@marimphil wrote:
exudate within the pleural cavity?
Answer
2>In which of the following sites do myxopapillary ependymomas most
frequently occur?
Answer
3>A patient has long-standing severe hemolytic anemia characterized by
hypochromic cells. Electrophoresis studies demonstrate a near complete
absence of beta chains. Several years later, the patient develops
cardiac failure. Intracardiac deposition of which of the following
would be most likely to contribute to the cardiac failure?
Answer
4>Before being approved by the FDA, a chemical is tested for
carcinogenicity by examining its mutagenic effects on bacterial
cells in culture. Which of the following tests is used to make
this determination?
Answer
5.A sharp instrument passing through the superior orbital fissure
would most likely sever the
Answer
6>An elderly nursing home patient has had multiple small strokes.
On several occasions she has aspirated food, and neurological
examination reveals that her gag reflex is absent. These findings
suggest involvement of the nucleus of which of the following
cranial nerves?
Answer
7>A new antifungal medication is being tested in Phase I clinical
trials. Examination of the pharmacokinetic properties of the drug
reveals that the half-life of the drug is 6 hours. If a continuous
intravenous infusion of this drug were started on a research subject,
how long would it take to reach 75% of steady state?
Answer
8>A leukemia patient who has undergone multiple courses of chemotherapy
develops herpes simplex encephalitis. Which of the following would you
expect a CT scan of the patient's brain to show?
Answer
9>An 18-year-old male takes an employment physical examination that is
remarkable for a systolic murmur preceded by a distinct click on
auscultation. The patient has unique body features, with unusually
long legs and long, tapering fingers. An ocular examination reveals
dislocation of the lens. An abnormality of which of the following
Answer
10>A third-world patient develops muscle weakness and atrophy with
fasciculations and hyporeflexia. The patient's condition continues
to deteriorate, and his legs are eventually permanently paralyzed.
The virus that can cause these problems is usually spread by which
of the following routes?
Answer
11>Physical examination of a 45-year-old diabetic patient demonstrates a
pulsatile abdominal mass. Radiographic studies demonstrate a 10 cm
diameter aneurysm of the abdominal aorta with foci of calcification
in the walls. Which of the following is the most likely etiology
for this aneurysm?
Answer
12>A 40-year-old woman is being seen by a physician for the 10th time
this year for evaluation of vague aches and pains. An extensive prior
evaluation has excluded the possibility of serious disease. During the
interview with this patient, she makes repeated statements along the
lines of, "What I want doesn't matter. Do what you want." And, "I'm
Answer
13>A 60-year-old man suddenly becomes completely blind in one eye, and
angiography demonstrates occlusion of the central retinal artery.
Which of the following is the most likely cause of the occlusion?
Answer
14 A mailman gets a severe bite wound from a pit bull guarding
a junkyard. The wound is cleansed and he receives a booster
injection of tetanus toxoid and an injection of penicillin G.
Several days later, the wound is inflamed and purulent. The
exudate is cultured on blood agar and yields gram-negative rods.
Antibiotic sensitivity tests are pending. The most likely agent
to be isolated is
Answer
15.A 7-year-old boy is referred to a specialty clinic
because of digestive problems. He often experiences severe
abdominal cramps after eating a high fat meal. He is worked
up and diagnosed with a genetic defect resulting in a deficiency
of lipoprotein lipase. Which of the following substances would most
likely be elevated in this patient's plasma following a fatty meal?
Answer
16>A 49-year-old woman is in an automobile accident and sustains a closed
head injury. A CT scan does not show any intracranial hemorrhage, but
reveals a small tumor at the cerebellopontine angle of the brain.
Which of the following nerves is most likely to be affected by
this tumor?
Answer
17>A 47-year-old male patient presents with painful arthritis in the
right big toe and uric acid renal stones. He has been taking allopurinol
for his condition. What biochemical defect would likely be found in this
patient?
Answer
18>A 3-year-old boy is brought to the emergency department after the acute
onset of headache, vomiting, nuchal rigidity, and impaired mental status.
MRI reveals a posterior fossa tumor that fills the 4th ventricle. Surgery
is immediately started, and intraoperative consultation leads to
Answer
19>A diabetic mother gives birth to a baby who dies in the first week of life.
Autopsy reveals a severe cardiac malformation. Which of the following is
the most likely diagnosis?
Answer
20>There is a classic zoo story about a cage with three monkeys in it. The
largest monkey steals the middle-sized monkey's banana. The middle-sized
monkey then screams with rage, hits the smallest monkey on the head, and
then steals his banana. The middle-sized monkey is using which of the
following mechanisms of defense?
Answer
21>A 10-year-old boy has a small tumor in the wall of the right lateral
ventricle. A biopsy of this tumor is consistent with subependymal giant
cell astrocytoma. Which of the following lesions may also be present in
this patient?
Answer
22>Physical examination of a 45-year-old diabetic patient demonstrates a
pulsatile abdominal mass. Radiographic studies demonstrate a 10-cm
diameter aneurysm of the abdominal aorta with foci of calcification
in the walls. Which of the following is the most likely etiology for
the aneurysm?
Answer
23>A 19-year-old female, who recently moved from her family's home in another
state, is hospitalized for attempting suicide by taking an overdose of
antidepressant medications. On the third day of her hospital stay, she
insists, under threat of a lawsuit, that her medications be stopped and
that she be discharged from the hospital so she "can go home and finish
the job." Her sensorium is clear. Her physician should
Answer
24>Which of the following could prevent an allergen from reacting with a
specific IgE molecule present on the mast cell membrane?
