INI SS Pediatric Neurology July 2023 Session Recall PDF

INI SS Pediatric Neurology July 2023 session recall 1.Spasmus nutans triad ( nystagmus+ head nodding+ torticollis) - Not seen Generalized tremulousness 2.Rabies localizes in - Insular cortex - Brainstem -Anterior horn cell 3.CMS worsened with pyridoxine - DOK7 4..Which is false -NCC is mor...

INI SS Pediatric Neurology July 2023 session recall 1.Spasmus nutans triad ( nystagmus+ head nodding+ torticollis) - Not seen Generalized tremulousness 2.Rabies localizes in - Insular cortex - Brainstem -Anterior horn cell 3.CMS worsened with pyridoxine - DOK7 4..Which is false -NCC is more common than TB for infratenrorial location -False -TB has lipid peak in MRS -true 5.? Benign infantile convulsion resolved by 3 yrs, no family history, MRI normal, likely mutation ? - SCN1B -SCN2A? - GABR2 6. Tassinari syndrome characteristic EEG true is - 3 Hz SWD on eye closure - 4-6 Hz SWD on hyperventilation 7. 9yr old child with absence sz and limb jerks during absences Which of EEG is not possible -Normal background - 3 Hz spike and wave discharges on hyperventilation -3 Hz spike and wave discharges on photic stimulation  INI SS Pediatric Neurology July 2023 session recall 8.All of following AED act on calcium ch except - Levera -Carbamazepine - Gabapentin 9.Prolonged TPN ,abnormal gait ,parkinsonian like features with -Manganese toxicity -Vit E deficiency? -Copper deficiency - … deficiency 10. 14 yr girl with dysarthria and dystonia and T2 MRI image showing Caudate and putamen hyperintensity - Wilson’s disease - PKAN - PLAg2 neuro degeneration 11 Progressive myoclonic epilepsy with pas positive inclusions in muscle biopsy ( Lafora body disease) Mutation in gene -EPM2B -Cystatin B -EPM2A - Neu1 12. Girl with altered sensorium, akinetic mutism scenario with preceding h/o fever, cough , coryza 7 days back -Encephalitis lethargica ? -ANEC - Anti GAD encephalitis  INI SS Pediatric Neurology July 2023 session recall 13. 3 yr old with fever, cough, Coryza 1 week back now with encephalopathy , MRI image with Caudate putamen involvement and death of sibling at 15 mo age s -? ANEC (RANBP 2 mutation can cause family history )ANE1 possible - Japanese encephalitis 14.Which newer AED are excreted by kidney? - Zonisamide - Lamotrigine - Gabapentin ( also, pregabalin,vigabatrin and topiramate) -Lacosamide 15. Excess daytime sleepiness with h/o always on the go and difficulty concentrating (?ADHD ) All abormal except ? -REM latency 140 min -AHI 4 - Sleep efficiency 80% - Periodic leg movement index 11 16.Narcolepsy 1 criteria all right except - CSF hypocretin 140 pg/ml - Cataplexy seen - SOREMP>2 - MSLT 97th centile and BP > 90th centile - Exogenous steroids - Cushings disease? - 27. Amyotrophic lateral sclerosis case with vomiting, hyperpigmentation, alacrimia, achalasia. Which of the following is false? -AAAS gene mutation with ALADIN protein - true - Addisonian crisis possible - true - VLCFA is hallmark 28. Sural sparing pattern electrodiagnostically is typical in - Guillian barre syndrome - B12 deficiency - Diabetic neuropathy - CMTD  INI SS Pediatric Neurology July 2023 session recall 29.Boy with quadriparesis , altered sensorium, MRI s/o subcortical and periventricular asymmetrical hyper intensity , spinal cord lesion D6-D12, Aqp4 MOG,OCB negative ( Here it looks like LETM) False is? - Does not fulfil criteria for DT - TOC is IV Merhyl prednisolone f/b oral steroids - Decision on subsequent Immuno modulation based on further imaging after 4-6 weeks - Diagnosis is ADEM- false 30. Cup of optic disc is lost in - Primary optic atrophy - Secondary optic atrophy - Papilledema 31.RAPD ( Relative afferent pupillary defect ) due to lesion in - Impaired signalling to Edingar westpal nucleus - Impaired signalling to Lateral geniculate body - Early occulomotor palsy 32.Below MRI image in ? MCA territory Due to -Venous infarct -Lacunar infarct -Hypoperfusion iinjury -Watershed infarcts 33.Girl with stroke causing left hemiparesis with more weakness of left arm and face with dysgraphia and impaired 2 point discrimination , due to ? - Rt ACA ( has more LL involved) - ACA-MCA watershed area infarct ( has only limb paresis ,not face )  INI SS Pediatric Neurology July 2023 session recall - Rt internal capsule lesion ( both upper and lower limbs dually involved) - Rt MCA (UL more involved than LL) 34.Visual hallucination is side effect of - Itraconazole - Caspofungin -Amphoterecin B - Voriconazole 35. DSS with oliguria on day 4 , evidence based indication to start RRT is - Fluid overload > 15% - Rise in creat > 300%? - pH < 7.25 36.Most important regulator of alternate complement pathway is - Protectin - Propierdin - Factor H - 46 37. Thomsen vs Becker myotonia can be differentiated based on - Channelopathy - Calf hypertrophy - myotonia - Early childhood onset in Thomsen 38. 