Case presentation Neurology Pediatrics History O F 9

History 강O정 F / 9 yr 5 mon C. C : Seizure D :

Admission Hx : 1. 1995년 6월 infantile spasm으로 서울대병원 입원 2. 2000년 6월 ketogenic

Review of system Fever / chilling ( - / - ) Cough / Sputum

Physical Examination I G/A BP 110 / 70 (75~90 P), PR 95회/min Not so

HEENT Normocephaly without deformity Not pale conjunctivae Not icteric sclera Not dried lip &

Physical Examination II Chest Sym. expansion s retraction Clear breath sound without rale No

Neurologic Examination Alert mental state Isocoric pupil with PLR (+/+) Corneal Rx (+/+) Motor

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

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

Lab finding Intial lab CBC 5200 / 13. 8 / 40 / 133 k

Progress HOD #4 s/p Grid insertion Vital BP 117/40 PR 89회/min CBC 8600/12. 9/38/117

POD #1 Fever (+) Seizure (-) A/N/V (-/+/-) Vital BP 120/80 PR 103회/min CBC

POD #4 Fever (-) Cough/Sputum (-/-) Seizure (+) Lt. Side EBD, eye blinking, G.

Study Brain MRI (2000. 6. 14) Slightly increased signal intensity at Rt. hippocampus Brain

PET (2003. 9. 19) Decreased FDG uptake in the Rt. Occipital and parieto-occipital region,

EEG 2000. 17 GPFAs, fast arousal responses, occasional independent sharp waves from Rt. occipital

2004. 1. 2 asymmetry attenuation of alpha rhythms on Rt. occipital region and occasional

Pathologic finding Cortical dysplasia, presence of giant dysplastic neurons and balloon cells in cortex

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Case presentation Neurology, Pediatrics

History 강O정 F / 9 yr 5 mon C. C : Seizure D : 10초-5분 (30 -40회/day) PHx: NSVD at IUP 40 wks B. Wt = 3. 4 kg No perinatal asphyxia No neonatal hyperbilirubinemia Head trauma (-) CNS infection (-) Vaccination Hx : as scheduled

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

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

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

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

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

Physical Examination II Chest Sym. expansion s retraction Clear breath sound without rale No wheezing, No rhonchi Heart Regular 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

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

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

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

Lab finding Intial lab CBC 5200 / 13. 8 / 40 / 133 k (25/63/5) electrolyte 139 / 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)

Progress HOD #4 s/p Grid insertion Vital BP 117/40 PR 89회/min CBC 8600/12. 9/38/117 k (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 88회/min Brain CT : Rt. occipital lobectomy

POD #1 Fever (+) Seizure (-) A/N/V (-/+/-) Vital BP 120/80 PR 103회/min CBC 10470/9. 8/27/167 k (75/16/6) Electro 130/3. 8/101/19 (204) POD #2 Seizure(+) Lt. Side EBD 및 eye blinking 양상(5초간 3회)

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

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.

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.

EEG 2000. 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.

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

Video-EEG Monitoring

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

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