Answer
25>During a fight, a 32-year-old man is hit on the back of the neck with a
chair. A CT scan reveals a bony fragment that penetrated the lateral
portion of the dorsal columns. Which of the following functions would
most likely be affected by a lesion at this site?
Answer
26>A 40-year-old, formerly obese woman presents to her physician. She was
very proud of having lost 80 lbs. during the previous 2 years, but now
noticed that her "hair is falling out." On questioning, she reports
having followed a strict fat-free diet. Her alopecia is probably related
to a deficiency of which of the following vitamins?
Answer
27>A 50-year-old woman presents with a 5-year history of headaches,
generalized tonic-clonic seizures, and bilateral leg weakness. Skull
films reveal hyperostosis of the calvarium. Biopsy of the responsible
lesion shows a whorling pattern of the cells. Which of the following
is the most likely diagnosis?
Answer
28>A 53-year-old man develops acute, excruciating chest pain that radiates
to his back. En route to the emergency room, he becomes unresponsive,
and is pulseless on arrival. Resuscitation attempts are unsuccessful.
Autopsy reveals massive hemoperitoneum due to a ruptured aortic
dissection. There is a jagged intimal tear in the ascending aorta,
with a dissecting hematoma in the media, extending from the aortic
valve to the renal arteries. Which feature of this scenario most
strongly suggests hypertension as the cause of the aortic dissection?
Answer
29>A 68-year-old, well-developed, well-nourished black male presents to
the emergency department complaining of shortness of breath. He denies
chest pain. He has no significant past medical history and takes no
medications. A chest x-ray shows clear lung fields, mild cardiomegaly
and a widened thoracic aorta with linear calcifications. An MRI of the
chest shows aortic dilatation in the thorax, extending proximally,
with atrophy of the muscularis and wrinkling of the intimal surface.
What is the most likely etiology of this condition?
Answer
30>A 60-year-old man presents to a physician because of difficulty in reading
and coming down stairs, which he attributes to an inability
to "look down." Physical examination reveals that the patient
looks around by moving his head rather than his eyes and als
o shows a distinctive axial rigidity of neck, trunk, and
proximal limb muscles. He shows poverty of movement and
dysarthric speech. Mentally, the patient responds very
slowly but has better memory and intellect than are
initially apparent. Which of the following pathologic
Answer
31>At which of the following sites is the characteristic triple helical
structure of the collagen initially formed?
Answer
32>The nucleus that lies immediately medial (and deep) to the uncus is the
Answer
33>A 62-year-old female is brought to the emergency room by her husband
with complaints of shortness of breath. Which of the following physical
findings would be the most reliable indicator that she is experiencing
heart failure?
Answer
34> A couple brings their son in to a specialty clinic for evaluation of
recurrent bacterial infections involving the respiratory tract. Other
family members have a similar disorder, as noted in the pedigree above.
Which of the following is the most likely diagnosis?
Answer
35>Two young boys are playing at a daycare center. One holds a ball on top
of some blocks that the other child has placed on the floor. The second
child helps steady the blocks, then the first child lets go of the ball
, knocking the blocks down to the floor. They both watch and then repeat
the process. These children are most likely
Answer
36>In which of the following neurodegenerative conditions would you expect
to observe the phenomenon known as anticipation?
Answer
38>Which of the following antihistamines would be the most appropriate
treatment for an airline pilot with hay fever?
Answer
39>A newborn baby has multiple hemorrhages. Clotting studies demonstrate
an elevated prothrombin time. An abnormality of which of the following
biochemical processes is likely present in this patient?
Answer
40>Examination of an autopsy specimen from a Mexican immigrant demonstrates
a heart with massive dilation of the aortic root and adjacent aortic arch.
Opening the aorta reveals a smooth interior wall without obvious lesions.
If a histological section through the aortic wall is made, which of the
following would most likely be seen?
Answer
41>A 16-year-old girl is brought to emergency room by her parents for
severe right foot pain. The patient states that the pain started 1
day prior to presentation. She cannot recall any recent trauma,
and denies any past medical or surgical problem. She is active
and walks at least 1 hour daily in the nearby forest. She goes
to high school and is doing very well. She gets along well with
Answer
42>A newborn has a heart rate of 130/min, irregular respirations, and
active muscle movements with good tone. He coughs and grimaces in
response to stimulation; he is pink in color, except for his hands
and feet, which are slightly bluish. This neonate's APGAR score is
43>Which of the following amino acids would most likely be found on the
surface of a protein molecule?
Answer
44>A 46-year-old man sustains a spider bite on his upper eyelid, and an
infection develops. The physician is very concerned about spread of
the infection to the dural venous sinuses of the brain via emissary
veins. With which of the following dural venous sinuses does the
superior ophthalmic vein directly communicate?
Answer
45>A 10-week-old apparently healthy infant is laid down for a nap. The
mother sits in a rocking chair nearby reading. At one point, she
hears the baby make a single small cry, but she keeps reading because
the baby quiets quickly. Later, she gets up to check on the child,
whom she finds dead.Careful autopsy would be most likely to reveal
which of the following cardiac findings?
Answer
46>A 40-year-old HIV-positive man with a CD4 cell count of 25/mm3
complains of progressive memory loss, confusion, and incontinence.
