case presentation neurology, pediatrics. history 강 o 정 f / 9yr 5mon c.c : seizure d :10 초 -5...
TRANSCRIPT
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