Noggins
A 14-year-old girl presents with unilateral, throbbing headaches associated with nausea and photophobia. She reports missing school during episodes and says her mother has similar headaches. What is the most appropriate first-line acute treatment?
A. Sumatriptan
B. Propranolol
C. Acetaminophen with codeine
D. Gabapentin
E. Topiramate
Sumatriptan
Triptans (like sumatriptan) are first-line acute treatments for moderate to severe migraines in adolescents. They work by constricting cerebral blood vessels and blocking pain pathways. Preventive agents (like propranolol and topiramate) are not used acutely.
A 7-year-old child presents with fever, headache, photophobia, and vomiting. On exam, he is lethargic and has a new right-sided facial droop. You suspect bacterial meningitis. What is the most appropriate next step?
A. Perform lumbar puncture immediately
B. Start IV fluids and await blood test results
C. Order head CT before lumbar puncture and start empiric antibiotics
D. Administer acetaminophen and observe
E. Delay antibiotics until LP and imaging are completed
Order head CT before lumbar puncture and start empiric antibiotics
In children with signs of increased intracranial pressure or focal neurologic deficits, such as altered mental status or cranial nerve palsies, you must obtain head imaging prior to LP. BUT crucially: DO NOT delay empiric antibiotics — start them right away after blood cultures.
A term infant is delivered after placental abruption. APGARS are 1,2,and 7 after 1, 5, and 10 minutes, respectively. Twelve hours later, the infant is observed having a seizure. What is the most likely cause of this infant's seizure?
A. Intrapartum hypoxia and ischemia
B. Hydrocephalus
C. Intracranial hemorrhage
D. Idiopathic
E. Hypoglycemia
Intrapartum hypoxia and ischemia
Intrapartum or antepartum asphyxia resulting in either global or focal brain ischemia is the most common cause of seizures in the term
A 6-year-old boy presents with progressive weakness over 3 days. He cannot walk without assistance and has absent deep tendon reflexes. He had watery diarrhea one week ago. His vital capacity is declining. CSF shows albuminocytologic dissociation. What is the best next step?
A. Start oral corticosteroids
B. Initiate intravenous immunoglobulin
C. Start high-dose antibiotics
D. Order electromyography only
E. Administer pyridostigmine
Initiate intravenous immunoglobulin
Guillain-Barré syndrome often follows viral or Campylobacter infection and presents with ascending flaccid paralysis, areflexia, and albuminocytologic dissociation in CSF (high protein, normal cells). Steroids are NOT effective in GBS.
A 10-year-old boy was diagnosed with a concussion 3 weeks ago after a fall during soccer. He now has persistent headaches, difficulty concentrating, and mood changes. What is the best next step?
A. Initiate head CT scan
B. Return to full sports activity
C. Begin amitriptyline therapy
D. Reassure and implement a gradual return-to-learn and return-to-play protocol
E. Refer for neurosurgical consultation
Reassure and implement a gradual return-to-learn and return-to-play protocol
Post-concussion syndrome is a clinical diagnosis. Imaging isn't needed unless there are red flags. Management is supportive, with a stepwise return to cognitive and physical activity. Most cases resolve with time.
A 7-year-old girl presents with fever, headache, and confusion. Exam shows neck stiffness and photophobia. MRI shows temporal lobe enhancement. What is the most likely diagnosis?
A. Bacterial meningitis
B. Brain abscess
C. Herpes simplex encephalitis
D. ADEM
E. Tuberculous meningitis
Herpes simplex encephalitis
HSV encephalitis typically affects the temporal lobes and presents with altered mental status, fever, and focal neurologic signs. Temporal lobe enhancement on MRI is a classic finding.
A 1-hour-old male infant presents with abnormal shaking and jerking movements of all four extremities and lip-smacking. He was born at 38 + 1weeks, and delivery was complicated by meconium in the amniotic fluid, variable decelerations, and a nuchal cord. His APGAR score was 3 at 1 minute and 4 at 5 minutes of life. He required mouth suctioning and stimulation for resuscitation initially. Maternal complications during pregnancy include maternal obesity and gestational diabetes controlled with diet and exercise. His serum glucose immediately after the episode is 50 mg/dL. Which of the following is the most likely cause of his current symptoms?