MRI reveals moderate brain atrophy. The patient subsequently dies
of disseminated aspergillosis. Autopsy confirms the presence of
moderate cerebral atrophy. Histologically, there are multifocal
lymphohistiocytic infiltrates with numerous microglial nodules
and scattered multinucleated giant cells. Which of the following
conditions was most likely responsible for this patient's neurologic
symptoms?
Answer
47> The pedigree above illustrates the mode of inheritance for which of
Answer
48>A 45-year-old man develops a large meningioma that compresses the brain.
A head CT scan demonstrates a subfalcine herniation. This herniation
would most likely damage which of the following structures?
Answer
49>A middle-aged patient develops a gelatinous, well-circumscribed mass
in her right cerebral hemisphere. Biopsy of the mass reveals neoplastic
cells with a "fried egg" morphology. Which of the following tumors does
this patient most likely have?
Answer
50>A patient develops an excruciatingly painful infection of the anterior
half of the external ear canal. Which of the following nerves transmits
this impulse?
Answer
Answers
1>The correct answer is A. An exudate results from leakage of
protein-rich fluid from the plasma into the interstitium. It is usually
the result of increased vascular permeability caused by inflammation.
Exudates also contain numerous acute or chronic inflammatory cells,
depending on the inciting event. Of the above choices, only bacterial
pleuritis would produce an exudate. If pleuritis is caused by pyogenic
organisms, the exudate is purulent (neutrophil-rich). If pleural
inflammation is due to mycobacterial infection or neoplastic
infiltration, the resulting exudate will contain chronic inflammatory
cells.
In contrast, a transudate contains less protein and few inflammatory
cells. There are two main mechanisms of transudate formation: 1)
decreased oncotic pressure, such as that which occurs in cirrhosis of
the liver, nephrotic syndrome, and protein-losing enteropathy (choices
B, D, and E); and 2) increased hydrostatic pressure, which may result
from congestive heart failure (choice C).
2>The correct answer is B. Myxopapillary ependymoma is a variant of
ependymoma, a tumor arising from ependymal cells. Histologically,
myxopapillary ependymoma contains a myxoid (mucus-rich) intercellular
matrix, in which spindly neoplastic ependymal cells are arranged in a
fascicular and papillary pattern (hence its designation). It is a benign
tumor that almost always occurs in the distal segment of the spinal
cord, ie, the conus medullaris. Once excised, the patient is cured.
The cerebellum (choice A) is the favorite site for pilocytic
astrocytomas, medulloblastomas, and hemangioblastomas, but not
ependymomas.
In general, classic ependymomas occur in close proximity to the
ventricular cavities, specifically, the 4th ventricle (choice C) in
children and the lateral ventricles (choice D) in adults. The
myxopapillary variant does not occur in either location.
A midbrain location (choice E) would be truly exceptional for any type of ependymoma.
3>The correct answer is B. The disease is beta thalassemia major,
which is a severe hemolytic anemia characterized by a failure to produce
the beta chains of hemoglobin (some HbF, the fetal form of hemoglobin,
is produced). The excess alpha chains are insoluble, leading to intra-
and extravascular hemolysis. These patients require large numbers of
transfusions, and iron overload with resulting secondary hemochromatosis
can contribute to eventual cardiac failure. The heart is also damaged
by the chronic high output state needed to compensate for the anemia.
Calcium (choice A) deposition is seen in damaged tissues and states with high serum calcium, such as hyperparathyroidism.
Magnesium (choice C), potassium (choice D), and sodium (choice E) are highly soluble and do not usually precipitate in tissues.
4>The correct answer is A. The test described is the Ames test, which
measures damage to DNA and correlates well with carcinogenicity in
vitro. It is relatively inexpensive to perform, compared to other tests
of carcinogenicity, and is frequently used as a screening test for
potential carcinogens.
The nitroblue tetrazolium test (choice B) is used to examine the ability
of neutrophils to undergo a respiratory burst, and is used in the
diagnosis of hereditary immunodeficiencies.
The Watson-Schwartz test (choice C) detects porphobilinogen in urine,
and is used in the The correct answer is C. Any tumor "filling the 4th
ventricle" blocks the circulation of cerebrospinal fluid (CSF). This
blockage leads to increased intracranial pressure, which manifests with
nausea, vomiting, headache, nuchal rigidity, and mental status changes.
If surgery is not performed promptly, cerebellar tonsillar herniation
and rapid death will ensue. In children, medulloblastoma and ependymoma
are the most frequent neoplasms presenting in this manner.
There is no evidence in this case suggesting that acute hemorrhage into
the 4th ventricular cavity (choice A) has occurred, nor is
medulloblastoma typically associated with this complication. CNS tumors
that frequently bleed are metastases from melanoma, renal cell
carcinoma, and choriocarcinoma.
Alterations in medullary function (choice B) lead to cardiorespiratory
instability and may be caused by direct tumor compression or
infiltration of the medulla, neither of which is supported by MRI
findings in this case.
Infiltration of the cerebellar vermis (choice D) is certainly seen in
many cases of medulloblastoma, a tumor that arises from this midline
cerebellar structure. However, this would lead to truncal ataxia and
gait instability, not symptoms of increased intracranial pressure.
