1
1) Patients with diabetes mellitus develop progressive stocking-glove neuropathies and
autonomic neuropathies for gastri...
2
6) Unilateral intracranial edema can push the hemisphere into the posterior fossa and the
brainstem downward. This is of...
3
12) The spinal cord in amyotrophic lateral sclerosis shows:
A) Anterior horn atrophy and gray discoloration of the corti...
4
17) Molecular diagnostic assays performed on muscle biopsy from the patient described
in Question 16 would show alterati...
5
22) Transmissible spongiform encephalopathies are known to cross animal species and
include all of the following EXCEPT:...
6
28) When Parkinson patients have not only a shuffle, but also dementia, it is expected the
brain will show:
A) Lewy bodi...
7
33) Myoclonic epilepsy with ragged red fibers features all of the following EXCEPT:
A) Maternally inherited myoclonus, s...
8
39) Microscopy of a brain tumor shows packed “fried egg” tumor cells in a “chicken
wire” vascular stroma. Fine stippling...
9
44) Arteriosclerosis in the non-diabetic is usually most severe at the:
A) Middle cerebral artery origin
B) Bifurcation ...
10
49) A female neonate is noted to have a pronounced enlargement of head. She develops
convulsions. MRI reveals excessive...
11
31) Which of the following is the most common CNS malformation?
A) Polymicrogyria
B) Arnold-Chiari malformation
C) Spin...
12
C) Spinal cord
D) Meninges
38) The cells of origin of most adult CNS neoplasms are:
A) Arachnoid cells
B) Ependymal cel...
13
D) Pompe’s disease
E) Duchenne muscular dystrophy
44) A 12-month-old girl has increased weakness involving all of her e...
14
49) Following an episode of pharyngitis for less than a day, a 20-year-old woman
develops a headache and then becomes o...
15
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Block X Pathology Exam 1

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Block X Pathology Exam 1

  1. 1. 1 1) Patients with diabetes mellitus develop progressive stocking-glove neuropathies and autonomic neuropathies for gastric paresis and orthostatic hypotension because of: A) Metabolic acidosis of the extremities B) Recurrent episodes of ketoacidosis C) Disturbance of the pituitary-pancreas axis D) Progressive renal failure E) Thickening of vascular basement membranes 2) Cross section of a peripheral nerve showing onion bulbs by light and electron microscopy is not limited to, but suggests a diagnosis of: A) Arsenic poisoning B) Amyloidosis C) Charcot-Marie-Tooth disease D) Dejerine-Sottas disease E) Leprosy 3) Two weeks after receiving a flu shot, the patient returns with weakness of the legs that is ascending to the trunk. You hospitalize her as the weakness reaches the chest to interfere with breathing. Examination of her tissues would show: A) Anterior horn cell degeneration in the spinal cord B) Sympathetic ganglion inflammation and degeneration C) Central chromatolysis of the motor cortex D) Demyelination and subacute inflammation of the dorsal root ganglia E) Empty neurons in the cranial nerve nuclei 4) The Herpes zoster virus of chickenpox may induce: A) Atrophy of cranial nerves B) Bleeding into the dorsal root ganglion C) Constriction of peripheral nerve sheaths for atrophy D) Damage to anterior horn cells E) Empty nuclei in the hypoglossal nerve nucleus 5) When a peripheral nerve is cut, the anterior horn cell will lose much of its Nissl substance and have the nucleus become eccentric. This phenomenon is known as: A) Anterior atrophy B) Backflow biosphere C) Central chromatolysis D) Distal dystrophy E) Expanding exoplasm
  2. 2. 2 6) Unilateral intracranial edema can push the hemisphere into the posterior fossa and the brainstem downward. This is often associated with potentially fatal hemorrhagic lesions in the midbrain and pons which are termed: A) Apical meristem B) Backflow stenosis C) Cerebellar shrinkage D) Duret hemorrhages E) Ependymal sclerosis 7) Subarachnoid hemorrhage in the premature infant most likely comes from: A) Abuse by parents B) Base of the skull C) Choroid plexus D) Dropping the child during delivery E) Germinal matrix 8) Using a seatbelt most markedly reduces the incidence of: A) Arm injuries B) Base of frontal lobe and temporal tip trauma C) Coup-contrecoup injuries D) Caput succedaneum E) Epidural hematomas 9) Bullet exit wounds from the skull differ from entrance wounds in that they are: A) Larger and irregular B) Bilobed and smooth C) Contoured according to lobe of skull D) Diameter of the bullet and round E) Surrounded by powder burns 10) Which of the following brain malformations is characterized primarily by a small posterior fossa, dysplastic cerebellum and cerebellar tonsil herniation? A) Polymicrogyria B) Spina bifida C) Arnold-Chiari malformation D) Agenesis of the corpus callosum E) Anencephaly 11) The most common form of spinal muscular atrophy is Werdnig-Hoffman myopathy. It is an autosomal recessive disorder on chromosome 5q13.1 affecting: A) Survival motor neuron gene B) Schwann cell maturation and synapse genes C) Copper-zinc superoxide dismutase gene (SOD1) D) Tropomyosin 3 (TPM3), NEM2, and nebulin (NEB) genes E) Mitochondrial DNA (mtDNA)
