Moyamoya disease

1. Clinical Meeting
Dr. Ainaeek Zaman
Resident, Phase A(Year-1)
Pediatric Hematology and Oncology,BSMMU
Dr. Meher Nigar Nishi
Resident, Phase-B(year-4)
Pediatric neurology & neurodevelopment, BSMMU

2. Particulars of the Patient
Name: Mohona
Age: 8 years
Sex: female
Religion: Islam
Address: Munshiganj.
Date of admission: 20 september ,2021.
Date of examination: 20 september,2021
Informant : Mother

3. Chief complaints
 Sudden weakness of right side of the body for 12 days

4. H/O present illness
According to the statement of informant mother, her child was reasonably
well 12 days back then she developed sudden weakness on right side of
the body involving both upper & lower limbs in the form of difficulty in
walking and difficulty in holding any object by right hand .On query mother
gave history of occasional headache for last 2 years which had no
aggravation or relieving factor.

5. H/O present illness cont..
There was no history of unconsciousness, head trauma, vomiting,
convulsion, incontinence, swallowing difficulties or difficulties in speech.
There was no previous history of such type of illness, any bleeding
manifestation, cyanosis, or acute fluid loss. With the above complaints
she was admitted in BSMMU for further evaluation and management.

6. Birth history:
Antenatal History : Mother was on regular ANC.
Natal History : Delivered by NVD at term at home with average birth weight.
Postnatal History : Uneventful.
Developmental history:
She is developmentally age-appropriate.
History of past illness: nothing significant.

7. Feeding History
She was on exclusive breast feeding until her 6 months of age. Then
complementary feeding was started. Now she is on family diet.
Immunization History
She is immunized as per EPI schedule.
Family History
She is the only issues of her consanguineous parents. No other family
member has similar types of illness.

8. Socio-economic History
She belongs to a middle income family. Her father is a service holder, mother is a
home-maker. They lives in a paca house, drinks tube-well water and use
sanitary latrine.
Treatment History
No significant drug history

9. PHYSICAL EXAMINATION

10. General examination:
• Appearance : well alert, co-operative
• Pallor : Absent
• Jaundice : Absent
• Cyanosis : Absent
• Clubbing : Absent
• Koilonychia : Absent
• Leukonychia : Absent

11. General Examination cont..
• Edema : Absent
• Dehydration : Absent
• Skin survey : Normal
• BCG mark : Present
• Lymph nodes : Not enlarged
• Signs of meningeal irritation : Absent

12. General Examination cont..
• Ear, Nose and Throat : Normal
• Eye Evaluation : Normal
• Vital signs :
Temp - 98.0ᴼ F
Pulse - 84 beats/min
BP - 95/60 mmHg(SBP-50th centile and DBP-between 50th-90thcentile)
RR - 20 breaths /min

13. • Weight : 22 kg
• (10th to 25th centile )
Height : 120 cm
( 10th centile)

14. Systemic Examination

15. Nervous System Examination
Higher Psychic Function :
Conscious and co-operative
Speech – normal
Orientation – oriented
Cognition - normal
Cranial Nerves : intact

16. Motor Function
Lower limbs
• Bulk of muscle : normal
• Tone of the muscle : increase on right side, normal on left side
• Muscle power : on right side 3/5, 5/5 on left side
• Reflexes
• Clonus : Absent
• Involuntary movement: Absent
right left
Knee jerk increased N
Ankle jerk increased N
Planter reflex extensor Flexor

17. Motor function cont..
• Upper limbs
No visible muscle wasting
Tone of the muscle : increase on right side, normal on left side
Muscle power : 4/5 on right side , 5/5 on left side
Reflexes :
• right left
Biceps Increase N
Triceps Increased N
Supainator increased N

18. Motor function cont..
• Sensory function: Intact
• Cerebellar Function: Intact
• Fundoscopic examination : Normal
• Gait: Hemiplegic gait

19. Respiratory System Examination
Inspection :
• Respiratory Rate : 20/min
• Shape of the chest : Normal
• Chest Movement : Symmetrical
Palpation :
• Trachea : Centrally Placed
• Chest expansibility : Symmetrical
• Vocal Fremitus : normal

