This child presents with drooping eyelids and squint that worsens in the evenings. - Myasthenia gravis. - Ask about weakness of other muscles, response to rest. - Tensilon test and acetylcholine receptor antibody levels would help confirm the diagnosis.

1. NEUROLOGY
Post Graduate CME
OSCE - Neurology
Bai Jerbai Wadia Hospital for Chidren,
Mumbai

2. 1) 4 yrs old, boy , FTND, Normal motor,
mental and language milestones.
Complaints from school – aggressive,
cant sit in one place, restless,
forgetful and impatient, problems with
friends, fights
• Diagnosis?
• 3 cardinal features of this condition
• 2 treatment options

3. • ADHD
• Inattention, hyperactivity and impulsivity
• Medications (methylphenidate,
amphetamines, fluoxitine, atomoxitine),
Behavior therapy

4. 2)This child has
epilepsy, MR, ataxia,
unprovoked
laughter, severe
speech delay
• Diagnosis?
• Chromosome involved
• Inheritance
• Name a syndrome with
the same
chromosomal
abnormality

5. • Angelman syndrome
• 15q11.2-12
• Uniparental disomy – usually maternal (60-
75%)
- occ. paternal
- normal chromosome
? Dominant mutations
• Prader Willi – 15q11-13 (paternal origin)

6. 3) 2 yrs old child born prematurely [28wks]
comes with complaint of delayed
achievement of motor milestones.
Examination shows hypertonia with brisk
reflexes in all 4 limbs more marked in lower
limbs.
• What is the diagnosis? Define.
• What is the MRI picture?
• What is the treatment and when do you start it?
• Name 2 conditions this child will be at risk for?

7. • Spastic Diplegia. Increased tone in all 4
extremities and face. LL>UL> face
• Periventricular leukomalacia
• Early intervention
• Learning disabilities,
• Visual concerns
• Epilepsy
• Delayed mental development
• Behavior concerns

8. 4.A full term ,male child develops jaundice
on day 3 of life, (S. bilirubin –34 mg%)and
undergoes an exchange transfusion .
• What is the immediate complication likely to
occur?
• Where is the anatomical abnormality?
• What are the long term complications?
• Name 1 investigations you would insist on
after discharge?

9. • Kernicterus
• Globus pallidus, dentate nucleus, cerebellar
vermis, cochlear nuclei
• Choreoathetoid cerebral palsy, dystonic /
dyskinetic CP, Sensorineural deafness,
• Audiometry testing

10. 5. A child with apparently normal birth
history :MS- social smile at 3 mths,
Sitting at 8 months, rt hand
preference at 9 mths, walking
independently at 1.6 yrs.
• What is your diagnosis?
• What investigation will you do?
• What relevant maternal history will you
ask for?

11. • Congenital hemiplegia
• MRI brain
• Maternal injury, threatened abortion,
APC resistance, coagulation problem

13. 6. Diagnosis
• 3 Differential diagnosis
• 3 differentiating features

14. • Ring enhancing lesion
• NCC, tuberculoma, toxoplasma,
• abscess, tumor
• Peripheral vs central
• single vs multiple
• perilesional edema
• smooth vs irregular
• Scolex seen
• Wall thickness
• Intensity of cystic material

16. 7. Diagnosis
• Name 2 other skin lesions
• Inheritance
• Other systems you would examine
• Name 3 lesions seen in the brain

17. • Ash leaf macule in Tuberous sclerosis
• Adenoma sebaceum , shagreen patch,
café au lait spots
• Autosomal dominant
• Eyes, heart, kidneys, brain
• Tubers, subependymal nodules,
subependymal giant cell astrocytomas

18. 8. This 5 yr old comes with unsteadiness of
gait with recurrent respiratory infections.
• Diagnose the condition
Inheritance
3 salient features
2 lab
investigations

19. • Ataxia Telangiectasia
• Autosomal recessive
• FEATURES: Ataxia, telangiectasia,
immunodeficiency, recurrent sinopulmonary
infections, X ray hypersensitivity, malignancies
• LAB: Alpha feto proteins, Immunoglobulins,(low
IgA, IgG, high IgM), chromosomal breaks with
exposure to radiation.

