reading epilepsy SSMPseizures with specific ULEunlocalized lobar epilepsy specificspecific syndromes othersother syndromes not defined F/Gfocal and generalized features 59 24% J Korean Epilep Soc Volume 5 June, 2001 27
간질증후군의진단 - - - - 3. 임상-뇌파-MRI의연계 (Clinical-EEG-MRI correlations) - - Table 5. Clinical-EEG and Clinical-MRI correlations in patients with clinically localized lobar epilepsies and positive results of both EEG and MRI Clinical-EEG Clinical-MRI Clinical diagnosis N C ND D N C ND D TLE n=33 15 16 a 2 b 0 9 19 a 4 b 1 a Extra-TLE n=73 36 7 a 11 4 a 7 b 1913 a 6 c 38 15 a 155 a 10 b 5 a FLE n=14 7 3 a 1 b 32 a 1 c 8 6 a 0 0 PLE n=12 7 0 1 b 43 a 1 c 7 3 a 1 b 1 a OLE n=11 5 1 a 1 b 4 a 8 1 a 1 b 1 a RE n=24 12 3 a 5 2 a 3 b 4 a 7 5 a 114 a 7 b 1 a TPO n=12 5 0 3 2 a 1 b 4 c 8 0 21 a 1 b 2 a afocal IEDs or lesion bmultifocal/multilobar IEDs or non-focal lesions cgeneralized IEDs Cconcordant NDnot-discordant Ddiscordant Nnegative 28 J Korean Epilep Soc Volume 5 June, 2001
이병인 허경 김장성외 Table 6. Clinical-EEG, Clinical-MRI, and EEG-MRI correlations in clinically diagnosed lobar epilepsies Localized LE Unlocalized LE TLE Ex-TLE Subtotal Sympt Crypt Subtotal Total Clinical-EEG Concordant 16 12 7 3 23 15 3 3 7 3 10 6 33 21 Not-discordant 2 2 11 9 13 11 11 9 11 4 22 13 35 24 Discordant 0 19 9 19 9 0 1 0 1 21 9 Clinical-MRI Concordant 19 11 13 32 18 12 7 4 3 16 10 48 28 Not-discordant 4 2 17 21 13 8 5 8 4 16 9 37 22 Discordant 1 1 5 6 4 0 0 0 6 4 EEG-MRI Concordant 12 6 18 6 5 11 29 Not-discordant 2 10 12 5 2 7 19 Discordant 0 5 5 1 0 1 6 LElobar epilepsy, TLEtemporal lobe epilepsy, Ex-TLEextra-temporal lobe epilepsy, Symptsymptomatic, Cryptcryptogenic, number of patients with positive EEG and MRI Table 7. Combination of Clinical-EEG, Clinical-MRI and EEG-MRI correlations in clinically diagnosed lobar epilepsies Combinations of Localized Unlocalized corelations TLE Ex-TLE Subtotal Sympt Crypt Subtotal Total C-C-C 11 79% 2 10% 13 37% 3 25% 2 29% 5 26% 18 33% C-D/ND 2 14% 10 48% 12 34% 7 58% 5 71% 12 63% 24 44% ND-ND-ND 0 5 24% 5 14% 1 8% 0 1 5% 6 11% ND-D/ND 1 7% 2 10% 3 9% 1 8% 0 1 5% 4 7% D-D-D 0 2 10% 2 6% 0 0 0 2 4% Cconcordance NDnot-discordance Ddiscordance TLEtemporal lobe epilepsy, Ex-TELextra-temporal lobe epilepsy Symptsymptomatic Cryptcryptogenic J Korean Epilep Soc Volume 5 June, 2001 29
간질증후군의진단 고찰 30 - J Korean Epilep Soc Volume 5 June, 2001
이병인 허경 김장성외 - Table 8. Proposal of Modified Classification of Epileptic Syndromes Applied MRI Features 1. Genetic LRE GE PME Cerebral dysgenesis 2. Idiopathic LRE GE 3. Cryptogenic LRElocalizedlesional vs non-lesional Unlocallesional vs non-lesional GEspecific Non-specific 4. Symptomatic LRE localizedlesional vs non-lesional Unlocallocallesional vs non-lesional GE Specificlesional vs non-lesional Non-specificlesional vs non-lesional 5. Undetermined 6. Specific Syndrome 중심단어 J Korean Epilep Soc Volume 5 June, 2001 31
