Q1. A 73-year-old man presented to the emergency room with slurred speech, vertigo, and incoordination. Exam showed dysarthria, left beating nystagmus, decreased sensation in the left face and right trunk/limbs, and left upper extremity ataxia. Head CT was normal but Brain MRI revealed a medullary infarct. On hospital day 2 he had to be transferred to the ICU for repeated episodes of prolonged central apneas during sleep.
Which structures may have been damaged to result in this complication?
- The dorsal and ventral respiratory groups
- The raphe nuclei
- The ascending reticular activating system
- The chemoreceptor trigger zone
Correct Option A: The dorsal and ventral respiratory groups
Q2. You are performing a lumbar puncture in a 34-year-old woman who is being evaluated for possible demyelinating disease. She asks you how much fluid you will be collecting and how long it will take for her body to replace the fluid that has been removed. What should you say is the approximate rate of cerebrospinal fluid formation in adults?
- 0.10 mL per minute
- 0.35 mL per minute
- 0.50 mL per minute
- 0.75 mL per minute
Correct Option B: 0.35 mL per minute
Q3. Spasticity, a velocity-dependent increase in muscle tone, is a complication of many neurological disorders affecting the brain and/or spinal cord. Baclofen is often used to alleviate spasticity symptoms in neurological disease. Which of the following options best describes its mechanism of action?
- Alpha-2 adrenergic agonist that reduces the release of excitatory neurotransmitters
- Gamma-aminobutiric acid (GABA)-B receptor agonist
- Gamma-aminobutyric acid (GABA)-A receptor agonist
- Blocks calcium release from the sarcoplasmic reticulum in skeletal muscle cells
Correct Option B: Gamma-aminobutiric acid (GABA)-B receptor agonist
Q4. A 55-year-old woman with a history of rheumatic heart disease and atrial fibrillation wakes from sleep at 6am with right sided weakness and aphasia. She had recently missed a few days of her warfarin. She is assessed in the emergency room at 7am, and her presentation is consistent with acute stroke with an NIHSS of 21. A non-contrast head CT shows no acute intracranial blood. Her INR is 1.8, which makes her ineligible for IV tPA. CT angiography shows a large vessel occlusion in the M1 segment of her left middle cerebral artery. You clarify with her husband that she was asymptomatic when she went to bed at 10pm the night prior.
What further investigation is indicated to guide emergent management?
- Carotid Duplex ultrasound
- CT perfusion scan
- Echocardiography
- MRI brain with and without contrast
Correct Option B: CT perfusion scan
Q5. A 17-year-old boy presents with slow and limited eye movements. His past medical history is notable for sensorineural hearing loss as a child status post cochlear implant. A detailed ophthalmological examination shows mild bilateral external ophthalmoplegia with pigmentary retinopathy in both eyes. A lumbar puncture is performed, which shows increased CSF protein with 0 cells and a normal glucose level. Which of the following would be the most important next step in this patient’s evaluation?
- Tensilon testing
- 3-hour glucose tolerance test
- TSH and free T4 levels
- Cardiac evaluation
Correct Option D: Cardiac evaluation
Q6. Which of the following statements regarding single fiber electromyography (SFEMG) in the diagnosis of suspected autoimmune myasthenia gravis (MG) is most accurate?
- It is the most sensitive diagnostic test
- It is the most specific diagnostic test
- It is the most sensitive and the most specific diagnostic test
- It is neither a sensitive nor a specific diagnostic test
Correct Option A: It is the most sensitive diagnostic test
Q7. 50-year-old man with trisomy 21 is brought to your clinic by his caretaker/guardian for one year of forgetfulness and more disorganized behavior. After a thorough evaluation you diagnose him with early onset Alzheimer’s disease. His caretaker questions the diagnosis as she feels that he is “too young” to get Alzheimer’s disease. In educating the caretaker/guardian on why this patient is at risk for early onset Alzheimer’s disease you should plan to mention which of the following?
