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Welcome
to
Clinical Meeting
Presented 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 History
Antenatal : 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 History
Immunization as per EPI schedule started.
Feeding History
The 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 Examination
General
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 : Normal
Gum : Normal
Buccal mucosa, tonsils & fauces : Normal
Alimentary system examination
Abdomen
Inspection:
Umbilicus centrally placed & inverted
Engorged vein/visible peristalsis – absent
Palpation:
Soft, non tender, non-distended
Liver just palpable .
Spleen not palpable
Percussion:
Upper border of liver dullness : Rt. 5th ICS
Shifting dullness: Absent
Auscultation:
Bowel sound : Present
Bruit (Hepatic / Renal) : Absent
Salient Features
Ahona 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.C
Hb% 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..

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West syndrome case presentation

  • 1. Welcome to Clinical Meeting Presented by DR. Amlendra K. Yadav DR. Chandra Shekhar Bhagat Resident (phase-A)
  • 2. 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
  • 4. 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.
  • 5. 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.
  • 6. Birth History Antenatal : 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 History Immunization as per EPI schedule started. Feeding History The child is on Exclusive breast feeding.
  • 7. Milestones of Development Gross motor :- no neck control Fine motor :- palmar grasp present Language :- cooing Social :- smile present
  • 8. Consanguinity No H/O consanguinity. Family History She is the only issue of her parents. Other family member are healthy.
  • 9. History of Past Illness Nothing significant. Drug History Phenobarbitone for 2 weeks. Sodium Valproate for 2 weeks.
  • 10. 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 .
  • 11. Physical Examination General Appearance : alert, playfull  Pallor  Edema  Jaundice  Cyanosis Absent  Clubbing  Koilonychia  Dehydration  Neck vein : Not engorged
  • 12.  Lymph Node :- Not enlarged  Skin survey : – BCG mark present.  Signs of meningeal irritation : – Absent  Bony tenderness : – Absent  Fontanelle : - open but not bulged
  • 13. 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 )
  • 14. 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
  • 15. 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:
  • 16. Percussion note • Resonant all over the lung fields Auscultation • Breath sound : vesicular • Added sound : absent
  • 17. 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
  • 18. Mouth & Oral cavity: Tongue : Normal Gum : Normal Buccal mucosa, tonsils & fauces : Normal Alimentary system examination
  • 19. Abdomen Inspection: Umbilicus centrally placed & inverted Engorged vein/visible peristalsis – absent Palpation: Soft, non tender, non-distended Liver just palpable . Spleen not palpable Percussion: Upper border of liver dullness : Rt. 5th ICS Shifting dullness: Absent Auscultation: Bowel sound : Present Bruit (Hepatic / Renal) : Absent
  • 20. Salient Features Ahona 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.
  • 21. 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.
  • 24. Differnential diagnosis  1) Early Myoclonic infantile encephalopathy  2) Early infantile epileptic syndrome
  • 25. 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
  • 28. TORCH screening CMV IgG - Positive CMV DNA - Positive USG of Brain :- Normal Study.
  • 29. Inv./Date 06/01/2014 15/01/2014 C.B.C Hb% 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
  • 30. Final Diagnosis: West syndrome with CMV positive
  • 31. 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
  • 32. 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
  • 33. Follow-up PLAN  Weekly CBC examination  Repeat EEG before discharge  CT scan of brain