A. Seizures due to neonatal hypoglycemia
B. Benign myoclonic jerks of early infancy
C. Seizures due to hypoxic ischemic encephalopathy
D. Benign rolandic epilepsy
E. Neonatal abstinence syndrome
Seizures due to hypoxic ischemic encephalopathy
HIE is associated with low Apgar scores, low cord pH, neonatal seizures, and encephalopathy. Patients present with diminished spontaneous movements, respiratory or feeding difficulties, poor tone, abnormal posturing, absent primitive reflexes, or seizure activity.
A 4-year-old boy presents with progressive difficulty climbing stairs, frequent falls, toe walking, and calf enlargement. On exam, he uses his hands to push off his thighs when rising from the floor. Serum creatine kinase is markedly elevated. Genetic testing confirms a dystrophin gene mutation.
What is the most likely mode of inheritance for this condition?
A. Autosomal recessive
B. Autosomal dominant
C. Mitochondrial
D. X-linked recessive
E. X-linked dominant
X-linked recessive
Duchenne muscular dystrophy presents in early childhood with progressive weakness, especially proximal muscles, and calf pseudohypertrophy. CK is markedly elevated. It is X-linked recessive.
A 2-month-old infant presents with a bulging fontanelle, rapidly increasing head circumference, and downward deviation of the eyes (sunsetting). What is the most likely cause?
A. Craniosynostosis
B. Normal pressure hydrocephalus
C. Benign macrocephaly
D. Communicating hydrocephalus
E. Non-communicating hydrocephalus
Non-communicating hydrocephalus
Non-communicating (obstructive) hydrocephalus results from a blockage in CSF flow, often at the aqueduct or fourth ventricle. Symptoms include increased ICP signs like sunsetting eyes and a bulging fontanelle.
A newborn in the NICU is being observed for a suspected congenital infection. Blood and CSF cultures are pending. A head ultrasound reveals hydrocephalus, and a cranial CT scan shows calcifications throughout the brain. What is the most appropriate antimicrobial regimen for this patient?
A. Ampicillin and gentamicin
B. Zidovudine
C. Piperacillin/tazobactam and vancomycin
D. Pyrimethamine and sulfadiazine
E. Fluconazole
Pyrimethamine and sulfadiazine
Infants with congenital toxoplasmosis may present with chorioretinitis, cataracts, macrocephaly, and diffuse intracranial calcifications. Long-term sequelae include blindness, intellectual disability, and seizures. The preferred treatment regimen for congenital toxoplasmosis is pyrimethamine and sulfadiazine for 1 year
An 8-month-old boy is brought in for evaluation of repetitive episodes where he suddenly bends forward at the waist with arm extension, often in clusters shortly after waking. The parents describe them as "jackknife" movements occurring 5–10 times in a row. He has shown regression in motor milestones and is no longer reaching for toys as he did previously. Physical exam reveals central hypotonia and poor visual tracking. MRI of the brain is pending.
Which of the following EEG findings would be most characteristic of this condition?
A. 3-Hz spike-and-wave discharges
B. Generalized polyspike and wave pattern
C. Hypsarrhythmia
D. 14 and 6 Hz positive spikes
E. Generalized slowing with focal temporal spikes
Hypsarrhythmia
This clinical presentation is classic for infantile spasms - spasms (brief, symmetric flexion or extension movements, often in clusters), developmental delay or regression, often presents between 3–12 months of age.
The hallmark EEG finding is hypsarrhythmia — a chaotic, high-amplitude, disorganized pattern with multifocal spikes and slow waves, typically seen interictally.
A 6-month-old male is brought in for evaluation of progressive weakness. His parents report that he was able to roll over at 4 months but has since become increasingly floppy and now struggles to lift his head. He does not sit independently. On exam, he has hypotonia, areflexia, tongue fasciculations, and a bell-shaped chest. He responds to sound and light appropriately and shows no facial weakness. Genetic testing confirms a homozygous deletion of the SMN1 gene.
Which of the following best describes the underlying pathophysiology of this condition?