- The extra copy of chromosome 21 results in an extra copy of the valosin-containing protein gene
- The extra copy of chromosome 21 results in an extra copy of the prion protein gene
- The extra copy of chromosome 21 results in an extra copy of the presenilin 1 gene
- You suspect a history of abuse given that the patient’s early onset Alzheimer’s disease suggests repetitive head trauma as a child and/or young adult
- The extra copy of chromosome 21 results in an extra copy of the amyloid precursor protein gene
Correct Option E: The extra copy of chromosome 21 results in an extra copy of the amyloid precursor protein gene
Q8. A medical student in the beginning of 3rd year comes to your office with a complaint of excessive tiredness. She is having trouble concentrating and finds herself dozing off in lectures and during morning rounds. She has always been a “night owl”. In college, she took all afternoon classes so she could sleep in. She would go to bed at 2 AM and get up at 10 or 11 AM and had no trouble falling asleep or staying awake during the day. In the first 2 years of medical school she skipped all morning lectures. However, as a third year she has to be in the hospital no later than 7 AM to pre-round on her patients. She tries to go to bed at 9:00 PM, but lies awake in bed until the middle of the night despite being “exhausted.” She has trouble getting up at 5:30 AM when her alarm goes off. On her days off, she goes to sleep when she’s tired, usually “pretty late,” falls asleep easily and then sleeps in, sometimes until after noon. Her partner says she does not snore or wake up gasping. She has not experienced any episodes of muscle weakness or paralysis. She admits that she is stressed but not more than usual. She drinks socially but denies any illicit drug use. She drinks several cups of coffee throughout the morning but not after 2 PM.
What is the most likely diagnosis?
- Obstructive sleep apnea
- Sleep onset Insomnia
- Behaviorally-induced insufficient sleep syndrome
- Delayed Sleep Phase Disorder
- Narcolepsy
Correct Option D: Delayed Sleep Phase Disorder
Q9. A 45-year-old woman with a 60 pack-year smoking history presents with signs and symptoms of pancerebellar degeneration. Review of systems include malaise and 20 pounds of unintentional weight loss. There is a family history of breast cancer. You suspect that the patient has paraneoplastic cerebellar degeneration (PCD) after ruling out other etiologies of cerebellar ataxia. She is treated empirically with corticosteroids while awaiting results of her autoantibody testing without any response. Her antibody panel on serum and CSF returns positive for Purkinje cell cytoplasmic antibody type 1 (PCA-1)-IgG (or anti-Yo). A thorough screen including body CT, whole body PET, mammography, breast MRI, and transvaginal ultrasound fails to identify a malignancy.
Which of the following statements most accurately reflects accepted recommendations for future cancer screening in this individual?
- Given the strong suspicion for an underlying malignancy the patient should continue to undergo a repeat screen, identical or comparable to her original screen, every 6 months for at least 3-4 years
- Given that her initial screen was negative there is no need for repeat screening for an occult malignancy and this should be treated as a primary autoimmune disorder
- The lack of an occult malignancy makes it more likely that this is not paraneoplastic, and alternative etiologies should be pursued
- Additional screening will add too many delays and the patient should be referred to a surgeon to undergo an exploratory laparotomy given your strong concern for an underlying ovarian cancer
Correct Option A: Given the strong suspicion for an underlying malignancy the patient should continue to undergo a repeat screen, identical or comparable to her original screen, every 6 months for at least 3-4 years
Q10. A 48-year-old man reports a 7-year history of progressive asymmetric hearing loss without any additional neurologic deficits by history or examination. When a 256 Hz vibrating tuning fork is placed at the vertex of the head, the patient reports hearing it better in his right ear. When each ear is tested separately, the sound from air conduction is perceived as louder than bone conduction on both sides. Which of the following lesions should be suspected?
- Brainstem glioma
- Acoustic neuroma on the right
- Acoustic neuroma on the left
- A chronic stroke in the distribution of the anterior inferior cerebellar artery
Correct Options C: Acoustic neuroma on the left
Q11. A 58 year male with AML, who completed induction therapy with standard dose cytarabine and idarubicin is admitted with c/o fever (38.6 degrees C). CBC demonstrates a leukocyte count of 1100/μL and an absolute neutrophil count of 90/μL. He reports a stiff neck, headache and develops nausea. What is the minimum platelet count you would require in this patient to proceed with a diagnostic lumbar puncture?