9yr old with Limb girdle weakness, toe walking , scapular winging - Sarcoglycanopathy - Calpainopathy  INI SS Pediatric Neurology July 2023 session recall 39. Male with tuberous sclerosis , true is - Cardiac Rhabdomyomas regress with age - Leiyomyomatosis is 40.?Congenital myaesthenia scenario ( fatiguable weakness with more limb girdle involvement , RNST done ,all can be present except? - Decremental response > 10% is diagnostic -Baseline Decremental can be seen in some - post exhaustion weakness and Decremental response starts in 5 min. - post exercise incremental response seen in some 41. Clinical scenario 3yr male ,with fever( 2 spikes daily) , hsm ,lymphadnopathy , normal platelet high ESR, TLC increased , diagnosis - Acute leukemia - SOJIA 42. Indomethacin works in - Cluster headache - Paroxysmal hemicrania - Cranial neuralgia 43.All are demyelinating except - CMT 1 - CMT 2 - CMT 4 - Tomaculous neuropathy ( hypermyelinating) 44. Polyuria with polydipsia ( Urine osm 110, serum osm 310, water deprivation test, urine osm increased to 150, and after Inj. vasopressin , urine osm increased to 160 mosm /kg. What is the best treatment  INI SS Pediatric Neurology July 2023 session recall -Oral desmopressin ? -Nasal desmopressin -Oral Thiazide -Water restriction 45. Incidental Hepatitis B detected pretonsillectomy, with normal LFTs , no jaundice, no hepatomegaly HbSAg and HBeAG positive with HBV DNA 20000 copies/ ml. Next step? -Wait and watch, follow up with LFTs -Start oral entecavir -Liver biopsy -Start s.c interferon 46. 4 month infant with isolated left axillary LN enlarged, AFB and gene xpert positive , next step ? - Start ATT treatment for 6 months - Take LN biopsy and send for histopathology 47.Ketogenic diet contraindicated in all except? - Fatty acid oxd defect - Porphyria - Carnitine PT def - Phosphofructokinase def 48.Clinical scenario of 12 yr girl with acute abdominal pain, vomiting, developed seizures, delusions and hallucinations, LFTs normal, likely diagnosis - SLE - Acute intermittent Porphyria?  INI SS Pediatric Neurology July 2023 session recall 49.All are seen in extravascular hemolysis except? - high LDH -high Indirect bili -high retic -hemoglobinuria 50.Vaccine related adverse effect are all true except? - Pertusis- Encephalopathy - rotavirus- intussusception - Hepatitis B – Brachial neuritis --Rubella- arthritis 51.Which of the following is used to know publication bias? - Funnel plot - Forest plot 52.30 day old with high TLC, neutrophilic leucocytosis, normal platelet count, persistent umbilical cord ,likely diagnosis ? -Leukocyte adhesion defect - Kostmann syndrome 53.Violent flinging movements of limbs , site of lesion? ( Ballsimus) - Contralateral sub thalamic nucleus 54. Commonest cause of opticochiasmatic arachnoiditis is -Naegleria - Acanthamoeba - TB meningitis  INI SS Pediatric Neurology July 2023 session recall 55.All of the following neurons relay in thalamus except - Olfactory neurons 56. Pedigree showing mitochondrial inheritance 57. 3 yr old with recurrent wheeze more than 3 times in past 3 months Best treatment is - Not needed - Start Inhaled budesunide - Give salbutamol only at each wheeze episode - Oral prednisolone in each epi 58.Round pneumonia is - Mycoplasma -Strep pneumoniae 59. M.c mutation for permanent neonatal diabetes -KCNJ11 ? -ABCC8 -Chr 6q -Ins 60.ABG kept in room air for long, late processed, changes seen are? - pH, pO2 reduce , pCO2 rises - increase pH, pO2, pCO2 61.Which of following is mitochondrial inheritance ? - OTC def -Kearns Sayre  INI SS Pediatric Neurology July 2023 session recall 62. Brown Vailetto Von layre syndrome is a ? - Tubulinopathy -Riboflavinopathy 63.HIE staging of neonate at 12 hrs life with seizures, hypotonia, lethargy, mydriasis ? -Stage 1 -Stage 2 64. All of the following reflexes disappear except ? - Moro,s reflex -Parachute reflex -Landau reflex -Tonic neck reflex 65.Developmental age of a child who has neck control, bidextrous grasp, can sit momentarily even without support but no pincer grasp - 9-12 months -6-7 months 66. Ataxia Telangiectasia , true is - Low AFP levels -Raised AFP levels 67. Which one is FGFR23 independent hypophosphatemic rickets ? -NAPI 2C defect - Tumor osteomalacia - AR ENPPI - AR DMP1  INI SS Pediatric Neurology July 2023 session recall 68.Image identify ? -Subdural hematoma -Epidural hematoma - SAH - Parenchymal bleed 69. Which one will not cause euvolemic hyponatremia ? - Post op -Cerebral salt wasting syndrome - Hypothyroidism - Adrenal insufficiency 70. Choice of fluid for resuscitation after initial bolus in severe DKA with K 4.5, Na 124, glucose 350, pH 6.9? -NS with 40meq/l KCL -NS with 20ml/l KCL - half NS with 20meq/l KCL - 5% dextrose 71. Neonatal resuscitation increased to 100% FIO2 after ? -30s -60s -90s -120s 72. Unusually intense eye gaze seen in ? - Rett syndrome - Fragile X syndrome - Creatine deficiency  INI SS Pediatric Neurology July 2023 session recall 73. Paradoxical reaction after TB is usually seen after? - 2-4 weeks of ATT - 8-10 weeks of ATT - 6 months of ATT - Stopping ATT 74. Comparison of proportion bêtween 2 population done by - Chi square - Kruskal wall is - Student t test - Mc Neman test