Board 2017 Past Paper PDF

Child with multiple types of seizures including myoclonic, difficult to control. On levetiracetam & lamotrigine. Investigations show high LFT. What are you going to do? a\. Add clobazam b\. Add valproic acid c\. Add carbamazepine d\. Maximize levetiracetam **Correct Answer:** d. Maximize leveti...

Child with multiple types of seizures including myoclonic, difficult to control. On levetiracetam & lamotrigine. Investigations show high LFT. What are you going to do? a\. Add clobazam b\. Add valproic acid c\. Add carbamazepine d\. Maximize levetiracetam **Correct Answer:** d. Maximize levetiracetam 2. Pt went for tonsillectomy and deteriorated after anesthesia (Central Core Disease). What to give? a\. IV Dantrolene **Correct Answer:** a. IV Dantrolene 3. 10 y old girl diagnosed as MS. Presenting with visual loss. Picture of MRI brain (showing WM lesions in area postrema and scattered in the brain "doesn't fit MS") and a picture of MRI spine showing longitudinal extensive involvement of the spinal cord. She was started on high dose steroids but didn't improve. What to do next? a\. IV rituximab b\. IV Ig c\. Plasma exchange **Correct Answer:** c. Plasma exchange 4. Pt with opsoclonus myoclonus, diagnosed with Neuroblastoma. Received ACTH, chemotherapy, and radiotherapy (Treated and improved). She presented again with opsoclonus myoclonus. What to do? a\. ACTH b\. IV Ig c\. Prednisolone **Correct Answer:** b. IV Ig 5. Pt has recurrent Staphylococcus aureus meningitis. She also has rhinorrhea. What to do next? a\. Thin cuts CT brain b\. MRI brain c\. Full immunological work up **Correct Answer:** a. Thin cuts CT brain 6. Pt with sore throat and high ASO titer. She developed chorea. Whom to consult first? a\. Infectious Disease b\. Rheumatology c\. Cardiology **Correct Answer:** c. Cardiology 7. 6 y old child diagnosed with BECTs, when to start treatment? a\. Older age group b\. Treatment is always indicated c\. If the seizures are close to each other **Correct Answer:** c. If the seizures are close to each other 8. Pt diagnosed with leukemia, was started on dexamethasone, methotrexate, and (a 3rd chemotherapy that I don't remember). She developed bilateral leg pain even with touch. What is the most probable cause? a\. Compartment pain syndrome b\. Side effect of chemotherapy **Correct Answer:** b. Side effect of chemotherapy 9. Child with macrocephaly, normal development. What to do next? a\. Measure parents\' head circumference 10. Aura of palpitation and sweating, localization? a\. Insular cortex 11. Pt with ADHD on methylphenidate. He developed tics but the parents are ok with it because he improved on the medication. What are you going to do? a\. Change the medication b\. Decrease the dose c\. Discontinue methylphenidate d\. Wean off methylphenidate and start another medication **Correct Answer:** b. Decrease the dose 12. Pt post renal transplant, developed focal seizure with automatism. Which AED to start? a\. Phenytoin b\. Carbamazepine c\. Valproic acid d\. Lamotrigine **Correct Answer:** b. Carbamazepine 13. A neonate found to have Café au lait spots, what to do next? a\. Ophthalmology exam b\. MRI brain c\. Echocardiogram d\. Check parents for the same spots **Correct Answer:** d. Check parents for the same spots 14. Neonate with myoclonus, not in clusters, normal development and examination. Has a sibling who had the same symptom but is now normal. Which of the following is correct? a\. KCNQ2 channel mutation (Benign familial neonatal seizures) b\. Na+ channel mutation **Correct Answer:** a. KCNQ2 channel mutation (Benign familial neonatal seizures) 15. Patient with episodes of one-sided headache with lacrimation that improves after indomethacin. What is the most probable diagnosis? a\. Paroxysmal hemicrania 16. A patient with inflammatory bowel disease presents with leg paresthesias and falls. On examination, the patient has impaired vibration and position sense, a positive Babinski sign bilaterally, ankle areflexia, and a positive Romberg sign. Which of the following is the most likely cause? a\. Selenium deficiency b\. Vitamin B12 deficiency c\. Vitamin E deficiency d\. Vitamin K deficiency **Correct Answer:** b. Vitamin B12 deficiency 17. A child was hit by a ball on the head. He was fine but afterward he developed headache and photophobia. Which of the following is the most probable diagnosis? a\. Concussion 18. Patient with SLE, presenting with seizure. MRI brain picture showing stroke in the parieto-occipital region. Which of the following is true? **Note:** The question is incomplete. 