Medulloblastoma characteristically spreads to the subarachnoid space
(choice E), from which the neoplasm may metastasize to distant sites
such as spinal cord. Plaques of medulloblastoma are often found on the
cerebellar surface, creating a characteristic sugar coating, but this
would not cause any significant blockage of CSF circulation.
The correct answer is C. Any tumor "filling the 4th ventricle" blocks
the circulation of cerebrospinal fluid (CSF). This blockage leads to
increased intracranial pressure, which manifests with nausea, vomiting,
headache, nuchal rigidity, and mental status changes. If surgery is not
performed promptly, cerebellar tonsillar herniation and rapid death will
ensue. In children, medulloblastoma and ependymoma are the most
frequent neoplasms presenting in this manner.
There is no evidence in this case suggesting that acute hemorrhage into
the 4th ventricular cavity (choice A) has occurred, nor is
medulloblastoma typically associated with this complication. CNS tumors
that frequently bleed are metastases from melanoma, renal cell
carcinoma, and choriocarcinoma.
Alterations in medullary function (choice B) lead to cardiorespiratory
instability and may be caused by direct tumor compression or
infiltration of the medulla, neither of which is supported by MRI
findings in this case.
Infiltration of the cerebellar vermis (choice D) is certainly seen in
many cases of medulloblastoma, a tumor that arises from this midline
cerebellar structure. However, this would lead to truncal ataxia and
gait instability, not symptoms of increased intracranial pressure.
Medulloblastoma characteristically spreads to the subarachnoid space
(choice E), from which the neoplasm may metastasize to distant sites
such as spinal cord. Plaques of medulloblastoma are often found on the
cerebellar surface, creating a characteristic sugar coating, but this
would not cause any significant blockage of CSF circulation.
diagnosis of porphyrias.
The Widal test (choice D) is used to diagnose typhoid fever.
The Woellner enzyme test (choice E) detects heterophil antibodies in
patients with Epstein-Barr virus infection, such as infectious
mononucleosis.
5>The correct answer is A. A good way to remember what goes through
the superior orbital fissure is that everything that innervates the eye,
other than the optic nerve, passes through this fissure. This includes
the oculomotor nerve (CN III), the trochlear nerve (CN IV), the
ophthalmic nerve (V1), and the abducens nerve (CN VI).
The facial nerve (CN VII; choice B) passes through the internal auditory meatus.
The mandibular nerve (V3; choice C) passes through the foramen ovale.
The maxillary nerve (V2; choice D) passes through the foramen rotundum.
The middle meningeal artery (choice E) passes through the foramen spinosum.
The ophthalmic artery (choice F) passes through the optic canal.
The optic nerve (choice G) passes through the optic canal.
6>The correct answer is B. Cranial nerve IX is the glossopharyngeal
nerve, which has a nucleus in the medulla and is necessary for the gag
reflex. The gag reflex is elicited by touching either side of the
posterior pharynx with a tongue blade, producing bilateral elevation of
the palate and bilateral contraction of the pharyngeal muscles. The
afferent of this reflex arc consists of the ipsilateral glossopharyngeal
nerve, while the vagus nerve, bilaterally, supplies the efferent limb.
While the glossopharyngeal nerve may seem to be one of the less
important cranial nerves, you should remember to test for its function,
as a loss of gag reflex can lead to the patient's death secondary to an
aspiration pneumonia. This form of pneumonia can be difficult to treat,
as it commonly is due to a mixed flora, which may include a variety of
anaerobes.
Cranial nerve VII (choice A) is the facial nerve, which supplies motor function to the face, but does not supply the oropharynx.
Cranial nerve XII (choice C) is the hypoglossal nerve, which supplies the tongue. It is not involved in the gag reflex.
Cranial nerve XI (choice D) is the spinal accessory nerve, which supplies the trapezius and sternocleidomastoid.
Cranial nerve VIII (choice E) is the vestibulocochlear nerve, responsible for hearing and equilibrium.
7>The correct answer is D. The rule of thumb is that the plasma
concentration will reach 50% in one half-life, 75% in two half-lives,
87.5% in three half-lives, etc., so that the difference between the
current drug level and 100% halves with each half-life. In this
instance, it takes two half-lives to reach 75%. The half-live of this
drug is 6 hours, so two half-lives is 12 hours.
8>The correct answer is E. Herpes simplex can cause a necrotizing,
hemorrhagic acute encephalitis that may rapidly produce death. The
encephalitis characteristically involves the lower portions of the
cerebral cortex, notably the temporal lobes and the base of the frontal
lobes, possibly because the infection spreads from the oropharynx.
9>The correct answer is D. The genetic condition is Marfan syndrome,
which is characterized by skeletal, ocular, and cardiovascular
abnormalities. Typically, Marfan patients are tall with very long legs
and tapering fingers. Laxity of joints is present, so that the thumb can
be extended back to the wrist. Chest and spinal column deformities may
be present as well. The most frequent cardiovascular anomalies are
incompetence of the aortic valve, aortic dissection, and mitral valve
prolapse ("floppy valve"). The latter may give rise to mitral
regurgitation with the typical auscultatory phenomenon of a systolic
click followed by a murmur. The most characteristic ocular change is
ectopia lentis, i.e., dislocation of the lens. Most deaths are due to
rupture of aortic dissections. The gene mutated in Marfan syndrome
encodes fibrillin, a 350-kD protein that serves as scaffolding for the
deposition of elastin and formation of elastic fibers. Elastin (choice
C) is a 70-kD protein that constitutes the central core of elastic
fibers. Abundant elastin is found in the walls of large arteries,
uterus, skin, and ligaments. Although elastic fibers are disrupted by
mutations of the fibrillin gene, the structure of elastin protein is
intact in Marfan syndrome.