20. 9. 8 yr old boy with headache,
vomiting, ataxia, diplopia.
• Clinical diagnosis?
• MRI shows SOL
• .Name 2 supra and infra tentorial SOLs
• For this SOL what other abnormalities
will you expect

21. • Raised ICT
• Supra- Craniopharyngioma, astrocytoma, Gliomas,
ganglioglioma
• Infra- meduloblastoma, glioma
• Craniopharygioma
• Panhypopitutarism, Hypothyroidism, GH deficiency,
Gonadotrophin deficiency, cortisol def.

22. 10. CSF picture
Protein 1640mg% ,
sugar 45/92 ,
cells 150 P40 L60
• Identify the condition
• 3 pathological etiologies
• 3 features on CT scan
• Treatment options

23. • TBM
• Ischaemia, edema, exudates
• Infarct, basal exudates, hydrocephalus (
communicating /non communcating)
tuberculomas,
• 4 drug AKT, steroids, anti edema, shunt
surgery,

24. 11. Child with fever, headache ,
vomiting and altered sensorium.
• CSF picture :proteins 130,
sugar 40/90,
cells 240 P40 L 60
• Give 3 differentials
• Give 2 other investigations
• Treatment options

25. • Partially treated pyogenic meningitis,
early TBM, aseptic, viral meningitis
• Latex agglutination, HSV DNA PCR, CT
scan with contrast, EEG
• Antibiotics, acyclovir 10 mg/kg 8 hrly for
14 days, AKT, supportive

26. 12.Child with fever , convulsions,
altered sensorium
CSF picture : protein 62,
cells 95 P10 L90,
sugars 45/80,
RBCs 80/ hpf
• Additional 2 investigations of choice
• Treatment with dose

27. • EEG - PLEDS-periodic lateralizing
epileptiform discharges
• HSV PCR,( ? HSV IgG, IgM ), CT scan /
MRI with contrast – bi/ uni temporal
hyperintensities
• Acyclovir 10mg/kg/dose 8 hrly for 14 -21
days

28. 13)1.5 yrs old child with fever and
convulsions.
CSF picture : proteins 80,
sugar 20 /60,
cells 500 , P 90 L 10.
• Diagnose / 3 common organisms
• Child develops tense AF with focal
convulsions on day 8. Probable causes?
• 3 Long term sequelae

29. • Pyogenic meningitis; pneumococci, H
influenza, meningococci
• Subdural empyema, cerebral absess,
infarction.
• Sensorineural deafness,
hydrocephalus, CP, MR, Epilepsy,
behavioural problems

30. 14)11 months old comes with
neuroregression from 5 months of
age. He is hypotonic on examination.
• Name 3 systems you would like to
examine?
• Differentials
• Give 4 DDs of cherry red spots
• Investigation of choice

31. • CNS, abdominal, ophthalmology,
• Krabbes, GSD [Pompes], Tay sachs,
gangliosidosis
• GM 1Gangliosidosis, sialidosis, tay
sachs, niemann picks, sandhoffs, MLD,
mucolipidosis
• Leucocyte enzymes, fibroblast culture

32. 15)18 months old child comes with
recurrent strokes , ptosis , ataxia and
myopathy.
• Diagnosis
• Investigation of choice
• Inheritance
• Name 2 more conditions of the same
metabolic category

33. • MELAS
• Mitochondrial deletion study
MRI with spectroscopy
CSF Lactate, Blood Lactate.
ABG-Anion Gap
Metabolic Screen
• Maternal transmission
• MERRFS, Kearn Sayers, Leighs, Alpers

34. 16) 6 months old female child, FTND,
has asymmetric infantile spasms,
developmental retardation and
choroidal lacunae. MRI clinches the
diagnosis.
• What is the diagnosis?
• What is the MRI picture?
• What is the inheritance pattern?

35. • Aicardi syndrome
• Corpus callosum agenesis
• Posterior choroidal cysts,
• choroidal lacunae
• X linked Dominant

36. 17) A boy presents with this skin
lesion-
Give 4 differential diagnosis
When and how much would you
investigate?