19. A 15-year-old female with severe abdominal pain, nausea, vomiting, tachycardia, normal LFTs, MRI normal, CSF normal, after 4 days developed mild weakness and bilateral wrist drop. What investigation to send? a\. Urine Porphobilinogen (PBG) and Aminolevulinic Acid (ALA) 20. Which of the following differentiates between acquired demyelinating neuropathy from genetic? a\. Presence of conduction block and temporal dispersion in nerve conduction studies 21. A patient with Guillain-Barré Syndrome started to improve on the 7th day and didn't receive treatment yet. He started to ambulate and is stable. What to do next? a\. Start IV Ig b\. Start steroids c\. No need for treatment currently **Correct Answer:** c. No need for treatment currently 22. A colleague asked you to prescribe for him an antipsychotic medication he is using. What should you do? **Answer:** Advise him to seek appropriate medical care and refrain from prescribing. 23. A junior resident talks to you about a senior who humiliates her in rounds. What should you do? **Answer:** Take the issue seriously and address it with appropriate supervisory staff or department leadership. 24. Patient with headache related to lack of sleep. What is the next step? a\. Advise to improve sleep hygiene 25. Patient with medication overuse headache. What is the next step? a\. Abruptly discontinue the overused medication 26. Child with history of febrile seizures. Then developed afebrile and febrile seizures sometimes on the right and sometimes on the left side. What is the most probable diagnosis? a\. Dravet syndrome (SCN1A mutation) 27. Neonate with episodes of apnea and sudden jerky movement with touch. What is the most likely diagnosis? a\. Hyperekplexia 28. Which antiepileptic drug is excreted in breast milk? a\. Ethosuximide 29. Scenario about dopa-responsive dystonia, regarding treatment? a\. Levodopa-carbidopa (Sinemet) 30. Patient with episodes of alternating weakness aggravated by crying. What is the diagnosis? a\. Moyamoya disease 31. Which of the following is a major criterion for Tuberous Sclerosis? **Answer:** Hypomelanotic macules (≥3), Angiofibromas (≥3), Shagreen patch, etc. 32. The \"second wind\" phenomenon is seen in which condition? a\. McArdle disease (Glycogen storage disease type V) 33. Myokymia is seen in: a\. Episodic ataxia type I 34. Patient with GBS who started to improve on the 7th day without treatment, now ambulatory and stable. What to do next? a\. No need for treatment currently 35. Patient with Fragile X syndrome. What test to send? a\. Methylation PCR for FMR1 gene to detect CGG repeats 36. Regarding NF1 inheritance, what are you going to tell the parents? a\. It is autosomal dominant with a 50% chance of transmission, high penetrance but variable expression 37. Regarding the use of everolimus in Tuberous Sclerosis, which of the following is correct? a\. It is an mTOR inhibitor used for treating SEGA, seizures, angiomyolipomas, etc. 38. Patient diagnosed with medulloblastoma post resection. Regarding the prognosis of her cognitive function? a\. It depends on several factors including age at diagnosis, treatment modalities, and presence of postoperative complications 39. A child is born with myelomeningocele. What is the risk of recurrence in future pregnancies? a\. 2-5% 40. A neonate was born with myelomeningocele. The mother says that her husband hit her in the 34th week of gestation. What to tell her? a\. An injury at that gestational age has nothing to do with the presentation 41. Patient with medication overuse headache. What is the next step? a\. Abruptly discontinue the overused medication 42. Patient with metachromatic leukodystrophy. What is the deficient enzyme? a\. Arylsulfatase A 43. Patient presenting with seizures, neuroimaging shows holoprosencephaly. What additional investigation is indicated? a\. Endocrine evaluation including pituitary hormones and electrolytes 44. A patient had a concussion with normal CT brain and no neurological deficit. When can he go back to playing football? a\. After a minimum of 5 days and following gradual return-to-play protocol if asymptomatic 45. Two children in the same household developed neurological symptoms. MRI brain shows multiple small round