Collagen (choice A) is affected in a different set of genetic diseases,
including Ehlers-Danlos syndromes and osteogenesis imperfecta.
Dystrophin (choice B) is a large transmembrane protein whose function is
essential in maintaining the structural integrity of striated muscle
fibers. Mutations of the dystrophin gene, located on X chromosome, are
responsible for muscular dystrophy.
Mutations of the gene for myosin b-chain (choice E) account for more
than one third of cases of hypertrophic cardiomyopathy, a condition
characterized by hypertrophy of the left ventricle. Hypertrophic
cardiomyopathy is not associated with mitral valve prolapse.
10>The correct answer is D. The disease is poliomyelitis, which is
caused by the poliovirus, a picorna virus. The virus is spread via the
fecal-oral route and can then cause paralysis by infecting the
alpha-motor neurons of the anterior horn of the spinal cord. Early
symptoms include malaise, headache, fever, nausea, abdominal pain, and
sore throat.
Bacterial and fungal infections can be spread by dirt contact with an open wound (choice A).
AIDS is an example of a disease spread by injection (choice B) or exchange of body fluids.
Malaria is an example of a disease spread by mosquitoes (choice C).
Tetanus is the classic example of disease spread by a puncture wound of the foot (choice E).
11>The correct answer is A. This is a typical presentation of an
abdominal aortic aneurysm, which is almost always due to severe
atherosclerosis. The foci of calcification described occur within the
atherosclerotic plaques, and indicate severe atherosclerotic disease.
Congenital weakness of vessels (choice B) can produce berry aneurysms, especially in cerebral vessels in the circle of Willis.
Cystic medial necrosis (choice C) can produce dissecting aneurysms, especially in Marfan's syndrome.
Syphilitic aneurysms (choice D) typically involve the aortic root as it leaves the heart.
Vasculitis (choice E) can produce aneurysms in small arteries.
12>The correct answer is A. This scenario is classic for "dependent"
personality. Look for reliance on others, subordination of own needs,
and fear of abandonment. Note that in real life, patients may show
symptoms of more than one personality disorder.
Histrionic personality disorder (choice B) is characterized by
theatricality, suggestibility, a strong desire for attention, and
shallowness.
Obsessive-compulsive personality disorder (choice C), also called
anancastic personality disorder, is characterized by obsessions,
perfectionism, rigidity, and self-doubt.
Paranoid personality disorder (choice D) is characterized by
suspiciousness, oversensitivity, querulousness, and an unforgiving
character.
Schizoid personality disorder (choice E) is characterized by emotional coldness, solitude, and social insensitivity.
13>The correct answer is A. The point of this question is that
sometimes the obvious explanation is the correct one. Occlusion of the
central retinal artery rapidly causes irreversible blindness with loss
of the inner retinal layers. (The photoreceptor rod and cone cells are
maintained by the pigment epithelium.) The site of occlusion is
typically just posterior to the cribriform plate. A garden-variety
atheroma or embolism is overwhelmingly the most common cause of central
retinal artery occlusion.
Despite all of the teaching about the risk of blindness in temporal
arteritis (choice B), this disorder causes only 10% of central retinal
artery occlusions.
Hypertension (choice C) is more apt to cause bleeding than thrombosis.
Polycythemia vera (choice D) could (rarely) cause occlusion because of increased blood viscosity and a tendency for thrombosis.
Tumor (choice E) might also cause retinal artery thrombosis, but this would be far rarer than atheroma.
14>B
15>C
16>The correct answer is A. The facial nerve and the
vestibulocochlear nerve emerge from the brain stem at the
cerebellopontine angle. These are the two nerves which will be initially
affected by a tumor in this region.
The glossopharyngeal nerve and vagus nerve (choices B and E) emerge from
the brain stem at the post-olivary sulcus. This is caudal to the
cerebellopontine angle.
The optic nerve (choice C) exits from the optic chiasm on the ventral
surface of the diencephalon. This is rostral to the cerebellopontine
angle.
The trigeminal nerve (choice D) emerges from the brain stem at the
anterolateral surface of the pons. This is rostral and ventral to the
cerebellopontine angle.
17>The correct answer is B. This patient has gout, characterized by
painful joints due to the precipitation of uric acid crystals caused by
excessive production of uric acid (a minority of cases are associated
with underexcretion of uric acid). Kidney disease is also seen due to
accumulation of uric acid in the tubules. The disease mostly affects
males, and is frequently treated with allopurinol, an inhibitor of
xanthine oxidase. Xanthine oxidase catalyzes the sequential oxidation of
hypoxanthine to xanthine to uric acid.
A defect in urea synthesis (choice A) would result in the accumulation of ammonia.
Phenylketonuria is a disease in which tyrosine cannot be produced from
phenylalanine (choice C). It is characterized by a musty body odor and
mental retardation.
Defective topoisomerases (choice D) would affect DNA unwinding, and therefore replication.
Leukotrienes (choice E) are potent constrictors of smooth muscle and would more likely lead to bronchoconstriction.