37. • Café au lait spot.
• DD- NF,Mc cune albright, TS, Ataxia
telangiectasia, Maffuci, any
phakcomatosis, Chediak Higashi
• If asymptomatic –No treatment
VEP-Periodic intervals if
abnormal do MRI for
optic glioma
• If symtomatic –VEP, MRI,
EEG

38. 18)
• Name the sign
• 3 DDs
• 3 investigations in
sequence of
importance
• Inheritance/ locus

39. • Gowers Sign
• Duchenne, SMA III, Limb girdle
dystrophy, BMD, myopathy
• Dystrophin gene study, EMG /NCV,
CPK levels
• X linked recessive/ Xp 21

40. • Diagnosis
19)
• 2 associated
abnormalities
• Classical CNS picture
in CT scan and is
described as
• Inheritance

41. • Sturge Weber syndrome
• Glaucoma, intracranial calcification,
intractable epilepsy, hemangioma
• Tram track calcification
• Sporadic inheritance

42. • Identify
• 20) • 2 salient features
• Criteria for diagnosis
in newborn period
are called:
• Child develops
spastic
quadriparesis.What
will you suspect?

43. • Downs syndrome
• Facies, congenital heart defects,
hypothyroidism ,deafness, hypotonia
• Halls Criteria
• Atlanto-axial dislocation

44. • Diagnose
• 22)
• Inheritance
• 2 associated
abnormalities
• Investigation for
Raised ICT

45. • Apert syndrome
• Autosomal dominant
• Proptosis, syndactyly, MR
• 3 D CTScan

46. • This child came
• 23) with
neuroregression
from 7 months of
age with abn hand
movts
• Diagnosis / DD
• 3 salient features
• Which milestones
are delayed?

47. • Retts syndrome /
Autism
• Autism, stereotypies,
microcephaly, loss of
fine purposeful hand
movements
• Social, language

48. 24) 10 month old , male, presents with
progressive decreased activity since 3
months of age, breathing difficulty since
8 mths and pneumonia. Examination
shows alert child with hypotonia and
absent reflexes.
• What is the diagnosis ?
• How do you confirm?
• What is the inheritance?
• Name 3 antenatal / natal features to ask for?

49. • SMA 1
• EMG/NCV, Gene studies
• SMA- AR,
• Decreased fetal kick count,
polyhydramnious, previous fetal loses,
weak cry, resp problems at birth.

50. 25) 3 yrs old girl comes with drooping of both
eyelids and squint noticed since 15 days.
Abnormality seems to increase in the
evenings.
• Diagnosis?
• Any 2 relevant questions?
• How do you confirm? Name 2 relevant
investigations.
• Drug of choice.

51. • Myasthenia gravis
• Any other activity tires with time eg.
Eating, walking, speech,
• Edrophonium test / Neostigmine test
• Anticholinesterase antibody, EMG with
repititive stimulation, CTScan chest
• Pyridostigmine

52. • 26 ) Label the
• parts shown

53. • Corpus callosum
• Lateral ventricle
• 4 th ventrricle
• Pons
• medulla

54. EPILEPSY

55. 1)4 Yrs old boy with normal motor and
mental development becomes aphasic for
last 15 days. He had a single GTC at 3.6
yrs.CNS examination is normal.
• Investigation of choice
• Diagnosis
• Treatment options

56. • EEG
• Landau Kleffner syndrome
• IV Ig , Steroids, AEDs

57. 2) 6 yrs old girl with delayed development.
Perinatal hypoglycemia. Symptomatic
infantile spasms at 7 mths. Then focal
seizures, generalised, myoclonic and now
tonic.
• Diagnosis?
• Drugs used?
• AEDs to be avoided?
• Non pharmacological treatment modalities

58. • Lennox Gastaut syndrome
• Valproate, Lamotrigine, Topiramate,
clobazam
• Carbamazepine, phenobarbitone
• Corpus callosotomy, Ketogenic diet,
vagal nerve stimulation

59. 3) 10 yrs old boy presents with lip smacking,
facial and eye deviation on left lasting 2
minutes after falling asleep. Similar
history 3 and 6 months back.
• Investigation of choice?
• Diagnosis?
• Which investigation you need not do?
• Prognosis?
• Drug of choice?

60. • EEG
• Rolandic epilepsy/ BECTS
• MRI/Neuroimaging
• Good,
• Carbamazepine /oxcarbamazepine

61. 4)4 days old newborn, FTND presents
with multiple multifocal seizures. He
has a normal systemic examination.
• DD- 3 most imp
• If strong family history of neonatal
seizures present, what will u think

62. • Hypocalcemia,
• hypomagnesemia,
• 5 th day fits,
• BFNS,
• hypoglycemia
• BFNS

63. 5) 6 yrs old boy comes with left sided
focal seizures preceded by aura of
fearfulness. He has history of
prolonged febrile seizures at 1 yr of
age. EEG shows right temporal
epileptiform activity.
• What MRI picture do you expect?
• Which virus has been suspected for the
same lesion?
• What are the treatment options?