A. Demyelination of peripheral motor nerves
B. Mutation in dystrophin leading to sarcolemmal instability
C. Degeneration of anterior horn cells in the spinal cord
D. Autoantibodies targeting acetylcholine receptors
E. Defective mitochondrial energy production in muscle tissue
Degeneration of anterior horn cells in the spinal cord
This is Spinal Muscular Atrophy (SMA) Type 1 (Werdnig-Hoffmann disease) — the most severe infantile form. It presents with: progressive proximal muscle weakness, hypotonia and areflexia, tongue fasciculations, bell-shaped chest due to diaphragmatic breathing, preserved cognition and sensation. SMA is caused by a homozygous deletion or mutation in the SMN1 gene, leading to loss of anterior horn cells in the spinal cord and lower motor neuron degeneration.
A 9-year-old girl is evaluated for recurrent headaches. The headaches are accompanied bynausea and vomiting and are triggered by noise and fatigue. The headaches improve aftersleeping in a darkened room. She is a good student, but headaches have caused her to be absent several times. Her parent has had similar symptoms for many years.
Physical and neurological examinations are unremarkable.
What is the probable underlying pathophysiological mechanism of the patient's pain?
A. Cerebral vasoconstriction
B. Primary neuronal dysfunction
C. Activation of the trigeminal hypothalamic pathway
D. Peripheral activation or sensitization of myofascial nociceptors
Primary neuronal dysfunction
Migraine is the most common cause of childhood headaches, and they present with symptoms similar to those seen in adults. Primary neuronal dysfunction is the underlying mechanism. Analgesics are the treatment of choice. If these do not provide sufficient relief, then a trintan may be prescribed
A 10-year-old immunocompromised child presents with fever, confusion, seizures, and new-onset hemiparesis. MRI reveals ring-enhancing lesions in the basal ganglia and thalamus. What is the most likely pathogen?
A. Herpes simplex virus
B. Toxoplasma gondii
C. Epstein-Barr virus
D. Streptococcus pneumoniae
E. Enterovirus
Toxoplasma gondii
In immunocompromised patients (e.g., HIV, chemotherapy), toxoplasmosis is a common cause of focal CNS lesions. MRI shows ring-enhancing lesions, often in the basal ganglia or thalamus. HSV typically affects the temporal lobes, and EBV is more associated with CNS lymphoma. Enteroviruses cause diffuse meningoencephalitis, not focal lesions. Strep pneumo is the most common cause of bacterial meningitis in children and adults. It typically causes diffuse meningeal inflammation, not focal brain lesions; it does not cause ring-enhancing lesions.
A 6-year-old child presents with morning myoclonic jerks and occasional generalized tonic-clonic seizures. EEG shows 4–6 Hz polyspike and wave discharges. Which is the most likely diagnosis?
A. Absence epilepsy
B. Juvenile myoclonic epilepsy
C. Lennox-Gastaut syndrome
D. Benign Rolandic epilepsy
E. Febrile seizures
Juvenile myoclonic epilepsy
Juvenile myoclonic epilepsy (JME) typically presents in adolescents with morning myoclonus, often followed by generalized seizures. The EEG finding of polyspike-and-wave discharges is characteristic.
A 12-year-old previously healthy boy presents with progressive blurry vision, dysarthria, and generalized weakness over the past 48 hours. He also reports dry mouth and difficulty swallowing. Exam reveals bilateral ptosis, facial weakness, and diminished gag reflex. His pupils are sluggishly reactive to light. Reflexes are slightly decreased, but sensation and cognition are normal. One week ago, he sustained a puncture wound to his leg while mountain biking; the wound was not cleaned thoroughly and now appears swollen and tender with surrounding erythema.
Which of the following is the most appropriate next step in management?
A. Administer human botulism immune globulin (BabyBIG®)
B. Administer equine-derived botulinum antitoxin and initiate wound debridement
C. Begin high-dose IV corticosteroids
D. Start intravenous immunoglobulin (IVIG) for suspected Guillain-Barré syndrome
E. Order MRI of the brain and lumbar spine
Administer equine-derived botulinum antitoxin and initiate wound debridement
This presentation is consistent with wound botulism, which can occur after contamination of a puncture or crush wound with Clostridium botulinum spores. The bacteria produce neurotoxin locally, leading to: cranial nerve dysfunction (ptosis, dysarthria, dysphagia), descending symmetric flaccid paralysis, normal sensory exam and mental status
Treatment includes: equine-derived botulinum antitoxin (for children >1 year old), wound debridement, antibiotics (penicillin G or metronidazole). BabyBIG® is only for infant botulism, not wound or foodborne forms.