18>The correct answer is C. Any tumor "filling the 4th ventricle"
blocks the circulation of cerebrospinal fluid (CSF). This blockage leads
to increased intracranial pressure, which manifests with nausea,
vomiting, headache, nuchal rigidity, and mental status changes. If
surgery is not performed promptly, cerebellar tonsillar herniation and
rapid death will ensue. In children, medulloblastoma and ependymoma are
the most frequent neoplasms presenting in this manner.
There is no evidence in this case suggesting that acute hemorrhage into
the 4th ventricular cavity (choice A) has occurred, nor is
medulloblastoma typically associated with this complication. CNS tumors
that frequently bleed are metastases from melanoma, renal cell
carcinoma, and choriocarcinoma.
Alterations in medullary function (choice B) lead to cardiorespiratory
instability and may be caused by direct tumor compression or
infiltration of the medulla, neither of which is supported by MRI
findings in this case.
Infiltration of the cerebellar vermis (choice D) is certainly seen in
many cases of medulloblastoma, a tumor that arises from this midline
cerebellar structure. However, this would lead to truncal ataxia and
gait instability, not symptoms of increased intracranial pressure.
Medulloblastoma characteristically spreads to the subarachnoid space
(choice E), from which the neoplasm may metastasize to distant sites
such as spinal cord. Plaques of medulloblastoma are often found on the
cerebellar surface, creating a characteristic sugar coating, but this
would not cause any significant blockage of CSF circulation.
19The correct answer is E. Maternal diabetes is best known for causing
large but immature-for-age babies. There is also a specific association
between maternal diabetes and transposition of the great vessels. In
transposition of the great vessels, the aorta takes off from the
anterior part of the right ventricle and the pulmonary trunk takes off
from the posterior part of the left ventricle. This produces a complete
separation of the systemic and pulmonary circulations. Without surgical
correction, most affected infants die within the first months of life,
although a patent ductus arteriosus, patent foramen ovale, or
ventricular septal defect may allow enough mixing of blood to
temporarily sustain life.
In atrial septal defect (choice A) blood can pass from one atrium to the other.
Associate coarctation of the aorta (choice B) with Turner syndrome.
Eisenmenger's syndrome (choice C) is a shift from a left-to-right shunt
to a right-to-left shunt secondary to developing pulmonary hypertension.
Tetralogy of Fallot (choice D) consists of a ventricular septal defect,
an overriding aorta, pulmonic stenosis, and right ventricular
hypertrophy. It is the most common cause of early cyanosis.
20The correct answer is A. This is an example of displacement. In this
defense mechanism, there is a transfer of emotion from a person, object,
or situation with which it is appropriately associated to another that
causes less distress. Displacement is common and often destructive to
other individuals, such as when a man is fired from his job and
subsequently beats his wife or children. In the medical setting, the
hospital staff is a frequent target of displacement when family members
react to their own feelings of guilt about someone's death.
Projection (choice B) occurs when someone attributes his or her own thoughts to a different person.
Reaction formation (choice C) is the unconscious adoption of behavior opposite to one's true feelings.
Regression (choice D) is the adoption of behavior more appropriate to a younger age.
Repression (choice E) is the deeply subconscious blocking of memories or emotions.
21The correct answer is B. This item tests your knowledge of
neurocutaneous syndromes, a group of hereditary conditions characterized
by concomitant neoplastic or hamartomatous lesions predominantly
affecting the skin and nervous system. Subependymal giant cell
astrocytoma is a peculiar astrocytic tumor that grows from the walls of
the lateral ventricles. It is pathognomonic of tuberous sclerosis, which
is caused by mutations of TS1 or TS2 genes. Tuberous sclerosis
manifests with multiple hamartomatous lesions in the skin, CNS, and
visceral organs. Cortical tubers are malformed (hamartomatous) nodules
of the cortex, probably resulting from faulty cortical development.
Other lesions include shagreen patches and ash-leaf spots on the skin,
cardiac myomas, and renal angiomyolipomas.
Café-au-lait spots (choice A) are found in both types of
neurofibromatosis. Lisch nodules (choice D) are small pigmented nodular
lesions of hamartomatous nature that are present in the iris of patients
with neurofibromatosis type 1. Schwannomas of the 8th cranial nerve
(choice E), especially when bilateral, are typically associated with
neurofibromatosis type 2.
Hemangioblastoma (choice C) is a vascular tumor of unknown histologic
origin that frequently develops in the cerebellum of patients with von
Hippel-Lindau syndrome. Renal cell carcinomas are also common in this
disease. Von Hippel-Lindau syndrome is caused by autosomal dominant
mutations of the VHL gene, a tumor suppressor gene. You may recall that
mutations of VHL gene are also found in the majority of sporadic renal
cell carcinomas
--------------------------------------------------------------------------------22The
correct answer is A. This is a typical presentation of an abdominal
aortic aneurysm, which is almost always due to severe atherosclerosis.
The foci of calcification described occur within the atherosclerotic
plaques, and indicate severe atherosclerotic disease.
Congenital weakness of vessels (choice B) can produce berry aneurysms.
Cystic medial necrosis (choice C) can produce dissecting aneurysms (e.g., in Marfan's syndrome).
Syphilitic aneurysms (choice D) typically involve the aortic root as it leaves the heart.
Vasculitis (choice E) can produce aneurysms in small arteries.
23The correct answer is C. The physician should obtain an emergency
order of detention, regardless of her threats of a lawsuit. The woman
clearly still has suicidal intent, demonstrated by her expressed
verbalizations, and is therefore a danger to herself.
