Pediatric Neurology OSCE (PG CME -Wadia)

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Pediatric Neurology OSCE (PG CME, Wadia Hospital)

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  • Pediatric Neurology OSCE (PG CME -Wadia)

    1. 1. NEUROLOGY Post Graduate CME OSCE - NeurologyBai Jerbai Wadia Hospital for Chidren, Mumbai
    2. 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. 3. • ADHD• Inattention, hyperactivity and impulsivity• Medications (methylphenidate, amphetamines, fluoxitine, atomoxitine), Behavior therapy
    4. 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. 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. 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. 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. 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. 9. • Kernicterus• Globus pallidus, dentate nucleus, cerebellar vermis, cochlear nuclei• Choreoathetoid cerebral palsy, dystonic / dyskinetic CP, Sensorineural deafness,• Audiometry testing
    10. 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. 11. • Congenital hemiplegia• MRI brain• Maternal injury, threatened abortion, APC resistance, coagulation problem
    12. 12. 6. Diagnosis• 3 Differential diagnosis• 3 differentiating features
    13. 13. • 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
    14. 14. 7. Diagnosis• Name 2 other skin lesions• Inheritance• Other systems you would examine• Name 3 lesions seen in the brain
    15. 15. • 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
    16. 16. 8. This 5 yr old comes with unsteadiness ofgait with recurrent respiratory infections.• Diagnose the condition Inheritance 3 salient features 2 lab investigations
    17. 17. • 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.
    18. 18. 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
    19. 19. • Raised ICT• Supra- Craniopharyngioma, astrocytoma, Gliomas, ganglioglioma• Infra- meduloblastoma, glioma• Craniopharygioma• Panhypopitutarism, Hypothyroidism, GH deficiency, Gonadotrophin deficiency, cortisol def.
    20. 20. 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
    21. 21. • TBM• Ischaemia, edema, exudates• Infarct, basal exudates, hydrocephalus ( communicating /non communcating) tuberculomas,• 4 drug AKT, steroids, anti edema, shunt surgery,
    22. 22. 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
    23. 23. • 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
    24. 24. 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
    25. 25. • 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
    26. 26. 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
    27. 27. • Pyogenic meningitis; pneumococci, H influenza, meningococci• Subdural empyema, cerebral absess, infarction.• Sensorineural deafness, hydrocephalus, CP, MR, Epilepsy, behavioural problems
    28. 28. 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
    29. 29. • CNS, abdominal, ophthalmology,• Krabbes, GSD [Pompes], Tay sachs, gangliosidosis• GM 1Gangliosidosis, sialidosis, tay sachs, niemann picks, sandhoffs, MLD, mucolipidosis• Leucocyte enzymes, fibroblast culture
    30. 30. 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
    31. 31. • MELAS• Mitochondrial deletion study MRI with spectroscopy CSF Lactate, Blood Lactate. ABG-Anion Gap Metabolic Screen• Maternal transmission• MERRFS, Kearn Sayers, Leighs, Alpers
    32. 32. 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?
    33. 33. • Aicardi syndrome• Corpus callosum agenesis• Posterior choroidal cysts,• choroidal lacunae• X linked Dominant
    34. 34. 17) A boy presents with this skin lesion-Give 4 differential diagnosisWhen and how much would you investigate?
    35. 35. • 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
    36. 36. 18) • Name the sign • 3 DDs • 3 investigations in sequence of importance • Inheritance/ locus
    37. 37. • Gowers Sign• Duchenne, SMA III, Limb girdle dystrophy, BMD, myopathy• Dystrophin gene study, EMG /NCV, CPK levels• X linked recessive/ Xp 21
    38. 38. • Diagnosis19) • 2 associated abnormalities • Classical CNS picture in CT scan and is described as • Inheritance
    39. 39. • Sturge Weber syndrome• Glaucoma, intracranial calcification, intractable epilepsy, hemangioma• Tram track calcification• Sporadic inheritance
    40. 40. • Identify• 20) • 2 salient features • Criteria for diagnosis in newborn period are called: • Child develops spastic quadriparesis.What will you suspect?