lesions. What is the most likely cause? a\. Neurocysticercosis 46. Patient with anemia, hepatosplenomegaly, and vertical gaze palsy. What is the diagnosis? a\. Niemann-Pick disease type C 47. Which of the following syndromes can be treated with marijuana (cannabidiol)? a\. Dravet syndrome 48. An 8-year-old girl had two attacks of loss of consciousness triggered by minor injury. Examination is normal. What is the diagnosis? a\. Reflex anoxic seizures 49. A patient with Guillain-Barré syndrome. What is the treatment? a\. Intravenous immunoglobulin (IV Ig) 50. A patient with behavior changes responsive to steroids. What test to order? a\. TSH and antithyroid antibodies (TPO, thyroglobulin) 51. A patient had URTI symptoms. She developed later on visual symptoms (color vision and visual acuity affected). Father is against MRI brain. You convince the father that MRI brain is needed to: a\. R/O demyelinating disease b\. R/O optic nerve compression c\. R/O tumor **Correct Answer:** a. R/O demyelinating disease 52. Scenario about Erb's or Klumpke\'s palsy. Asking about localization. Which nerve roots are involved in Erb\'s palsy? a\. C5-C6 b\. C8-T1 c\. L4-L5 d\. T2-T3 **Correct Answer:** a. C5-C6 53. Patient referred from cardiology and has congenital heart disease. Picture (Fundoscopy showing morning glory disc). What is the most probable diagnosis? a\. Williams syndrome b\. PHACE syndrome c\. CHARGE syndrome d\. Sturge-Weber syndrome **Correct Answer:** b. PHACE syndrome 54. Which of the following differentiates between acquired demyelinating neuropathy from genetic neuropathy? a\. Presence of conduction block and temporal dispersion on nerve conduction studies b\. Uniform slowing of conduction velocities c\. Sensory nerve action potentials are preserved d\. Early age of onset **Correct Answer:** a. Presence of conduction block and temporal dispersion on nerve conduction studies 55. Which of the following conditions is associated with loss of previously acquired language skills in children? a\. Landau-Kleffner syndrome b\. Childhood Absence Epilepsy c\. Rett syndrome d\. Autism Spectrum Disorder **Correct Answer:** a. Landau-Kleffner syndrome 56. A patient with anti-NMDA receptor encephalitis is most likely to have which of the following features? a\. Orofacial dyskinesias b\. Optic neuritis c\. Hemiplegia d\. Upper motor neuron signs **Correct Answer:** a. Orofacial dyskinesias 57. A patient with behavior changes responsive to steroids. What test to order? a\. TSH and antithyroid antibodies (TPO, thyroglobulin) 58. Which of the following is a major criterion for Tuberous Sclerosis? a\. Cardiac rhabdomyoma b\. Café au lait spots c\. Neurofibromas d\. Lisch nodules **Correct Answer:** a. Cardiac rhabdomyoma 59. Which of the following statements is correct regarding Duchenne and Becker muscular dystrophy? a\. Duchenne is caused by an out-of-frame mutation, Becker by an in-frame mutation b\. Both are caused by in-frame mutations c\. Both are caused by out-of-frame mutations d\. Mutations have no effect on the reading frame **Correct Answer:** a. Duchenne is caused by an out-of-frame mutation, Becker by an in-frame mutation 60. In Horner syndrome, anhidrosis occurs in lesions of which neurons? a\. First-order neurons only b\. Third-order neurons only c\. First and second-order neurons d\. All orders of neurons **Correct Answer:** c. First and second-order neurons 61. A patient with a tension-type headache. Regarding treatment, which of the following is correct? a\. Abortive therapy includes acetaminophen or NSAIDs b\. Preventive therapy is never indicated c\. Opioids are first-line therapy d\. Ergotamines are effective abortive agents **Correct Answer:** a. Abortive therapy includes acetaminophen or NSAIDs 62. A patient presents with a cerebellar tumor, underwent surgery, and post-operatively is now mute without new neurological deficits. Which of the following is correct regarding this condition? a\. It is transient and typically improves over weeks to months b\. It is permanent and unlikely to improve c\. It indicates severe cerebellar damage d\. Speech therapy is ineffective in this condition **Correct Answer:** a. It is transient and typically improves over weeks to months 63. A child is born with myelomeningocele. What is the risk of recurrence in future pregnancies? a\. Less than 1% b\. 2-5% c\. 10% d\. 