Pediatric Neurology Quiz July 2023
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Questions and Answers

Which of these conditions is known for causing generalized tremulousness?

  • Wilson’s disease
  • Pyridoxine dependency (correct)
  • Rabies
  • DOK7
  • What is the false statement regarding neuroinfections?

  • Rabies localizes in the anterior horn cell
  • NCC is always detected on MRI
  • NCC is more common than TB for infratentorial location (correct)
  • TB has lipid peak in MRS
  • In which condition would you expect the EEG findings to show 3 Hz SWD on eye closure?

  • Severe myoclonic epilepsy
  • Absence epilepsy
  • Tassinari syndrome (correct)
  • Lafora body disease
  • Which of the following AEDs does not act on calcium channels?

    <p>Levetiracetam</p> Signup and view all the answers

    Which of the following mutations is associated with progressive myoclonic epilepsy in Lafora body disease?

    <p>EPM2A</p> Signup and view all the answers

    What abnormal sleep-related characteristic is least likely to be associated with excessive daytime sleepiness and ADHD?

    <p>REM latency 140 min</p> Signup and view all the answers

    Which feature is not typical for narcolepsy type 1 criteria?

    <p>Exogenous steroids</p> Signup and view all the answers

    In a 3-year-old child with encephalopathy following a viral infection, what is the likely condition associated with caudate putamen involvement?

    <p>ANEC</p> Signup and view all the answers

    What is a true statement regarding cardiac rhabdomyomas in male patients with tuberous sclerosis?

    <p>Cardiac rhabdomyomas regress with age</p> Signup and view all the answers

    In the context of congenital myasthenia, which factor is NOT diagnostic?

    <p>Decremental response greater than 10%</p> Signup and view all the answers

    In the case of a 3-year-old male with fever, hepatosplenomegaly, and lymphadenopathy, which diagnosis is likely?

    <p>Acute leukemia</p> Signup and view all the answers

    What condition is Indomethacin NOT typically used for?

    <p>Cervicogenic headache</p> Signup and view all the answers

    Which diagnosis is indicated by a urine osmolarity response to vasopressin that is less than expected?

    <p>Nephrogenic diabetes insipidus</p> Signup and view all the answers

    In the case of incidental Hepatitis B detected pretonsillectomy, which is the best next step?

    <p>Watch and follow up with LFTs</p> Signup and view all the answers

    What is the likely next step for a patient with positive AFB and gene xpert, and isolated left axillary lymphadenopathy?

    <p>Start ATT treatment immediately</p> Signup and view all the answers

    Which of the following adverse effects is NOT related to vaccines?

    <p>Measles - Anaphylaxis</p> Signup and view all the answers

    Which statement regarding Amyotrophic Lateral Sclerosis is false?

    <p>VLCFA is a hallmark.</p> Signup and view all the answers

    In the case of a boy with quadriparesis and altered sensorium, which statement is false?

    <p>Diagnosis is ADEM.</p> Signup and view all the answers

    The loss of cup of optic disc is associated with which condition?

    <p>Papilledema.</p> Signup and view all the answers

    Which condition would NOT typically cause a relative afferent pupillary defect (RAPD)?

    <p>Retinal detachment.</p> Signup and view all the answers

    Below imaging reveals an issue in the MCA territory. Which cause is incorrect?

    <p>Hypoxia.</p> Signup and view all the answers

    In a case of a girl with stroke-related left hemiparesis, which explanation is misleading?

    <p>Rt ACA has more LL involved.</p> Signup and view all the answers

    Visual hallucination is a side effect of which medication?

    <p>Voriconazole.</p> Signup and view all the answers

    For dialysis in DSS with oliguria on day 4, which indication is incorrect?

    <p>Low potassium levels.</p> Signup and view all the answers

    Which condition is characterized by a mutation in the KCNJ11 or ABCC8 genes related to permanent neonatal diabetes?

    <p>M.c mutation</p> Signup and view all the answers

    What would be the best treatment for a 3-year-old with recurrent wheezing more than three times in the past 3 months?

    <p>Start inhaled budesonide</p> Signup and view all the answers

    Which of the following reflexes does not typically disappear with age?

    <p>Parachute reflex</p> Signup and view all the answers

    Which of these conditions is NOT associated with euvolemic hyponatremia?