    41. 41. • Downs syndrome• Facies, congenital heart defects, hypothyroidism ,deafness, hypotonia• Halls Criteria• Atlanto-axial dislocation
    42. 42. • Diagnose• 22) • Inheritance • 2 associated abnormalities • Investigation for Raised ICT
    43. 43. • Apert syndrome• Autosomal dominant• Proptosis, syndactyly, MR• 3 D CTScan
    44. 44. • This child came• 23) with neuroregression from 7 months of age with abn hand movts • Diagnosis / DD • 3 salient features • Which milestones are delayed?
    45. 45. • Retts syndrome / Autism• Autism, stereotypies, microcephaly, loss of fine purposeful hand movements• Social, language
    46. 46. 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?
    47. 47. • SMA 1• EMG/NCV, Gene studies• SMA- AR,• Decreased fetal kick count, polyhydramnious, previous fetal loses, weak cry, resp problems at birth.
    48. 48. 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.
    49. 49. • 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
    50. 50. • 26 ) Label the• parts shown
    51. 51. • Corpus callosum• Lateral ventricle• 4 th ventrricle• Pons• medulla
    52. 52. EPILEPSY
    53. 53. 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
    54. 54. • EEG• Landau Kleffner syndrome• IV Ig , Steroids, AEDs
    55. 55. 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
    56. 56. • Lennox Gastaut syndrome• Valproate, Lamotrigine, Topiramate, clobazam• Carbamazepine, phenobarbitone• Corpus callosotomy, Ketogenic diet, vagal nerve stimulation
    57. 57. 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?
    58. 58. • EEG• Rolandic epilepsy/ BECTS• MRI/Neuroimaging• Good,• Carbamazepine /oxcarbamazepine
    59. 59. 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
    60. 60. • Hypocalcemia,• hypomagnesemia,• 5 th day fits,• BFNS,• hypoglycemia• BFNS
    61. 61. 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?
    62. 62. • Mesial temporal sclerosis• Human herpes virus 6• Temporal lobectomy
    63. 63. 6) What does this EEG show?• What is the drug of choice?• Prognosis?• One OPD procedure to confirm diagnosis
    64. 64. • 3 Hz spike and wave activity in Absence seizure – childhood /Juvenile• Valproate, Lamotrigine, clobazam• Good in childhood, slightly less for juvenile• Hyperventilation
    65. 65. Jerk jerk
    66. 66. 7) Diagnosis• Confirmatory test• 3 therapeutic options
    67. 67. • SSPE –burst suppression pattern• CSF measles IgG• Ribavarine, interferons, inosiplex, amantidine
    68. 68. 8) Diagnosis• Commonest etiology• Drug of choice
    69. 69. • PLEDS• Herpes encephalitis• Acyclovir
    70. 70. 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
    71. 71. • Lorazepam [0.05mg/kg],• Diazepam [0.3mg/kg],• midazolam[ 0.2mg/kg]• Phenytoin[ 20mg/kg],• phenobarb[20/kg]• Midazolam drip,• propofol,• thiopentol
    72. 72. 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?
    73. 73. • Infantile spasms, West syndrome• EEG, MRI• Hypsarrhythmia• ACTH / steroids, Vigabatrin, Valproate /Topiramate / Nitrazepam
    74. 74. 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?
    75. 75. • 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
    76. 76. 12) Topiramate• 2 indications• 2 side effects• Contraindicated in• Tablet strength? Syrup? Any other preparation known?
    77. 77. • 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
    78. 78. 13) Carbamazepine• Commonest side effects• Not used in• Higher derivative with advantage• Syrup strength
    79. 79. • Ataxia , diplopia, rash, hyponatremia, cognitive decline, behavior concerns• Absence, myoclonic• Oxcarbazepine, less side effects• 5ml = 100mg
    80. 80. 14)Lamotrigine• Indication, Dose• Synergistic with?• What dosage modification done?• Life threatening side effect?
    81. 81. • Partial, generalied, LGS, JME;• 3-7 mg/kg/d• Valproate,• Reduce the dose of VPA prior to starting• Steven Johnson syndrome
    82. 82. • 15)Name 3 newer AEDs• Name a carbonic anhydrase inhibitor used as AED
    83. 83. • Ocarbamazepine, Topiramate, Lamotrigine, Vigabatrine, Gabapentine, Zonisamide, Leviteracetam, Felbamate, Tiagabine• Acetazolamide, Topiramate
    84. 84. 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
    85. 85. • Pyridoxine• Biotin• Metabolic workup, MRI – dysplasia, metabolic
    86. 86. 17) 1st unprovoked generalised seizure in a 2 yr old child• Essential investigation• Optional investigation• Treatment of choice
    87. 87. • EEG• MRI, biochemistry, CSF• Nil , unless specific indication
    88. 88. http://oscepediatrics.blogspot.in

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