25% **Correct Answer:** b. 2-5% 64. A neonate was born with myelomeningocele. The mother says that her husband hit her in the 34th week of gestation. What do you tell her? a\. An injury at that gestational age has nothing to do with the presentation b\. The injury is most likely the cause c\. Further investigations are needed to determine the cause d\. It\'s impossible to know the cause **Correct Answer:** a. An injury at that gestational age has nothing to do with the presentation 65. Which of the following is correct regarding the inheritance of Neurofibromatosis type 1 (NF1)? a\. It is autosomal dominant with high penetrance and variable expression b\. It is autosomal recessive with low penetrance c\. It is X-linked dominant with male lethality d\. It is a mitochondrial inheritance pattern **Correct Answer:** a. It is autosomal dominant with high penetrance and variable expression 66. A patient with Wilson disease. Which of the following is correct regarding this condition? a\. Low serum ceruloplasmin levels and decreased serum copper concentration b\. High serum ceruloplasmin levels and increased serum copper concentration c\. Normal ceruloplasmin levels with high urinary copper excretion d\. Wilson disease does not affect liver function **Correct Answer:** a. Low serum ceruloplasmin levels and decreased serum copper concentration 67. Most common cause of transient cerebral arteriopathy is: a\. Varicella zoster virus b\. Herpes simplex virus c\. Cytomegalovirus d\. Epstein-Barr virus **Correct Answer:** a. Varicella zoster virus 68. A patient with spinal injury post RTA. Which of the following requires PICU admission? a\. Injury above T10 b\. Injury below T12 c\. Injury confined to the coccyx d\. Injury limited to soft tissues **Correct Answer:** a. Injury above T10 69. An 11-year-old girl presents with unsteadiness, seizures, and deteriorating school performance. On examination, she is unable to look upward, has dysarthria, and is ataxic. Bone marrow analysis reveals sea-blue histiocytes and foam cells. Filipin staining shows accumulation of cholesterol in lysosomes of cultured fibroblasts. Which other condition is associated with this disease? a\. Cataplexy b\. Conductive hearing loss c\. Growth hormone deficiency d\. Renal calculi **Correct Answer:** a. Cataplexy 70. A patient with anemia, hepatosplenomegaly, and vertical gaze palsy. What is the diagnosis? a\. Niemann-Pick disease type C b\. Gaucher disease c\. Tay-Sachs disease d\. Wilson disease **Correct Answer:** a. Niemann-Pick disease type C 71. An 8-year-old girl had two attacks of abnormal movements. 1) When she was in the restaurant with her family, she was passing the food and her elbow hit the table then she was drowsy then LOC. 2) When she was playing football, her head hit the floor then she was drowsy then LOC. Examination is totally normal. What is the diagnosis? a\. Reflex anoxic seizures b\. Absence seizures c\. Vasovagal syncope d\. Temporal lobe epilepsy **Correct Answer:** a. Reflex anoxic seizures 72. A patient presents with seizures, neuroimaging shows holoprosencephaly. What is the investigation you would like to order? a\. Endocrine evaluation including pituitary hormones and electrolytes b\. Genetic testing for trisomy 21 c\. Visual evoked potentials d\. Audiological evaluation **Correct Answer:** a. Endocrine evaluation including pituitary hormones and electrolytes 73. A patient with metachromatic leukodystrophy (MLD). What is the deficient enzyme? a\. Arylsulfatase A b\. Galactocerebrosidase c\. Hexosaminidase A d\. Glucocerebrosidase **Correct Answer:** a. Arylsulfatase A 74. A patient with Guillain-Barré Syndrome (GBS). What is the treatment? a\. Intravenous immunoglobulin (IVIG) b\. High-dose steroids c\. Interferon beta d\. Methotrexate **Correct Answer:** a. Intravenous immunoglobulin (IVIG) 75. A colleague asked you to prescribe for him an antipsychotic medication he is using. What should you do? a\. Refuse and advise him to seek appropriate medical care b\. Prescribe the medication as requested c\. Provide a small supply until he can see his doctor d\. Inform hospital administration **Correct Answer:** a. Refuse and advise him to seek appropriate medical care 76. A junior resident talks to you about a senior who humiliates her in rounds. Which of the following is correct? a\. Take the issue seriously and address it with appropriate supervisory staff b\. Ignore her concerns as part of medical training c\. Advise