NEUROLOGY Post Graduate CME OSCE - NeurologyBai Jerbai Wadia Hospital for Chidren, Mumbai
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) 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?
• 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
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?
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?
6. Diagnosis• 3 Differential diagnosis• 3 differentiating features
• 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
7. Diagnosis• Name 2 other skin lesions• Inheritance• Other systems you would examine• Name 3 lesions seen in the brain
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
• 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
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
• 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
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
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
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
• MELAS• Mitochondrial deletion study MRI with spectroscopy CSF Lactate, Blood Lactate. ABG-Anion Gap Metabolic Screen• Maternal transmission• MERRFS, Kearn Sayers, Leighs, Alpers
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?
• Aicardi syndrome• Corpus callosum agenesis• Posterior choroidal cysts,• choroidal lacunae• X linked Dominant
17) A boy presents with this skin lesion-Give 4 differential diagnosisWhen and how much would you investigate?
• 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
18) • Name the sign • 3 DDs • 3 investigations in sequence of importance • Inheritance/ locus
• Gowers Sign• Duchenne, SMA III, Limb girdle dystrophy, BMD, myopathy• Dystrophin gene study, EMG /NCV, CPK levels• X linked recessive/ Xp 21
• Diagnosis19) • 2 associated abnormalities • Classical CNS picture in CT scan and is described as • Inheritance
• This child came• 23) with neuroregression from 7 months of age with abn hand movts • Diagnosis / DD • 3 salient features • Which milestones are delayed?
• Retts syndrome / Autism• Autism, stereotypies, microcephaly, loss of fine purposeful hand movements• Social, language
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?
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.
• 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
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
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
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?
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
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?