west syndrome case presentation
DESCRIPTION
case presentationTRANSCRIPT
Welcome
to
Clinical MeetingPresented by
DR. Amlendra K. Yadav
DR. Chandra Shekhar Bhagat
Resident (phase-A)
Particulars of the Patient
Name : Ahona
Age : 3 month
Sex : Female
Address : Narayangonj
Religion : Islam
Date of Admission : 05/01/2014
Date of Exammination : 06/01/2014
Chief Complaints
• Seizure for 2 months
History of Present illness
According to the statement of informant mother , her child
was reasonably well two month back then she developed
seizure. The seizure was sudden, brief contraction of neck
and extremities. It occurred in cluster (3 – 4) episodes per
day and lasts for (3 - 5) minutes and during awakening.
There is no history of passing urine or defecation during
the episode of seizure.There was no postictical phemenon
.Mother also complainted that her child had no interest to
surroundings . There was no history of fever, vomitting,
respiratory problem, loss of consciousness or trauma.
For above mentioned complaints she visited general
pediatrician and was treated with anti epileptic drugs
for 4 weeks but the seizure didn’t improve .Then they
brought the child to BSMMU for better evaluation and
management.
Birth HistoryAntenatal : There was no history of fever, rashes,abortion
or sibs death.
Natal : Delivered by LUCS due to oligohydriamnios at
term .
Postnatal: Baby cried immediately after birth.
Immunization HistoryImmunization as per EPI schedule started.
Feeding HistoryThe child is on Exclusive breast feeding.
Milestones of Development
Gross motor :- no neck control
Fine motor :- palmar grasp present
Language :- cooing
Social :- smile present
Consanguinity
No H/O consanguinity.
Family History
She is the only issue of her parents.
Other family member are healthy.
History of Past Illness
Nothing significant.
Drug History Phenobarbitone for 2 weeks.
Sodium Valproate for 2 weeks.
Socio-Economical History
Belongs to poor socio-economical status
family , stays in pakka house , drink tubwell
water .
DRUG REACTION HISTORY
No past drug reaction history present .
Physical ExaminationGeneral
Appearance : alert, playfull
Pallor
Edema
Jaundice
Cyanosis Absent
Clubbing
Koilonychia
Dehydration
Neck vein : Not engorged
Lymph Node :- Not enlarged
Skin survey : – BCG mark present.
Signs of meningeal irritation : – Absent
Bony tenderness : – Absent
Fontanelle : - open but not bulged
Vital Signs
Temperature – 98o F
HR – 120 beats /min
BP – 70/40 mm Hg
RR – 40 breaths /min
ANTHROPOMETRY:
Weight - 5.5 kg, ( lies on 50th percentile)
Length - 62cm, ( lies on 25th percentile)
OFC - 38cm ( lies on 10th percentile )
Nervous system
• Patient is playfull
• Cranial nerve - Intact as per I exammined
• Motor :- Bulk of muscle – normal
Tone - normal
Jerk – Normal
• Sensory - normal
• Plantar – b/l extensor type
• Primitive reflex – normal
Inspection• Shape of the chest : normal• R/R : 38 breaths/min• Visible vein & Pulsation : absent• Scar mark : absent
Palpation• Trachea : centrally placed• Apex beat : left 5th ICS, medial to
midclavicular line
Respiratory System:
Percussion note
• Resonant all over the lung fields
Auscultation
• Breath sound : vesicular
• Added sound : absent
Inspection: No visible pulsation
Palpation:• Apex beat : left 5th ICS, • Thrill : absent • P2 : Not palpable• Lt. parasternal heave : absent
Auscultation:• 1st & 2nd heart sounds audible in all 4 areas • Murmur : absent
Cardiovascular system examination
Mouth & Oral cavity:
Tongue : NormalGum : NormalBuccal mucosa, tonsils & fauces : Normal
Alimentary system examination
AbdomenInspection:
Umbilicus centrally placed & inverted Engorged vein/visible peristalsis – absent
Palpation:
Soft, non tender, non-distendedLiver just palpable .Spleen not palpable
Percussion:Upper border of liver dullness : Rt. 5th ICSShifting dullness: Absent
Auscultation: Bowel sound : PresentBruit (Hepatic / Renal) : Absent
Salient FeaturesAhona 3 months old female child only issue of
non-consanguinous parent came from narayangonj,
was admitted with a complain of seizure for 2 month.
The seizure was sudden , brief contraction of neck
and extremities.It occurred in cluster (3 – 4) episodes
per day and lasts for 3 -5 min and during awakening.
There is no history of passing urine or defecation
during the episode of seizure . There was no post
ictical phemenon . Mother also complainted that her
child was not responsive to surrounding . She was
treated with anti-epileptic drugs for 4 weeks but the
condition didn’t improve. The child was delivered by
LUCS , there is no H/O perinatal Asphyxia.
On examination Ahona was alert ,playful , BP –
70/40 mm of hg , RR – 40/min , HR – 120 beats/min,
temperature – normal, BCG mark present, fontanels –
open but not bulged, signs of meningeal irritation -
absent ,lymph node – not enlarged , cranial nerve –
intact , bulk- normal , tone – normal , jerks – Normal,
plantar – b/l extension , lungs – B/L clear, P/A-Liver
just palpable . Other systemic exammination reveals
normal findings.
Provisional Diagnosis
???
Provisional Diagnosis
West-Syndrome
Differnential diagnosis
1) Early Myoclonic infantile encephalopathy
2) Early infantile epileptic syndrome
Differential diagnosis
D/D Point in favor Point against
West Syndrome
Seizure in cluster during
awakening
Developmental delay
Severe myoclonic
epilepsy in infancy
Age less than 2 month
Seizure in cluster
myoclonic jerks
Early infantile epileptic
syndrome
Age less than 2 month
Seizure in cluster
Tonic spasm
INVESTIGATION
AND
MANAGEMENT
E.E.G - 17/12/2013
Normal Findings
TORCH screening
CMV IgG - Positive
CMV DNA - Positive
USG of Brain :- Normal Study.
Inv./Date 06/01/2014 15/01/2014
C.B.CHb% 9.2 g/dl 9.4 g/dl
ESR 05 mm in 1st hr
T.C 17,000/cumm. 10,000/cumm.
D.C N=13%
L= 80%
N= 10%
L= 86%
Platelet count 3,00,000
SGPT 23 u/l
Sr.Creatinine 0.3 mg/dl
Eye Evaluation chorioretinitis
Hearing Test Normal
Final Diagnosis:
West syndrome with CMV positive
Follow-up on 16/01/2014
Subjective :- No new complain
Objective :- G/C – alert, playful, afebrile
respiratory rate – 38/min
heart rate – 110/min
temperature – 98o F
Blood pressure – 80/40mmhg
Asessment – Improving
Plan – stop inj. Gancyclovir
Rx after admission
Counseling
Breast feeding
Inj Gancyclovir-6mg/kg/dose- 12 hourly
Tab.Vigabatrin-100 mg/kg/day
Syp.Cefixime- 8 mg/kg/day
Developmental therapy
Follow-up PLAN
Weekly CBC examination
Repeat EEG before discharge
CT scan of brain
Thank You..