20. Respiratory system examination cont..
Percussion : Resonant
Auscultation :
• Breath Sound : normal
• No added sound

21. Cardiovascular system examination
Inspection :
• No visible pulsation.
Palpation :
• Apex Beat : Located in the Left 5th ICS , just medial to the mid-clavicular
Line.
• Thrill : Absent
• Left Parasternal Heave : Absent
• Palpable P2 : Absent

22. CVS examination Cont..
Auscultation :
• Heart Sound : 1st and 2nd Heart sounds are audible in all 4 cardiac areas.
• Murmur : Absent

23. Alimentary System examination
• Oral Cavity : Healthy
• Abdomen proper :
Inspection :
 Shape : Normal
 Umbilicus : Centrally Placed ,inverted
 No visible mass, no scar mark.

24. Alimentary system examination cont..
• Palpation :
 Abdomen is soft, non tender, no organomegaly
 Fluid thrill - Absent
• Percussion :
Percussion note : Tympanic
 Shifting dullness : Absent
• Auscultation :
Bowel sound : Present

25. Genitourinary System examination
• Kidneys : Not Palpable.
• Urinary Bladder : Not Palpable
• Genitalia : Normal

26. Salient features
Mohona, 8 years old girl, only issue of her consanguineous parents admitted
with the complaints of sudden weakness on right side of the body involving both
upper & lower limbs. Mother also complaints of occasional headachae for last 2
years.
She had no history of headache, vomiting, convulsion, unconsciousness,
visual disturbance, any trauma, any bleeding manifestation , cyanosis,
dehydration.

27. Salient feature cont..
She is vitally stable, anthropometrically well thriving. On nervous system
examination, higher psychic function is normal. Cranial nerves are intact,
features of upper motor type of lesion on right upper & lower limb. Sensory
function and cerebellar function is intact. she has hemiplegic gait. Other
systemic examination revealed no abnormality.

28. PROVISIONAL DIAGNOSIS
??

29. Acute Stroke Syndrome

30. Diagnosis Points in favor Points against
Acute stroke
syndrome
 History:
•Sudden weakness of right
side of body .
 O/E :
•Signs of upper motor
neuron lesion in right upper
& lower limb.

31. Differential diagnosis
• ICSOL

32. Points in favor Points against
• Weakness of right side of body
• O/E :
Signs of upper motor neuron lesion in
both right upper and lower limb.
• Sudden onset
• Course-not progressive
• O/E :
No features of raised ICP
Eye evaluation -normal
ICSOL

33. INVESTIGATION

34. Plan of investigations
• To diagnose:
Neuroimaging- MRI of brain, MRA of brain.
• To find the etiology :
• CBC with PBF
• Cardiac evaluation- chest x ray, ECG, ECHO.
• Antinuclear antibody (ANA).
• Prothrombotic condition- anti cardiolipin antibodies, anti phospholipid
antibody, antithrombin III, coagulation profile etc.

35. CBC Results
Hb% 13.7 gm/dl
ESR 33 mm in 1st hr
Total RBC 5.00 million/cmm
Total RBC 13,520/cmm ( N-58%, L- 36%)
Total Platelet 3,50,000/cmm
MCV 78.7fl
MCH 27.1 pg
MCHC 34.4 gm/dl
Investigations

36. MRI of Brain

37. MRI of brain

38. MRI of brain

39. MRI of brain

40. MRA of brain

41. MRA of brain
Normal MRA of brain
MRA of our patient
ICA
Basilar
MCA ACA

42. MRA of brain

43. Final diagnosis
Moyamoya Disease

44. Treatment
• Counseling
• Diet: Normal
• Tab : Aspirin (3mg/kg/day)
• Tab Flunarizine
• Physiotherapy

45. Follow-up on Day 1
Subjective Objective Assessment Plan
No new
complaints
Appearance- Normal
BP- 100/60 mm hg
Pulse-90 b/m, regular
Temp- normal
RR- 22/min
Nervous system exam:
Motor Function
Bulk – normal, symmetrical
Tone – Normal
Power –
right upper limb 3/5
right lower limb 3/5
Jerk – exaggerated on right side
Planter: Extensor on right side
Static Neurosurgery
consultation

46. On discharge
• Tab Aspirin
• Tab Flunarizine

47. 47