her to confront the senior directly d\. Tell her to document incidents but take no further action **Correct Answer:** a. Take the issue seriously and address it with appropriate supervisory staff 77. A patient with medication overuse headache. What is the next step? a\. Abruptly discontinue the overused medication b\. Taper the medication slowly over months c\. Switch to a different analgesic d\. Increase the dose of the current medication **Correct Answer:** a. Abruptly discontinue the overused medication 78. A patient with hypokalemic periodic paralysis. Which of the following triggers should be avoided? a\. High carbohydrate meals b\. Low sodium intake c\. Physical rest after exercise d\. Beta-blockers **Correct Answer:** a. High carbohydrate meals 79. Which of the following best describes the \"second wind\" phenomenon seen in McArdle disease? a\. Improved exercise tolerance after a brief rest during activity b\. Immediate fatigue with minimal exertion c\. Muscle cramps that worsen with continued activity d\. Sudden onset of muscle weakness after meals **Correct Answer:** a. Improved exercise tolerance after a brief rest during activity 80. A trauma patient with right-sided cerebellar signs and cranial nerve palsy. Regarding localization, which area is likely affected? a\. Right midbrain b\. Left pons c\. Right medulla d\. Left cerebellar hemisphere **Correct Answer:** a. Right midbrain 81. A patient with Horner syndrome. Which of the following is a correct statement? a\. Anhidrosis occurs in lesions of first and second-order neurons b\. Miosis is due to paralysis of the levator palpebrae superioris muscle c\. Ptosis is caused by overactivity of Müller's muscle d\. The condition is always associated with neck pain **Correct Answer:** a. Anhidrosis occurs in lesions of first and second-order neurons 82. Which antiepileptic drug is excreted in breast milk? a\. Ethosuximide b\. Phenytoin c\. Valproic acid d\. Phenobarbital **Correct Answer:** a. Ethosuximide 83. A child with a known diagnosis of Juvenile Myoclonic Epilepsy (JME). Regarding treatment, which of the following is correct? a\. Treatment is usually lifelong b\. Seizures typically resolve in adolescence c\. Carbamazepine is the drug of choice d\. Valproic acid should be avoided in all patients **Correct Answer:** a. Treatment is usually lifelong 84. A child with febrile seizures. Which of the following increases the risk to develop another febrile seizure? a\. First febrile seizure occurred before 12 months of age b\. Complex febrile seizure features c\. Family history of epilepsy d\. All of the above **Correct Answer:** d. All of the above 85. Regarding NF1, a child with Café au lait spots. What should be done next? a\. Ophthalmology examination to assess for optic pathway gliomas b\. Immediate MRI of the brain c\. Genetic testing without further examination d\. No action is needed until other symptoms develop **Correct Answer:** a. Ophthalmology examination to assess for optic pathway gliomas 86. A patient with an arachnoid cyst causing intractable seizures. The neurosurgeon suggests VP shunt placement. What is the best management? a\. Surgical removal or fenestration of the cyst without VP shunt b\. Proceed with VP shunt placement c\. Medical management with antiepileptics only d\. Observation without intervention **Correct Answer:** a. Surgical removal or fenestration of the cyst without VP shunt 87. A child post-RTA with a CT brain showing a skull fracture. There are no neurological deficits. What should be done next? a\. Neurosurgery consultation b\. Discharge home with reassurance c\. Start intravenous antibiotics d\. Order an MRI brain **Correct Answer:** a. Neurosurgery consultation 88. A child was hit by a ball on the head. He was fine but afterward developed headache and photophobia. What is the most probable diagnosis? a\. Concussion b\. School phobia c\. Migraine d\. Intracranial hemorrhage **Correct Answer:** a. Concussion 89. Patient with a history of worsening verbal development and EEG showing epileptiform discharges during sleep. What is the most likely diagnosis? a\. Landau-Kleffner syndrome b\. Benign rolandic epilepsy c\. Childhood absence epilepsy d\. Lennox-Gastaut syndrome **Correct Answer:** a. Landau-Kleffner syndrome 90. A patient presenting with frequent headaches that are relieved by rest and exacerbated by lack of sleep. What is the most appropriate advice? a\. Improve