CLINICAL MEETING

1. WELCOME TO CLINICAL
MEETING
Presented by:
DR. MOHTARAMA MOSTARI
DR. AZMERY SAIMA
Resident Phase-A
Neonatology

2. NUSRAT
09 months

3. Particulars of the patient
Name : Sujon.
Age : 5 years.
Sex : Male
Address : Comilla.
Date of visit in OPD : 20.12.16.
Date of examination : 20.12.16.
Informant : Father.

4. Presenting complaints
 Repeated episodes of seizure for last 3
years.
 Alternation of behavior for last 2 years..

5. History of present illness
According to the statement of the informant father,
Sujon developed repeated episodes of seizure for last 3
years, manifested by sudden contraction of neck and
both upper and lower extremities on to the trunk which
was bilaterally symmetrical, each episode occured in
clusters, each cluster consist of 3-4 volleys & 6-7
cluster/day .

6. For last 6 months pattern of seizure have
changed.There was tightening of all 4 limbs
followed by jerking movement with uprolling
of eyeball associated with post ictal drowsiness
for 1-2 hours, not associated with tongue bite,
bladder or bowel incontinence & altered level of
consciousness. Seizure occur 8-10 times per day.

7. Father also complained of alternation of behavior,
restlessness,hyperactivity & impaired
communication for last 2 years. With the above
complaints he was treated by village doctor with
irregular tab carbamazepine for 6 months. But the
condition was not improved so he visited paediatric
neurology OPD for evaluation and management.

8. Antenatal: Mother was on not regular antenatal check
up. She had no H/O Fever, Rash, HTN, DM .
Natal : Born at term by NVD at home . His birth
weight was average.
Post-natal:Nothing significant.
Birth history

9. EBF was given up to 6 months of his age , then
complimentary feeding was started now he is in
family diet.
Immunization history
Immunized as per EPI schedule
Feeding history

10. Developmental history
Standing achieved at 1 year &walking at 1 & half years.
Vision & hearing :intact.
Speech: 2 word sentence at 2 years.
Cognition:
Achieve toilet training at 2 years of age.
Had peer play 2 & half years.

11. Family history
He is the 4th issue of his consanguineous parents. His
elder brother is suffering from seizure disorder.
Socio economic history :
He belongs to low socioeconomic background. His father
is farmer & mother is a housewife and her father
monthly income around 20,000 taka. Lives in a pacca
house, Use sanitary latrine.
Treatment history: Irregular Tab Carbamazepine for
last 6 months.

12. Physical examination

13. Appearance : Alert, hyperactive, having multiple
red papular lesions over both cheek.
 Decubitus : On lying
 Nutrition : Average
 Pallor -
 Jaundice
 Cyanosis
 Dehydration
 Clubbing
 Koilonychia
 Leukonychia
 Oedema
General examination
Absent

14. Facial
angiofibroma

15.  BCG mark : Present
 Skin survey:
1.Multiple elongated hypopigmented areas in the
buttock & back of the thigh.
2.There are elevated skin lesion in the
lumbosacral & suprasternal region.
 Lymph nodes : Not palpable
 Signs of meningeal irritation: Absent
Back & spine: Normal

18. Shagreen patch

19. Vital Signs:
Temperature : 98 ° F
Pulse : 80 b/min
Respiratory Rate : 20 breaths / min
Blood Pressure : 90/50 mm Hg
( SBP& DBP<90th centile).

20. ANTHROPOMETRY
Height : 111 cm
(Lies between 50th and 75thth centile)
Weight : 15Kg
(Lies on 5th-10th centile)
OFC : 50 cm

21. Eye- opthalmological findings:normal.
Ear, nose, throat- normal

22. Systemic examination

23.  Higher psychic function:
I. Conscious, GCS 15/15,poorly co-operative.
II. Oriented about time,place,person:
III. Speech-decreased fluency.
IV. Behavior:Hyperactive,occational aggressive behavior.
V. Intelligence:cannot count fingers.
Cranial nerves: Intact.
Nervous system

24. Motor function
24
Upper Limb Right Left
Bulk Normal Normal
Tone Normal Normal
Power 5/5 5/5
Deep
Reflexes(biceps,
triceps, supinator)
Normal Normal
Involuntary
movements
Absent Absent

25. Lower limb Right Left
Bulk Normal Normal
Tone Normal Normal
Power 5/5 5/5
Deep
Reflexes(knee
jerk, ankle jerk)
Normal Normal
Plantar Flexor Flexor
Involuntary
movements
Absent Absent
25

26. Sensory Functions : Intact as far could be examined.
Cerebellar Functions Test : Intact as far could be
examined.
Gait: Normal.