    <p>Postoperative state</p> Signup and view all the answers

    At what time after initiating neonatal resuscitation should 100% FIO2 be increased?

    <p>60 seconds</p> Signup and view all the answers

    Which condition is characterized by a mitochondrial inheritance pattern?

    <p>Kearns Sayre syndrome</p> Signup and view all the answers

    Which of the following is a known common cause of opticochiasmatic arachnoiditis?

    <p>TB meningitis</p> Signup and view all the answers

    What condition is described by violent flinging movements of limbs, indicating a lesion in which part of the brain?

    <p>Contralateral subthalamic nucleus</p> Signup and view all the answers

    Study Notes

    Spasmus Nutans

    • Not seen in INI SS Pediatric Neurology July 2023 session recall
    • Triad includes nystagmus, head nodding, and torticollis

    Rabies Localization

    • Localizes in the brainstem

    CMS Worsened with Pyridoxine

    • Suggests DOK7 mutation

    NCC vs TB

    • NCC is more common than TB for infratenrorial location is false

    Benign Infantile Convulsion

    • Likely mutation for SCN1B
    • No family history, resolution by 3 years, and normal MRI

    Tassinari Syndrome

    • Characteristic EEG findings include 3Hz SWD on eye closure and 4-6Hz SWD on hyperventilation

    9yr old Absence Seizures with Limb Jerks

    • Normal background EEG is possible
    • 3Hz spike and wave discharges on hyperventilation is possible
    • 3Hz spike and wave discharges on photic stimulation is possible

    AEDs Acting on Calcium Channels

    • Levera acts on calcium channels
    • Carbamazepine, gabapentin do not act on calcium channels

    Prolonged TPN

    • Abnormal gait and Parkinsonian-like features can indicate manganese toxicity

    14yr Girl with Dysarthria and Dystonia

    • T2 MRI showing caudate and putamen hyperintensity suggests Wilson’s disease

    Progressive Myoclonic Epilepsy with PAS Positive Inclusions

    • Lafora body disease mutation is in EPM2B

    Altered Sensorium, Akinetic Mutism

    • H/o fever, cough, and coryza suggests ANEC

    3yr Old with Encephalopathy and Family History

    • Encephalitis lethargica with RANBP2 mutation can cause family history

    Newer AEDs Excreted by Kidney

    • Zonisamide, lamotrigine, gabapentin, pregabalin, vigabatrin, and topiramate are excreted by the kidney

    Excess Daytime Sleepiness

    • REM latency of 140 min is abnormal
    • AHI of 4 is abnormal
    • Sleep efficiency of 80% is abnormal
    • Periodic leg movement index of 11 is abnormal

    Narcolepsy

    • CSF hypocretin of 140 pg/ml is abnormal
    • Cataplexy is a diagnostic criteria
    • SOREMP>2 is a diagnostic criteria
    • MSLT 97th centile and BP > 90th centile is a diagnostic criteria

    Amyotrophic Lateral Sclerosis

    • AAAS gene mutation with ALADIN protein is true
    • Addisonian crisis is possible
    • VLCFA is a hallmark

    Sural Sparing Pattern

    • Sural sparing pattern is typical in diabetic neuropathy

    Boy with Quadriparesis and MRI Findings

    • Does not fulfil criteria for DT
    • TOC is IV methylprednisolone followed by oral steroids
    • Decision on subsequent immunomodulation is based on further imaging after 4-6 weeks
    • Diagnosis is NOT ADEM

    Cup of Optic Disc

    • Cup of the optic disc is lost in primary and secondary optic atrophy

    RAPD

    • RAPD is due to impaired signaling to the Edinger-Westphal nucleus

    MRI Image

    • The provided MRI image is in MCA territory

    Girl with Stroke

    • Stroke causing left hemiparesis with more weakness of the left arm and face, dysgraphia, and impaired 2-point discrimination suggests right ACA (has more LL involvement)

    Visual Hallucination

    • Visual hallucination is a side effect of voriconazole

    DSS with Oliguria

    • Evidence-based indication to start RRT is pH < 7.25

    Regulator of Alternate Complement Pathway

    • Factor H is the most important regulator of the alternative complement pathway

    Thomsen vs Becker Myotonia

    • Thomsen myotonia has early childhood onset, Becker myotonia does not

    9yr Old with Limb-Girdle Weakness

    • Sarcoglycanopathy and calpainopathy can cause limb-girdle weakness, toe walking, and scapular winging