간질증후군의진단 REFERENCES 1) Engel JJr. Classifications of the international league against epilepsy: time for reappraisal. Epilepsia 1998;39:1014-7. 2) Commission on classification and terminology of the international league against epilepsy. Proposal for classification of epilepsies and epileptic syndromes. Epilepsia 1985;26:268-78. 3) Commission on classification and terminology of the international league against epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia 1989;30:389-99. 4) Berg AT, Levy SR, Testa FM, Shinnar S. Classification of childhood epilepsy syndromes in newly diagnosed epilepsy interater agreement and reasons for disagreement. Epilepsia 1999;40:439-44. 5) Viani F, Beghi E, Atza G, Gulotta MP. Classifications of epilepsic syndromes advautages and limitations for evaluation of childhood epileptic syndromes in clinical practice. Epilepsia 1988;29:440-5. 6) Eslava-Cobos J, Narino D. Experience with the international league against epilepsy proposals for classification of epileptic seizures and the epilepsies and epileptic syndromes in a pediatric oatpatient epilepsy clinic. Epilepsia 1989;30:112-5. 7) Loiseau P, Dnche B, Loiseau J. Classification of epilepsies and epileptic syndromes in two different samples of patients. Epilepsia 1991;32:303-9. 8) Manford M, Hart YM, Sander JWAS, Shorvon SD. The national general practice study of epilepsy: the syndromic classification of the international league against epilepsy applied to epilepsy in a general population. Arch Neurol 1992;49:801-8 9) Shah KN, Ragadhyaksha SB, Shah VS, Shah NS, Desai VG. Experience with the international league against epilepsy classifications of epileptic seizures (1981) and epilepsies and epileptic syndrome (1989) in epileptic children in a developing country. Epilepsia 1992;33:1072-7. 10) Osservatorio Regionale Per L Epilessia (OREp), Lombardy ILAE classification of epilepsies: its applicability and practical value of different diagnostic categories. Epilepsia 1996;37:1051-9. 11) Osservatiorio Regionale Per L Epilessia (OREp), Lombardy. The contribution of tertiary centers to the quality of the diagnosis and treatment of epilepsy. Epilepsia 1997;38:1338-43. 12) Abdulizabar M, Ogunniyi A, Daif A, Al- Tahan A, Al-Bunyan M, Al-Rajeh S. Epilepsy classification and factors associated with control in Saudi adult patients. Seizure 1998;7:501-4 13) Eadie MJ. The ILAE classification of the epilepsies applied retrospectively to 1902 patients. Epilepsy Res 1996;25:277-84. 14) Zarrelli M, Beghi E, Rocca WA, Hauser WA. Incidence of epileptic syndromes in Rochester, Minnesota: 1980-1984. Epilepsia 1999;40:1708-14. 15) Resta M, Palma M, Dicuonzo F, et al. Imaging studies in partial epilepsy in children and adolescents. Epilepsia 1994;35: 1187-93. 16) Li LM, Fish DR, Sisodiya SM, Shorvon SD, Alsanjari N, Sterens JM. High resolution magnetic resonance imaging in adults with partial or secondary generalized epilepsy attending a tertiary referral unit. J Neurol neurosurg Psychiatry 1995;59:384-7. 17) Everitt AD, Sander JWAS. Classification of the epilepsies: time for a change? Eur Neurol 1999;42:1-10. 18) Mosewich RK, So EL. A clinical approach to the classification of seizures and epileptic syndromes. Mayo Clin proc 1996;71: 405-14. 19) Rektor I, Svejdova M, Kanovsky P, Landre E, Bancaud J, Lamarche M. Can epileptologists without access to intracranial EEG use reliably the International League Against Epilepsy Classification of the localization related epileptic syndromes? J Clin Neurophysiol 1997;14:250-4. 20) Manford M, Fish DR, Shorvon SD. An analysis of clinical seizure patterns and their localizing value in frontal and temporal lobe epilepsies. Brain 1996;119:17-40. 32 J Korean Epilep Soc Volume 5 June, 2001