  3. 3. 3 12) The spinal cord in amyotrophic lateral sclerosis shows: A) Anterior horn atrophy and gray discoloration of the corticospinal tract B) Lateral column pallor and posterior root atrophy C) Central cavitation D) Dorsal root atrophy and spinocerebellar degeneration E) Ependymal cell hypertrophy around the syrinx 13) Acute Coxsackie B myositis is associated with all of the following EXCEPT: A. Muscle lysis potentially blocking renal tubules B. Systemic symptoms such as fever and malaise C. Infiltration by lymphocytes and monocytes that become macrophages D. Elevated serum CPK and myosin E. Encysted worms 14) The malignant hyperthermia syndrome is characterized by all of the following EXCEPT: A. Family history of malignant hyperthermia B. Occurs in operations using volatile anesthetics or succinyl choline C. Muscle biopsy can be tested for caffeine/halothane sensitivity D. Muscle spasm, tachycardia, and cyanosis can be fatal E. Microscopic changes on diagnostic biopsy are rampant 15) A 21-year-old girl complains of persistent redness of the exposed skin (face, neck, V- line, forearms, and lower legs). She describes easy fatigability and can rise only with difficulty from a squatting position. Physical examination reveals erythema of the face and neck and a heliotropic rash. A muscle biopsy reveals infiltrates of lymphocytes and macrophages surrounding dead and dying muscle fibers. Elevated serum leves of which of the following would be expected in this patient? A) Albumin B) Blood urea nitrogen C) Creatine kinase D) Antistreptolysin O antibodies E) Rheumatoid factor 16) A 4-year-old boy is brought to the physician by his parents because he falls a lot, cannot jump, and tires easily. Physical examination reveals weakness in the pelvic and shoulder girdles and enlargement of the child’s calf muscles. The serum level of creatine kinase is elevated. A biopsy of the calf muscle reveals marked variation in size and shape of muscle fibers. There are foci of muscle fiber hypercontraction, coagulation necrosis, myophagocytosis, regenerating fibers and fibrosis. Which of the following is the most likely cause of death expected in this patient? A) Rhabdomyosarcoma B) Pulmonary embolism C) Respiratory insufficiency D) Dissecting aortic aneurysm E) Disseminated intravascular coagulation
  4. 4. 4 17) Molecular diagnostic assays performed on muscle biopsy from the patient described in Question 16 would show alterations in the length of primary transcript for which of the following muscle-associated proteins? A) Creatine kinase B) Desmin C) Dystrophin D) Glycogen phosphorylase E) Myosin 18) A 60-year-old man who had been treated for lung cancer complains of a rash on his face, neck and chest and pain in his upper arms and calves. He cannot raise his arms and climbs the stairs only with difficulty. A muscle biopsy reveals perivascular infiltrates of lymphocytes and plasma cells extending in between the muscle fibers. Which of the following is the most likely diagnosis? A) Toxic myopathy B) Myasthenia gravis C) Lambert-Eaton myasthenic syndrome D) Dermatomyositis E) Becker muscular dystrophy 19) The location of an infection in the CNS is highly suggestive of the pathway and organism. A frontal epidural abscess is most likely: A) Amoeba from swimming in fresh water B) Sarcoidosis from the lungs C) Candidiasis from the mastoid air cells D) Streptococcus pneumoniae from a frontal sinus E) Viral from the nasopharynx 20) Rabies (also avoidable by immunization) causes 10% of death in Thailand today. Most patients die showing: A) Apathy and confusion B) Rage and aggression C) Sexual hyperactivity D) Destruction of property E) Hyperesthesia and pneumonia 21) At postmortem the clinical diagnosis of rabies can be confirmed by finding: A) Negri bodies in the cytoplasm of hippocampus pyramidal neurons and Purkinje cells B) Non-caseating granulomas C) Amyloid depositions D) Intraerythrocytic parasites E) Moluscum bodies
