Board Review Edition

Here is the first ever Board Review Edition of the historical grand rounds. I took a fair bit of poetic license (most of the details are made-up), but everyone featured really had the disease in question. The correct answer will have "correct" written in white font next to it. Highlight to see.

1. One of the world's greatest ever Opera divas spent her last months locked in her Parisian apartment. On a home visit her doctor notice that she had an erythematous periorbital rash, red patches overlying her knuckles, and diffuse muscle pain. He warned her that this condition carries an increased risk of occult malignancy. Which antibody is her condition associated with?

a. anti-dsDNA
b. anti-microsomal
c. anti-Ro/SSA
d. anti-Jo correct, dermatomyositis
e. anti-Smith
f. anti-scl-70
g. pANCA

a. Leontyne Price
b. Renee Fleming
c. Birgit Nilsson
d. Maria Callas  correct

2. In 1911, an Austrian composer presented to Mt. Sinai Hospital complaining of a fever that he had for 2 weeks. He reported no other symptoms but on physical exam they noticed tender raised lesions on his palms, pale mucus membranes, splinter hemorrhages on his nails, and a low frequency murmur heard best at the lower left sternal border. How is the most likely pathogen identified when it's cultured?

a. Gram positive, catalase positive, coagulase positive cocci correct, staph aureus 
b. Gram positive, catalase positive, novobiocin resistant cocci
c. Gram positive, catalase negative, optochin resistant cocci
d. Gram positive, catalase negative, optochin sensitive cocci
e. Gram positive, catalase positive, novobiocin sensitive cocci

a. Franz Liszt
b. Gustav Mahler correct
c. Johannes Brahms
d.  Franz Schubert

3. This preeminent physicist, who became famous for his 1905 paper "On the Electrodynamics of Moving Bodies", died suddenly after an episode of intense chest pain. He had multiple cardiac risk factors included hyperlipidemia, hypertension, and smoking. An autopsy was not performed, but months prior his physician noted a large pulsating mass in his abdomen. Which of the following is not a possible cause of this event?

a. medial calcific sclerosis correct
b. Treponema Pallidum infection
c. mutation in the gene coding for collagen type III
d. defect in the fibrillin 1 gene
e. Takayasu's arteritis

a. Robert Oppenheimer
b. Richard Feynman
c. Paul Dirac
d. Albert Einstein correct

4.  The founder of the New York City Ballet and one of the greatest choreographers of the 20th century presented to NYU hospital with ataxia and new-onset dementia. Soon after he was admitted his attending physician performed a physical exam and found the patient delirious with hyperreflexia and startle myoclonus. He died four weeks later and a tissue biopsy was performed, which to the pathologist's surprise, was negative for alpha synuclein. What is the most likely cause/etiology of his disease?

a. tau protein aggregates
b. amyloid precursor protein
c. Infectious cause correct, creutzfeldt-jacob disease
d. Vitamin deficiency
e. Copper accumulation

a. Mikhail Baryshnikov
b. Rudolf Nureyev
c. George Balanchine correct
d. Vaslav Nijinski

5. This monarch of the British Empire underwent an infamous mental decline in their final years due to a series of severe psychological disturbances. However, their personal physicians noticed a constellation of other symptoms including episodes of abdominal pain, polyneuropathy, and dark red urine. There was never any documented photosensitivity or exposure to environmental toxins. This disease is characterized by an accumulation of which of the following subsrates:

a. porphobilinogen correct, acute intermittent porphyria
b. uroporphyrin
c. vWF multimers
d. protoporphyrin
e. glycine

a. King George III correct
b. King Edward VIII
c. Queen Elizabeth II
d. Diana, Princess of Wales

6. This composer, Michael's personal favorite, had a unique talent for presenting intense romanticism through a veil of elaborate civility. He suffered from chronic bronchitis and steatorrhea, and was always a very frail, delicate man. When he died at the age of 39, his autopsy report said he had TB, but the doctor admitted that the enlarged heart and abnormal lung findings pointed to a different pathology that was "unknown to medicine" at the time. He also had a sister who died of unknown causes at a young age, and he never had children despite having a female partner for over 10 years. What is the mechanism of the exotoxin produced by the most likely cause of his recurrent pulmonary infections?

a. protease that cleaves SNARE
b. activates T cells
c. over activates adenylate cyclase
d. Inactivation of 60S ribosome
e. inactivation of elongation factor EF-2 correct, pseudomonas in cystic fibrosis

a. Mozart
b. Bach
c. Chopin correct
d. Schubert