Case presentation

Neurology, Pediatrics

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

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

Seizure profile

Onset : 생후 1 개월경 Duration: 20-30 초Frequency: 30-40 회 /day

Aura: Not definite

Ictus : Eyeball rolling movement (to. Lt)

Fever (-) Cyanosis (-)

Postictal sleep (-)

Current med. : Lamictal 100mg

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 2002년 12 월까지 ketogenic diet 시행하였으며 영아 연축 증상은 호전 되었으나 eyeball rolling movement 소견 호전 없어 grid insertion 및 surgical Tx. 위해 입원함

Review of system

Fever / chilling ( - / - )

Cough / Sputum ( - / - )

Anorexia / Nausea / vomitting ( - / - / - )

Poor oral intake ( - )

Constipation / Diarrhea ( - / - )

Physical Examination I

G/A BP 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)

SKIN Warm & dryNo 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

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

Physical Examination II

Neurologic Examination

Alert mental state

Isocoric pupil with PLR (+/+)

Corneal Rx (+/+)

Motor & Sensory : intact

DTR : NL

AC (-/-) Babinski (-/-)

Psychologic evaluation

인지 검사 49 점 Mild mental retardation

언어 60 점 동작 47 점 사회성숙도 62 점

Initial impression

Lennox-Gastuat SDLennox-Gastuat SD

Delayed developmentDelayed development

R/O Cortical dysplasiaR/O Cortical dysplasia

Lab finding

Intial lab

CBC 5200 / 13.8 / 40 / 133k (25/63/5)

electrolyte 139 / 4.1 / 105 / 25 (82)

Ca / Mg 9.5 / 3.9

Ca++ / Mg++ 4.77/ 1.03

NH3 77

Drug level

Pb 39.37 (15-40)

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

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 )

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 (-)

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. 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.

2004. 1. 2

asymmetry attenuation of alpha rhythms

on Rt.occipital region and occasional asymmetric slowing on Rt.occipital area.

Video-EEG MonitoringVideo-EEG Monitoring

Pathologic finding

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

cortex and white matter

END

4013668 강민정 , F2003-10-2OP-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

4321PT

87654321SO

(Red Yellow)

(Red Green)

(Red Orange)

1 2 3 4 5 6 7 8 MO

RO

87654321SO

4321PT