1. Case presentation Neurology, Pediatrics

2. History 강 O 정 F / 9yr 5mon C.C : Seizure D :10 초 -5 분 (30-40 회 /day) PHx: NSVD at IUP 40wks B. Wt = 3.4kg No perinatal asphyxia No neonatal hyperbilirubinemia Head trauma (-) CNS infection (-) Vaccination Hx : as scheduled

3. Admission Hx : 1. 1995 년 6 월 infantile spasm 으로 서울대병원 입원 2. 2000 년 6 월 ketogenic diet 위해 상계백병원 입원 3. 2003 년 9 월 Pre-op evaluation 위해 본 원 입원 FHx: The 1st female baby No seizure history in family No known DM, HiBP, Pul tbc, Hepatitis

4. Seizure profile Onset : 생후 1 개월경 Duration: 20-30 초 Frequency: 30-40 회 /day Aura: Not definite Ictus : Eyeball rolling movement (to. Lt) Fever (-) Cyanosis (-) Postictal sleep (-) Current med. : Lamictal 100mg

5. Present Illness 본 9 년 2 개월된 여자환아는 생후 1 개월경 하루 2-3 회의 eyeball rolling movement 소견 보였으며, 생후 7 개월경 깜짝 놀라는 양상과 eyeball rolling movement 보여 local clinic 내원 이상 없다는 이야기 듣고 evaluation 없이 지내던중 1 년 8 개월경 infantile spasm 으로 서울대병원 입원 ACTH Tx. 시행하였으나 증세 호전 없어 2000 년 6 월 ( 생후 5 년 10 개월 ) 상계백병원 내원 2002 년 12 월까지 ketogenic diet 시행하였으며 영아 연축 증상은 호전 되었으나 eyeball rolling movement 소견 호전 없어 grid insertion 및 surgical Tx. 위해 입원함

6. Review of system Fever / chilling ( - / - ) Cough / Sputum ( - / - ) Anorexia / Nausea / vomitting ( - / - / - ) Poor oral intake ( - ) Constipation / Diarrhea ( - / - )

7. Physical Examination I G/ABP 110 / 70 (75~90P), PR 95 회 /min Not so ill looking appearance Alert mental state Moderate developed moderate nourished HC 55cm(>97p) Wt 23kg(50-75p) Ht 122cm(25-50p) SKINWarm & dry No abnormal skin rash

8. HEENT Normocephaly without deformity Not pale conjunctivae Not icteric sclera Not dried lip & tongue No throat injection Neck No palpable cervical LNs No throat injection

9. Chest Sym. expansion s retraction Clear breath sound without rale No wheezing, No rhonchi HeartRegular heart beat s murmur No thrill, No heaving Abdomen Soft & flat Normoactive bowel sound No palpable mass No organomegaly Back & Ext.No LOM No pitting edema Physical Examination II

10. Neurologic Examination Alert mental state Isocoric pupil with PLR (+/+) Corneal Rx (+/+) Motor & Sensory : intact DTR : NL AC (-/-) Babinski (-/-)

11. Psychologic evaluation 인지 검사 49 점 Mild mental retardation 언어 60 점 동작 47 점 사회성숙도 62 점

12. Initial impression Lennox-Gastuat SD Lennox-Gastuat SD Delayed development Delayed development R/O Cortical dysplasia R/O Cortical dysplasia

13. Lab finding Intial lab CBC5200 / 13.8 / 40 / 133k (25/63/5) electrolyte139 / 4.1 / 105 / 25 (82) Ca / Mg 9.5 / 3.9 Ca ++ / Mg ++ 4.77/ 1.03 NH 3 77 Drug level Pb 39.37 (15-40)

14. Progress HOD #4 s/p Grid insertion Vital BP 117/40 PR 89 회 /min CBC 8600/12.9/38/117k (86/7/5) Electro 132/3.7/97 (98) Brain CT : open craniotomy and grid insertion HOD #9 s/p Rt. occipital lobectomy Vital BP 105/48 PR 88 회 /min Brain CT : Rt. occipital lobectomy

15. POD #1 Fever (+) Seizure (-) A/N/V (-/+/-) Vital BP 120/80 PR 103 회 /min CBC 10470/9.8/27/167k (75/16/6) Electro 130/3.8/101/19 (204) POD #2 Seizure(+) Lt. Side EBD 및 eye blinking 양상 (5 초간 3 회 )

16. POD #4 Fever (-) Cough/Sputum (-/-) Seizure (+) Lt. Side EBD, eye blinking, G. tonic 양상 으로 10 초 -5 분간 약 10 여회 관찰됨. Vital BP 110/70 PR 84 회 /min POD #8 Fever (-) Cough/Sputum (-/-) Seizure (-)

17. Study Brain MRI (2000. 6.14) Slightly increased signal intensity at Rt. hippocampus Brain MRI (2003. 9.18) Within normal limit. Brain ictal SPECT (2003. 9. 18) Increased perfusion in the Rt. occipital lobe. Increased perfusion in the Rt. basal ganglia and Lt. cerebellum, likely due to diaschisis.

18. PET (2003. 9.19) Decreased FDG uptake in the Rt. Occipital and parieto-occipital region, suggestive of cortical dysplasia. Symmetric uptake in the basal gangliae and thalami. Brain interictal SPECT (2003. 9. 22) Severely decreased perfusion in the Rt. occipital and parietooccipital areas. Mildly decreased perfusion in the temporal lobe, basal ganglia, and thalamus on the Lt.

19. EEG 2000. 10. 17 GPFAs, fast arousal responses, occasional independent sharp waves from Rt. occipital region. 2004. 1. 2 asymmetry attenuation of alpha rhythms on Rt.occipital region and occasional asymmetric slowing on Rt.occipital area.

20. 2004. 1. 2 asymmetry attenuation of alpha rhythms on Rt.occipital region and occasional asymmetric slowing on Rt.occipital area.

21. Video-EEG Monitoring

26. Pathologic finding Cortical dysplasia, presence of giant dysplastic neurons and balloon cells in cortex and white matter

27. END

29. 4013668 강민정, F 2003-10-2 OP-Grid insertion 1 6 11 16 2 7 12 17 3 8 13 18 4 9 14 19 5 10 15 20 8 7 6 5 4 3 2 1 MO 4 3 2 1 PT 8 7 6 5 4 3 2 1 SO (Red Yellow) (Red Green) (Red Orange) 1 2 3 4 5 6 7 8 MO RO 8 7 6 5 4 3 2 1 SO 4 3 2 1 PT