sleep hygiene practices b\. Start daily NSAIDs c\. Order an MRI of the brain d\. Prescribe opioids for pain control **Correct Answer:** a. Improve sleep hygiene practices 91. A child presents with an inability to abduct the left eye and retraction of the globe upon attempted adduction. What is the most likely diagnosis? a\. Duane syndrome b\. Third nerve palsy c\. Sixth nerve palsy d\. Brown syndrome **Correct Answer:** a. Duane syndrome 92. A patient undergoing chemotherapy for leukemia develops seizures and visual symptoms. MRI shows findings consistent with PRES (Posterior Reversible Encephalopathy Syndrome). What is the most probable cause? a\. Methotrexate toxicity b\. Leukoencephalopathy due to chemotherapy c\. Intracranial hemorrhage d\. CNS infection **Correct Answer:** a. Methotrexate toxicity leading to PRES 93. A patient with unexplained behavioral changes shows improvement with steroids. Which investigation is most appropriate? a\. Anti-thyroid antibodies (TPO, thyroglobulin) and TSH levels b\. MRI of the brain c\. Lumbar puncture for CSF analysis d\. Electroencephalogram (EEG) **Correct Answer:** a. Anti-thyroid antibodies (TPO, thyroglobulin) and TSH levels 94. A patient with Dravet syndrome (SCN1A mutation). Which antiepileptic medication should be avoided? a\. Carbamazepine b\. Valproic acid c\. Clobazam d\. Topiramate **Correct Answer:** a. Carbamazepine 95. A patient with anti-NMDA receptor encephalitis did not improve with high-dose steroids. What is the next best step? a\. Start plasma exchange b\. Increase steroid dose c\. Begin antiviral therapy d\. Observe and support **Correct Answer:** a. Start plasma exchange 96. A child with esotropia and impaired vertical gaze after a trauma. What is the most likely diagnosis? a\. Basal skull fracture b\. Duane syndrome c\. Brown syndrome d\. Internuclear ophthalmoplegia **Correct Answer:** a. Basal skull fracture 97. A patient with Ohtahara syndrome shows which EEG pattern? a\. Burst suppression pattern during both sleep and wakefulness b\. Hypsarrhythmia c\. Slow spike and wave complexes d\. Fast rhythms during sleep **Correct Answer:** a. Burst suppression pattern during both sleep and wakefulness 98. A neonate has episodes of apnea and sudden jerky movements with touch. What is the most likely diagnosis? a\. Hyperekplexia b\. Infantile spasms c\. Myoclonic epilepsy d\. Benign neonatal sleep myoclonus **Correct Answer:** a. Hyperekplexia 99. Regarding the use of everolimus in Tuberous Sclerosis, which of the following is correct? a\. It is an mTOR inhibitor used for treating subependymal giant cell astrocytomas (SEGAs) b\. It is a chemotherapy agent used for all brain tumors c\. It is contraindicated in patients with renal angiomyolipomas d\. It has no effect on skin manifestations **Correct Answer:** a. It is an mTOR inhibitor used for treating subependymal giant cell astrocytomas (SEGAs) 100. Which of the following syndromes can be treated with cannabidiol (marijuana)? a\. Dravet syndrome b\. Febrile seizures c\. Childhood absence epilepsy d\. Benign rolandic epilepsy **Correct Answer:** a. Dravet syndrome 101. A trauma patient presents with right-sided cerebellar signs and cranial nerve palsy. Regarding localization, which area is likely affected? a\. Right cerebellum b\. Left cerebellum c\. Right midbrain d\. Left medulla **Correct Answer:** c. Right midbrain 102. Regarding patient identification in the hospital, which is the correct method? a\. Use only the patient\'s name b\. Use the medical record number (MRN) c\. Use both name and MRN d\. Use room number **Correct Answer:** c. Use both name and MRN 103. An 8-year-old girl had two attacks of abnormal movements: 104. When she was in the restaurant with her family, she was passing the food and her elbow hit the table then she was drowsy then LOC. 105. When she was playing football, her head hit the floor then she was drowsy then LOC. Examination is totally normal. What is the diagnosis? a\. Reflex anoxic seizures b\. Absence seizures c\. Vasovagal syncope d\. Temporal lobe epilepsy **Correct Answer:** a. Reflex anoxic seizures 104. An infant presents with seizures and an EEG showing burst-suppression pattern during both sleep and wakefulness. What is the most likely diagnosis? a\. Ohtahara syndrome (Early Infantile Epileptic Encephalopathy) b\. West syndrome c\. Lennox-Gastaut syndrome d\. Dravet syndrome **Correct