    Male with Tuberous Sclerosis

    • Cardiac rhabdomyomas regress with age
    • Leiyomyomatosis is a hallmark of tuberous sclerosis

    Congenital Myasthenia

    • Decremental response > 10% is diagnostic
    • Baseline decrement can be seen in some
    • Post-exhaustion weakness and decremental response starts in 5 min
    • Post-exercise incremental response is seen in some patients

    3yr Male with Fever

    • The scenario suggests acute leukemia
    • SOJIA is also possible

    Indomethacin

    • Indomethacin is effective in cluster headache and paroxysmal hemicrania

    Demyelinating Diseases

    • Tomaculous neuropathy (hypermyelinating) is NOT a demyelinating disease

    Polyuria with Polydipsia

    • The best treatment is nasal desmopressin

    Incidental Hepatitis B

    • The best course of action is wait and watch and follow up with LFTs

    Isolated Left Axillary LN

    • Start ATT treatment for 6 months

    Ketogenic Diet

    • Ketogenic diet is contraindicated in fatty acid oxidation defects, porphyria, carnitine palmitoyltransferase deficiency, and phosphofructokinase deficiency

    12yr Girl with Abdominal Pain

    • Acute intermittent Porphyria is a likely diagnosis

    Extravascular Hemolysis

    • High LDH, high indirect bilirubin, high reticulocyte count, and hemoglobinuria are seen in extravascular hemolysis
    • Pertussis-encephalopathy is a known vaccine-related adverse effect

    Publication Bias

    • Funnel plot is used to identify publication bias

    30 Day Old with High TLC

    • Leukocyte adhesion defect is a likely diagnosis

    Violent Flinging Movements

    • Violent flinging movements of limbs are due to a lesion in the contralateral subthalamic nucleus

    Optic Chiasmatic Arachnoiditis

    • TB meningitis is the most common cause of opticochiasmatic arachnoiditis

    Neurons Relaying in Thalamus

    • Olfactory neurons do NOT relay in the thalamus

    Mitochondrial Inheritance

    • Mitochondrial Pedigree will show maternal transmission

    3yr Old with Recurrent Wheeze

    • Start inhaled budesonide

    Round Pneumonia

    • Strep pneumoniae is a common cause of round pneumonia

    Permanent Neonatal Diabetes

    • ABCC8 mutation is the most common for permanent neonatal diabetes

    ABG Kept in Room Air

    • Late processing of ABG will show reduced pH, pO2 and increased pCO2

    Mitochondrial Inheritance

    • Kearns Sayre syndrome is a mitochondrial inherited disease

    Brown-Vailant-Von Layre Syndrome

    • Brown-Vailant-Von Layre syndrome is a tubulinopathy

    HIE Staging

    • HIE stage 2 at 12 hrs of life with seizures, hypotonia, lethargy, and mydriasis

    Reflexes

    • Parachute reflex does not disappear

    Developmental Age

    • 6-7 months

    Ataxia Telangiectasia

    • Raised AFP levels are seen in Ataxia Telangiectasia

    FGFR23 Independent Hypophosphatemic Rickets

    • NAPI 2C defect is an FGFR23 independent hypophosphatemic rickets

    Image Identification

    • The image is a subdural hematoma

    Euvolemic Hyponatremia

    • Post-op, cerebral salt wasting syndrome, hypothyroidism, and adrenal insufficiency can cause euvolemic hyponatremia

    Fluid Choice for DKA Resuscitation

    • Half NS with 20meq/l KCL is the correct choice

    Neonatal Resuscitation

    • Increase to 100% FIO2 after 30s in neonatal resuscitation

    Unusually Intense Eye Gaze

    • Unusually intense eye gaze is seen in autism

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    Description

    Test your knowledge on pediatric neurology with this quiz covering various conditions and their characteristics, including spasmus nutans, absence seizures, and more. Dive into key concepts such as mutation implications and EEG findings. Perfect for medical students or professionals looking to refresh their understanding.

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