Choices A, B, and D clearly place her in a position where she can carry out her plans to terminate her life.
Sedating her (choice E) is the second best choice since it will prevent
her from taking her life; however, sedation does not give therapists the
opportunity to address the underlying motivations for her suicidal
ideation.
24The correct answer is B. Blocking antibody is generally an IgG
antibody against the allergen. It is induced in the allergic patient by
administering small amounts of allergen over a period of time. When the
person is again exposed to the allergen, the IgG reacts with the
allergen before it can reach the IgE-coated mast cell.
Antihistamines (choice A) would block histamine receptors but would not
react with the allergen before it could reach the IgE-coated mast cell.
Cromolyn sodium (choice C) is a drug that stabilizes mast cell
membranes, thus inhibiting degranulation, but would have no effect on
allergen binding to IgE on mast cells.
Epinephrine (choice D) is the mainstay of therapy in severe cases of
immediate hypersensitivity (anaphylaxis). It increases intracellular
cyclic AMP, thus decreasing mast cell degranulation, and causes smooth
muscle dilation in the airways. Epinephrine would have no effect on the
binding of the allergen to IgE on mast cells.
Cyclic AMP is degraded in cells by the enzyme phosphodiesterase.
Theophylline (choice E) inhibits this enzyme, thereby increasing mast
cell cyclic AMP levels and making degranulation less likely without
affecting the binding of the allergen to IgE on mast cells.
25The correct answer is E. At this level, the lateral portion of the
dorsal columns (funiculus) is comprised of the fasciculus cuneatus.
Axons carrying tactile, proprioceptive, and vibratory information from
the ipsilateral arm enter the spinal cord via the dorsal root, ascend
the cord in the fasciculus cuneatus, and synapse in the nucleus cuneatus
of the caudal medulla. Secondary neurons from this nucleus give rise to
internal arcuate fibers, which decussate and ascend to the thalamus
(ventral posterolateral nucleus, VPL) as the medial lemniscus. Tertiary
neurons from the VPL project to the ipsilateral somatosensory cortex.
Therefore, damage to the fasciculus cuneatus would result in a deficit
in tactile, proprioceptive, and vibratory sense in the ipsilateral arm,
because the fibers that carry this information do not cross until they
reach the medulla.
Fine motor control of the fingers (choice A) would be carried
principally by the ipsilateral lateral corticospinal tract in the
lateral funiculus of the cord.
Motor control of the contralateral foot (choice B) is carried by the
ipsilateral corticospinal tract in the lateral funiculus of the cord.
Hemianhidrosis (lack of sweating) over half of the face (choice C) could
be produced by interruption of sympathetic innervation to the face. The
hypothalamospinal tract projects from the hypothalamus to the
intermediolateral cell column at levels T-1 to T-2. It descends in the
lateral funiculus of the cord. Interruption of this tract results in
Horner's syndrome (miosis, ptosis, hemianhidrosis).
Proprioception from the ipsilateral leg (choice D) is carried by the
fasciculus gracilis in the medial part of the dorsal columns.
26The correct answer is A. While it is hard to develop a deficiency in
oil-soluble vitamins (A, D, E, K) because the liver stores these
substances, deficiency states can be seen in chronic malnutrition
(specifically chronic fat deprivation) and chronic malabsorption.
Vitamin A is necessary for formation of retinal pigments (deficiency can
cause night blindness) and for appropriate differentiation of
epithelial tissues (including hair follicles, mucous membranes, skin,
bone, and adrenal cortex).
Vitamin C (choice B), which is water soluble rather than oil soluble, is necessary for collagen synthesis.
Vitamin D (choice C) is important in calcium absorption and metabolism.
Vitamin E (choice D) is a lipid antioxidant that is important in the stabilization of cell membranes.
Vitamin K (choice E) is necessary for normal blood coagulation.
27The correct answer is C. The most likely diagnosis is an intracranial
meningioma. Meningiomas are slow-growing, benign tumors comprising 15%
of intracranial tumors; they are most common in the elderly. They
originate from either dura mater or arachnoid and are sharply demarcated
from brain tissue. Meningiomas often incite an osteoblastic reaction in
the overlying cranial bones. Microscopically, the meningioma cells have
a tendency to encircle one another, forming whorls and psammoma bodies.
Clinically, they present as mass lesions; seizures may occur. The
superior parasagittal surface of the frontal lobes is a favorite site of
origin. This can often produce leg weakness, since the leg motor fibers
that pass down through the internal capsule originate in parasagittal
cortical regions. Treatment of meningiomas is usually surgical.
Arachnoid cysts (choice A) are formed by splitting of the arachnoid
membrane; most arachnoid cysts arise near the Sylvian fissure. They may
present with mass effect, but would be unlikely to produce seizures,
prominent focal signs, or reactive hyperostosis.
Glioblastoma multiforme (choice B) is an aggressive malignant
astrocytoma that would likely have killed the patient long before 5
years had elapsed.
Metastatic breast cancer (choice D) would generally look different
microscopically (the whorling cell pattern is characteristic of
meningioma). It would be unlikely for metastatic cancer to cause a
reaction in the overlying bone, or to be present long enough to cause
symptoms for 5 years.