Answer:** a. Ohtahara syndrome (Early Infantile Epileptic Encephalopathy) 105. A previously normal 18-month-old girl presents with developmental regression, microcephaly, and stereotypic hand-wringing movements. What is the most likely diagnosis? a\. Rett syndrome b\. Angelman syndrome c\. Autism spectrum disorder d\. Landau-Kleffner syndrome **Correct Answer:** a. Rett syndrome 106. A patient has a large brain abscess on MRI. Lumbar puncture was done and it was negative. What to do next? a\. PPD test b\. Aspiration and culture/sensitivity c\. Start broad-spectrum antibiotics empirically d\. Repeat lumbar puncture **Correct Answer:** b. Aspiration and culture/sensitivity 107. A child diagnosed with dopa-responsive dystonia. What is the treatment? a\. Levodopa-carbidopa (Sinemet) b\. Baclofen c\. Botulinum toxin injections d\. Anticholinergic medications **Correct Answer:** a. Levodopa-carbidopa (Sinemet) 108. Which of the following is associated with dopa-responsive dystonia (DYT5)? a\. GTP cyclohydrolase 1 gene mutation b\. Torsin A gene mutation c\. DYT1 dystonia d\. Wilson disease **Correct Answer:** a. GTP cyclohydrolase 1 gene mutation 109. A patient had a minor head injury during sports, with normal neurological examination and normal CT scan. When can he go back to playing football? a\. Immediately b\. After 24 hours c\. After being symptom-free for at least 5 days and following a gradual return-to-play protocol d\. After 3 months **Correct Answer:** c. After being symptom-free for at least 5 days and following a gradual return-to-play protocol 110. A child post-RTA with a CT brain showing a skull fracture. What to do next? a\. Neurosurgery consultation b\. Discharge home with reassurance c\. Start intravenous antibiotics d\. Order an MRI brain **Correct Answer:** a. Neurosurgery consultation 111. A child diagnosed with metachromatic leukodystrophy (MLD). What is the deficient enzyme? a\. Arylsulfatase A b\. Galactocerebrosidase c\. Hexosaminidase A d\. Glucocerebrosidase **Correct Answer:** a. Arylsulfatase A 112. A child presents with seizures; neuroimaging shows holoprosencephaly. What is the investigation you would like to order? a\. Endocrine evaluation including pituitary hormones and electrolytes b\. Genetic testing for trisomy 21 c\. Visual evoked potentials d\. Audiological evaluation **Correct Answer:** a. Endocrine evaluation including pituitary hormones and electrolytes 113. A junior resident talks to you about a senior who humiliates her in rounds. Which of the following is correct? a\. Advise her to confront the senior directly b\. Take the issue seriously and address it with appropriate supervisory staff or department leadership c\. Ignore her concerns as part of medical training d\. Tell her to document incidents but take no further action **Correct Answer:** b. Take the issue seriously and address it with appropriate supervisory staff or department leadership 114. A colleague asked you to prescribe for him an antipsychotic medication he is using. What should you do? a\. Prescribe the medication as requested b\. Refuse and advise him to seek appropriate medical care c\. Provide a small supply until he can see his doctor d\. Inform hospital administration **Correct Answer:** b. Refuse and advise him to seek appropriate medical care 115. A patient with tension-type headaches. Regarding treatment, which of the following is correct? a\. Preventive therapy includes amitriptyline b\. Opioids are first-line therapy c\. Ergotamines are effective abortive agents d\. Preventive therapy is not indicated **Correct Answer:** a. Preventive therapy includes amitriptyline 116. A patient reports frequent headaches that worsen with lack of sleep. What is the next step? a\. Advise to improve sleep hygiene b\. Start prophylactic medication c\. Order an MRI brain d\. Prescribe sedative medications **Correct Answer:** a. Advise to improve sleep hygiene 117. A patient with medication overuse headache. What is the next step? a\. Abruptly discontinue the overused medication b\. Taper the medication slowly over months c\. Switch to a different analgesic d\. Increase the dose of the current medication **Correct Answer:** a. Abruptly discontinue the overused medication 118. A child diagnosed with medulloblastoma underwent resection. Regarding the prognosis of her cognitive function: a\. It depends on several factors including age, treatment modalities, and