64. • Mesial temporal sclerosis
• Human herpes virus 6
• Temporal lobectomy

66. 6) What does this EEG show?
• What is the drug of choice?
• Prognosis?
• One OPD procedure to confirm
diagnosis

67. • 3 Hz spike and wave activity in Absence
seizure – childhood /Juvenile
• Valproate, Lamotrigine, clobazam
• Good in childhood, slightly less for
juvenile
• Hyperventilation

68. Jerk jerk

69. 7) Diagnosis
• Confirmatory test
• 3 therapeutic options

70. • SSPE –burst suppression pattern
• CSF measles IgG
• Ribavarine, interferons, inosiplex,
amantidine

72. 8) Diagnosis
• Commonest etiology
• Drug of choice

73. • PLEDS
• Herpes encephalitis
• Acyclovir

74. 9) 6 yr old male admitted with
prolonged generalised seizures.
• Immediate AEDs –name 2 with dose
• 2nd line AEDs –2 with dose
• 3rd line – name 2

75. • Lorazepam [0.05mg/kg],
• Diazepam [0.3mg/kg],
• midazolam[ 0.2mg/kg]
• Phenytoin[ 20mg/kg],
• phenobarb[20/kg]
• Midazolam drip,
• propofol,
• thiopentol

76. 10) 6 months old child with h/o
perinatal insult comes with
regression of social milestones and
clusters of startles on awakening .
• Diagnosis
• Name 2 investigations you will ask for?

77. • Infantile spasms, West syndrome
• EEG, MRI
• Hypsarrhythmia
• ACTH / steroids, Vigabatrin, Valproate
/Topiramate / Nitrazepam

78. 11) SODIUM VALPROATE
• Each 5ml of syrup contains
• Commonest 3 side effects
• Contraindicated in
• Synergistic activity with
• Dosage range
• With Lamotrigine what precautions Are
needed?

79. • 200 mg, or 40 mg/ml
• Weight gain, hair loss, hepatotoxic,
PCOD, hirsutism, hyperammonemia
• Inherited Metabolic disorder, underlying
liver dysfunction
• Lamotrigine
• 10-40 mg/kg/day
• Cut the dose of VPA when adding LMT

80. 12) Topiramate
• 2 indications
• 2 side effects
• Contraindicated in
• Tablet strength? Syrup? Any other
preparation known?

81. • Generalised, partial, Infantile spasms, LGS
• Wt loss, word finding difficulty – speech
regression, blurred vision, renal calculi,
hyperthermia
• thin children, speech concerns
• 25 / 50 / 100mg. No syrup. Sprinkle

82. 13) Carbamazepine
• Commonest side effects
• Not used in
• Higher derivative with advantage
• Syrup strength

83. • Ataxia , diplopia, rash, hyponatremia,
cognitive decline, behavior concerns
• Absence, myoclonic
• Oxcarbazepine, less side effects
• 5ml = 100mg

84. 14)Lamotrigine
• Indication, Dose
• Synergistic with?
• What dosage modification done?
• Life threatening side effect?

85. • Partial, generalied, LGS, JME;
• 3-7 mg/kg/d
• Valproate,
• Reduce the dose of VPA prior to
starting
• Steven Johnson syndrome

86. • 15)Name 3 newer AEDs
• Name a carbonic anhydrase inhibitor used
as AED

87. • Ocarbamazepine, Topiramate,
Lamotrigine, Vigabatrine, Gabapentine,
Zonisamide, Leviteracetam, Felbamate,
Tiagabine
• Acetazolamide, Topiramate

88. 16) 3 months baby has intractable
epilepsy since birth and delayed
development
• Name 2 vitamins you can try
• Does not respond to them – what
investigation next

89. • Pyridoxine
• Biotin
• Metabolic workup, MRI – dysplasia,
metabolic

90. 17) 1st unprovoked generalised seizure
in a 2 yr old child
• Essential investigation
• Optional investigation
• Treatment of choice

91. • EEG
• MRI, biochemistry, CSF
• Nil , unless specific indication

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