- ≤ 10,000/μL
- ≤ 20,000/μL
- ≤ 50,000/μL
- ≤ 100,000/μL
Correct Option C : ≤ 50,000/μL
Q12. A 35-year-old heroin addict is brought to the emergency room by a friend with severe muscle rigidity and altered mental status. The friend tells you that when the patient first started to feel ill and before he was encephalopathic, he commented on the fact that he had just been forced to switch to a new dealer. Laboratory evaluation shows lactic acidosis and myoglobinuria. You suspect strychnine poisoning. The neurotoxicity of strychnine is secondary to its effects on which of the following targets?
- Metabotropic glycine receptor
- Ionotropic glycine receptor
- Nicotinic acetylcholine receptor
- GABA-A receptor
- GABA-B receptor
Correct Option B: Ionotropic glycine receptor
Q13. A 25-year-old man vacationing in Hawaii presents to the emergency room with lower extremity numbness and weakness. That morning he had his first surfing lesson. He practiced laying prone on the board, paddling, and getting up quickly. After 2 hours, he felt a pop in his back followed by persistent back pain and paresthesias in his legs. This caused him to interrupt the lesson. He paddled back to shore but felt his legs were weak when he was walking to his hotel. Two hour later the weakness and numbness had worsened to the point that he could no longer walk and he trouble urinating. On examination, he had 0-1/5 strength in the bilateral lower extremities and a T-10 sensory level to pinprick but intact vibration and proprioception. A spine MRI showed T2 signal hyperintensity from T8 to the conus, increased signal on diffusion-weighted imaging and corresponding decreased signal on apparent diffusion coefficient mapping. Cerebrospinal fluid showed increased protein and mild elevation in red and white blood cells, with no oligoclonal bands.
Which of the following is the most likely diagnosis in this case?
- West Nile virus infection
- Multiple sclerosis
- Idiopathic transverse myelitis
- Surfer’s myelopathy
Correct Option D: Surfer’s myelopathy
Q14. A 38-year-old male with a history of recurrent nephrolithiasis presents to the emergency department with severe pain in his right flank that radiates to his groin and haematuria. He also complains of nausea and vomiting. A non-contrast CT scan confirms the presence of an 15 mm stone in the right kidney. What is the appropriate management for his condition?
- Medical management with increased fluid intake and alpha blockers
- Immediate referral for surgical intervention
- Shockwave lithotripsy (SWL)
- Ureteroscopy with laser lithotripsy
Correct Option C: Shockwave lithotripsy
Q15. A 6 year-old-boy, with a known case of sickle cell anaemia, is brought to the Hospital by his parents with the complaint of irritability, muscle weakness and pain, lethargy and palpitations. On examination, there was pallor, heart rate:102 beats /min, respiratory rate: 28/min, B.P:90/65mmHg. Laboratory findings include serum sodium:142 mmol/L, serum potassium:3.2mmol/L, serum chloride:107 mmol/L, serum Bicarbonate:15 mmol/L. Arterial blood gas shows ph:7.21. Urine analysis findings include pH 5.9, no casts, RBCs, WBCs, or protein. A diagnosis of renal tubular acidosis is suspected. Which of the following is associated with this patient's condition?
- High Anion Gap metabolic acidosis
- Normal Anion Gap metabolic acidosis
- Decreased Anion Gap metabolic acidosis
- Metabolic alkalosis
Correct Option B: Normal anion gap
Q16. A 67-year-old male undergoes coronary angiography for coronary artery disease. Twenty-four hours after the procedure, the patient complained of decreased urine output. He also has a history of chronic kidney disease. Laboratory analysis reveals an elevated creatinine level. Which of the following is the most likely cause of this patient’s current presentation?
- Contrast Nephropathy
- Acute interstitial nephritis
- Amyloidosis
- IgA nephropathy
Correct Option A: Contrast nephropathy
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