postoperative complications b\. All patients will have cognitive impairment c\. Cognitive impairment is rare in these patients d\. Cognitive function is solely determined by tumor histology **Correct Answer:** a. It depends on several factors including age, treatment modalities, and postoperative complications 119. A child in the PICU post neurosurgery is extubated but not talking, with no new neurological deficits. Which of the following is correct regarding this condition? a\. It is a transient condition known as cerebellar mutism b\. The speech loss is permanent c\. The patient likely has a new neurological deficit d\. Speech therapy is ineffective in this condition **Correct Answer:** a. It is a transient condition known as cerebellar mutism 120. A child is born with myelomeningocele. What is the risk of recurrence in future pregnancies? a\. Less than 1% b\. 2-5% c\. 10% d\. 25% **Correct Answer:** b. 2-5% 121. A neonate was born with myelomeningocele. The mother says that her husband hit her in the 34th week of gestation. What do you tell her? a\. An injury at that gestational age has nothing to do with the presentation b\. The injury is most likely the cause c\. Further investigations are needed to determine the cause d\. It\'s impossible to know the cause **Correct Answer:** a. An injury at that gestational age has nothing to do with the presentation 122. A child presents with headaches and MRI shows cerebellar tonsillar herniation 6 mm below the foramen magnum. What type of Chiari malformation is this? a\. Chiari I malformation b\. Chiari II malformation c\. Chiari III malformation d\. Chiari IV malformation **Correct Answer:** a. Chiari I malformation 123. MRI spine shows an extensive lesion involving the cervicothoracic area. What would you expect to find on examination? a\. Weakness in upper limbs and muscle atrophy in hands b\. Lower limb spasticity with preserved upper limb function c\. Sensory level at the lumbar region d\. Normal neurological examination **Correct Answer:** a. Weakness in upper limbs and muscle atrophy in hands 124. A patient with Guillain-Barré Syndrome (GBS). What is the treatment? a\. Intravenous immunoglobulin (IVIG) b\. High-dose steroids c\. Interferon beta d\. Methotrexate **Correct Answer:** a. Intravenous immunoglobulin (IVIG) 125. A 15-year-old patient presents with cognitive decline, myoclonic jerks, and EEG shows periodic complexes. Which investigation will help confirm the diagnosis? a\. Elevated anti-measles IgG antibodies in CSF b\. Serum anti-HSV antibodies c\. MRI showing demyelination d\. Nerve conduction studies **Correct Answer:** a. Elevated anti-measles IgG antibodies in CSF 126. A 15-year-old patient has been having academic difficulties. His mother says he has not been acting like himself. She has also seen quick jerking movements. EEG shows background slowing and generalized periodic epileptiform discharges every 4 seconds. CSF analysis shows oligoclonal bands. Which of the following is least likely to be found in this condition? a\. Cowdry B bodies b\. Demyelination and gliosis c\. Measles antibodies in the CSF d\. Progression to dementia **Correct Answer:** a. Cowdry B bodies 127. A patient with Duchenne muscular dystrophy (DMD). Regarding the genetic mutation, which statement is correct? a\. DMD is caused by an out-of-frame mutation in the dystrophin gene b\. DMD is caused by an in-frame mutation in the dystrophin gene c\. The reading frame is not affected in DMD d\. Becker muscular dystrophy has an out-of-frame mutation **Correct Answer:** a. DMD is caused by an out-of-frame mutation in the dystrophin gene 128. In Horner syndrome, anhidrosis occurs in lesions involving which neurons? a\. First and second-order neurons b\. Third-order neurons only c\. Lower motor neurons d\. Peripheral sensory neurons **Correct Answer:** a. First and second-order neurons 129. A neonate has episodes of apnea and sudden jerky movement with touch. What is the most likely diagnosis? a\. Hyperekplexia b\. Infantile spasms c\. Myoclonic epilepsy d\. Benign neonatal sleep myoclonus **Correct Answer:** a. Hyperekplexia 130. Which of the following syndromes can be treated with cannabidiol (CBD)? a\. Dravet syndrome b\. Febrile seizures c\. Childhood absence epilepsy d\. Benign rolandic epilepsy **Correct Answer:** a. Dravet syndrome **Your kind prayers** **Thank you to everyone who collaborated to gather the questions.**