Oligodendrogliomas (choice E) are glial tumors that could produce the
described clinical picture, but usually do not cause hyperostosis of the
calvarium or exhibit the characteristic whorling cell pattern
microscopically
28The correct answer is D. The two most common causes of aortic
dissection are hypertension and atherosclerosis. An important
distinction between the two is that hypertensive dissections generally
originate in the ascending aorta, at an intimal surface free of
atherosclerosis.
Dissection secondary to atherosclerosis is typically the consequence of a
ruptured aortic aneurysm, which originates in the abdominal aorta at
the iliac bifurcation. Dissections due to both hypertension and
atherosclerosis generally course through the wall within the media
(choice B). They both can involve the entire length of the aorta, and
may rupture anywhere along its course (choices A and C). Well recognized
sequelae of dissections include rupture through the adventitia,
compromise of major arterial branches or the aortic valve, cardiac
tamponade, and rapid exsanguination (choice E).
29The correct answer is D. Although rare now because of advances in
treatment, syphilitic aortitis and aneurysm are still seen, especially
in underserved populations. This complication generally occurs 10 to 40
years after initial infection. The vasa vasorum of the aorta undergoes
obliterative endarteritis, leading to atrophy of the muscularis and
elastic tissues of the aorta and dilatation. Linear calcifications are
often seen in the ascending aorta by x-ray. The intimal wrinkling or
"tree barking" is also a common feature. Syphilitic aneurysm can be
associated with respiratory distress, cough, congestive heart failure
and rarely, rupture.
Atherosclerosis (choice A) is the most common cause of aortic aneurysms.
These are most often located in the abdominal aorta, distal to the
renal arteries. Intimal wrinkling and linear calcifications are not
seen.
Hypertension (choice B) is usually responsible for dissecting aneurysms
located within 10 cm of the aortic valve. Patients present with sudden
chest pain, which is usually severe and tearing in nature. The chronic
hypertension causes a cystic medial necrosis, allowing the separation of
vessel layers.
Marfan's syndrome, an autosomal dominant connective tissue disorder
(choice C) is also associated with dissecting aneurysms, usually of the
ascending aorta. The patients are often very tall with arachnodactyly
and ligamentous laxity. Their life-span is generally shortened. This
patient's description and age are not consistent with this diagnosis.
Takayasu's arter itis (choice E) is a syndrome characterized by ocular
disturbances and weak pulses in the arms. It occurs most frequently in
young females. It is considered a giant cell arteritis, and does not
cause aneurysms.
30The correct answer is E. The disease is progressive supranuclear
palsy, a degenerative disorder characterized by ophthalmoplegia,
pseudobulbar palsy, axial dystonia, and bradykinesia. The presentation
described in the question is typical. The pathologic changes consist of
widespread neuronal loss and gliosis in subcortical sites with sparing
of the cerebral and cerebellar cortices.
Pigmented neurons in the substantia nigra (pars compacta) and locus
ceruleus (choice A) selectively degenerate in Parkinson's disease.
In Alzheimer's disease, there is diffuse cortical atrophy (choice B),
especially over the association cortex of frontal, temporal, and
parietal lobes, with relative sparing of primary sensory and motor
areas.
Selective frontal and temporal lobe atrophy (choice C) is characteristic of Pick's disease.
The caudate nucleus and putamen undergo severe atrophy (choice D) in
Huntington's disease. Cortical atrophy occurs to a lesser extent.
31The correct answer is B. Collagen formation begins with transcription
of mRNA from appropriate DNA genes in the nucleus. While still within
the nucleus, the mRNA is spliced. It is then transported through the
cytoplasm to the ribosomes on the rough endoplasmic reticulum.
Individual chains are translated on the ribosomes, with the ends feeding
into the endoplasmic reticulum lumen. Within the lumen, glycosylation
of the individual chains occurs. The material then moves toward the
Golgi bodies (whose lumens are connected to the endoplasmic reticulum)
where the triple helices of procollagen form. The procollagen is then
secreted into the extracellular space, where cleavage of pro-peptides
and cross- linking of different triple helices occurs, maturing the
collagen.
The extracellular space (choice A) is the site of procollagen cleavage and cross- linking.
The nucleus (choice C) is the site of mRNA transcription and splicing.
The rough endoplasmic reticulum (choice D) is the site of chain translation and glycosylation.
The smooth endoplasmic reticulum (choice E) does not participate in collagen synthesis
32The correct answer is A. The uncus, which is the medial protrusion of
the parahippocampal gyrus, is an external structure seen on the ventral
surface of the temporal lobe. The amygdala is a collection of nuclei
that lies directly beneath the uncus.
The caudate nucleus (choice B) is a deep nuclear structure that lies lateral to the lateral ventricles.
The claustrum (choice C) is a thin and elongated nucleus that lies just medial to the insular cortex.
The hippocampus (choice D) is a nuclear structure that lies in the interior of the parahippocampal gyrus.
The putamen (choice E) is a nuclear structure that resides lateral to the caudate, and medial to the claustrum.
The thalamus (choice F) is a deep nuclear structure, caudal to the caudate nucleus.
33The correct answer is A. A third heart sound (S3) is a low-pitched
sound occurring at the termination of rapid filling. In patients over 40
years of age, the appearance of a third heart sound strongly suggests
congestive heart failure. It also occurs in patients with
atrioventricular valve incompetence and can be a normal finding in some
young athletes.
A fourth heart sound (S4; choice B) can be a normal finding in some older patients who do not have congestive heart failure.
Ascites (choic