27. Inspection :
Shape of chest -Normal. Respiratory rate- 20 breath/min,
Movement of the chest- Symmetrical, Intercostal recession-
absent. Visible pulsation-absent.
Palpation: Trachea- central, apex beat – on left 4th
intercostal space, chest expansibility-Normal, vocal
fremitus - Normal.
Percussion: Resonant.
Auscultation: Breath sound-vesicular
vocal resonance-normal.
Respiratory system

28. Inspection:
 No visible pulsation
 Pulse : 80 b/min
Palpation:
 Apex beat : left 4th ICS at mid
clavicular line.
 Thrill : absent
 P2 : not palpable
 Lt. parasternal heave : absent
Auscultation:
 1st & 2nd heart sounds audible in all 4 areas
 Murmur : absent
Cardiovascular System

29. GASTROINTESTINAL SYSTEM
Oral cavity : Healthy .
Abdomen proper :
Inspection :
Abdomen is normal in size and shape.
Umbilicus centrally placed .
No visible vein or peristalsis .
Palpation :
Local temparature- normal, Tenderness - absent.
No organomegaly.
Percussion
Fluid thrill & Shifting dullness – absent.
Auscultation
Bowel sound – present.

30. GENITOURINARY SYSTEM
Inspection :
Abdomen is normal in size and shape.
Umbilicus centrally placed and inverted.
External genitalia normal.
Palpation :
Kidneys not ballotable
Bladder not palpable
Renal angle tenderness absent
Auscultation :
Renal bruits absent

31.  Look
no joint swelling, no redness, no deformity
 Feel
no tenderness
 Move
no restriction of joint movement.
Locomotor system

32. Salient feature
Sujon ,05 year old boy,4th issue of consanguineous
parents,came from Comilla was admitted with the
complaints of repeated episodes of seizure for last 3
years & alternation of behavior for last 2 years . Seizure
was initialy epileptic spasm in nature for about 10
months followed by seizure free period for 6 months &
finally he developed generelized tonic clonic seizure for
last 1 &half years. Alternated behavior was menifested
by restlessness, hyperactivity & impaired
communication. .

33.  Irregular Tab Carbamazepine for last 6 months.
 On examination , Sujon was found alert, conscious,
hyperactive, poorly co-operative, afebrile, vital signs
were within normal limits . Skin survey reveals facial
angiofibroma, ash leaf in the buttock & back of the thigh
& shagreen patch in the lumbosacral & suprasternal
region. Anthropometrically well thriving.

34. Nervous system examination reveals impaired higher
psychic function evident by poor co-operation,
hyperactivity , less intelligence & less fluent speech .
Cranial nerves, motor & sensory & cerebellar function
were intact. Other systemic examination reveals normal
finding.

35. Provisional Diagnosis

36. Provisional diagnosis
 Tuberous sclerosis complex with
epilepsy

37. Points in favour
 Major features:
1.Facial angiofibroma
2.Shagreen patch
3.Hypopigmented macule(>3)
 Seizure since 2 years of age.
 Change in the behaviour

38. Investigations
1.MRI of brain
T2 image shows
subependymal nodule

39. Flair image shows cortical
tuber along with
subependymal nodule

40. Flair image shows
cortical tuber

42. 2. EEG

44.  EEG – Epileptic encephalopathy.
 USG of whole abdomen – normal findings
 Echocardiography- A tiny PDA
good biventricular function

45. Final diagnosis
Tuberous sclerosis complex with
epilepsy.

46. Management
 Counseling of the parents
 Diet – normal
 Tab. vigabatrin 500mg (50 mg/kg)
 Follow up- psychological assessment
visual assessment

47. Follow up on 14.01.17
subjective objective assessment plan
Seizure free for
1.5 month.
Conscious
Facial angiofibroma
Ash leaf
Shagreen patch
Vitals – Normal
HS- S1+S2
Lungs- clear
Improving Psychological
assessment

48. THANK YOU