  5. 5. 5 22) Transmissible spongiform encephalopathies are known to cross animal species and include all of the following EXCEPT: A) Scrapie in sheep B) Bovine spongiform encephalopathy C) Chronic wasting disease in deer and elk D) Variant Creutzfeldt-Jakob disease in humans E) Lyme disease 23) People with kuru had all of the following EXCEPT: A) An inordinately high incidence in women and children B) Burns causing 10% of deaths C) Cortical amyloid plaques of prion proteins D) Accumulation of glucocerebrosides in foamy macrophages E) Spongy appearance to the cortex 24) Examination of a brain from a patient dying with multiple sclerosis tends to show all of the following EXCEPT: A) Atrophy sufficient to cause enlarged lateral ventricles B) Bilateral plaques around the frontal and occipital horns of the lateral ventricles C) Cerebellar plaques D) Pontine and spinal cord plaques E) Extensive involvement of the cortical gray matter 25) Loss of myelin in the corpus callosum following ingestion of red wine is called: A) Acute necrotizing hemorrhagic encephalitis B) Marchiafava-Bignami disease C) Central pontine myelinolysis D) Devic’s disease E) Gerstmann-Straussler-Scheinker disease 26) Alzheimer’s dementia patients may have all of the following EXCEPT: A) Atrophy of brain with hydrocephalus ex vacuo B) Perivascular Aβ40 amyloid deposition C) Cytoplasmic neurofibrillary tangles of tau protein D) Enlarged nucleus basalis of Meynert E) Extracellular senile plaques up to 200 μm in diameter 27) The Parkinson patient has lost more than 75% of neurons in the: A) Lenticular nuclei B) Basal ganglia C) Cortical layer 2 D) Substantia nigra of the pons E) Vermis of the cerebellum
  6. 6. 6 28) When Parkinson patients have not only a shuffle, but also dementia, it is expected the brain will show: A) Lewy bodies B) Negri bodies C) Both A and B D) Neither A nor B 29) A 35-year-old man presents with a history of behavioral and personality changes and unusual involuntary movements. Physical examination reveals chorea and dystonia. The patient’s mother and maternal grandfather had similar clinical symptoms. His mother died in a psychiatric institute, and his maternal grandfather committed suicide. MRI shows bilateral cerebral atrophy and enlargement of the lateral ventricles. Marked atrophy would also be expected in which of the following regions of this patient’s brain? A) Cerebellum B) Hypothalamus C) Substantia nigra D) Caudate nuclei E) Anterior horn of the spinal cord 30) Which of the following gene abnormalities would be expected in the patient described in Question 29? A) Deletion of an exon in the gene for presenilin B) Expansion of CAG trinucleotide repeats C) Frame shift mutation in the gene for superoxide dismutase D) Nondisjunction during meiosis of chromosome 21 E) Point mutation in the gene for the prion protein 31) A patient presents with slowly progressive, adult onset dementia. Exam of the retina reveals a cherry red spot at the macula of the retina. MRI shows global atrophy of the brain with enlarged ventricles. Brain biopsy reveals markedly swollen neurons that, on electron microscopy, are stuffed with dark, lipid-like material that chemically consists of GM2 gangiosides. The diagnosis is: A) Tay-Sachs disease B) Niemann-Pick disease C) Gaucher’s disease D) Krabbe’s globoid leukodystrophy E) Pelizaeus-Merzbacher disease 32) Because the brain and muscle are both highly dependent on energy, a disorder seemingly affecting only those two systems is highly suggestive of a defect in: A) Lysosomes B) Nucleoli C) Mitochondria D) Plasma membranes E) Endoplasmic reticulum
  7. 7. 7 33) Myoclonic epilepsy with ragged red fibers features all of the following EXCEPT: A) Maternally inherited myoclonus, seizures, and myopathy with occasional ataxia B) Cut brain shows brown discoloration of dentate and inferior olivary nuclei C) Muscle has ragged red fibers with large mitochondria D) Total loss of mammillary bodies, putamen, and caudate E) Two mutations affecting mitochondrial tRNA and protein synthesis 34) Deficiency of arylsulfatase A (chr 22q) can lead to infantile, juvenile, or adult: A) Adrenoleukodystrophy B) Krabbe’s globoid leukodystrophy C) Canavan’s spongiform leukodystrophy D) Metachromatic leukodystrophy E) Pelizaeus-Merzbacher disease 35) Carbon monoxide poisoning reveals a selective vulnerability of the: A) Medial portion of the globus pallidus B) Brainstem nuclei C) Corpus callosum D) Dentate nucleus E) Putamen 36) The rule of 70s with regard to brain tumors includes all of the following EXCEPT: A) 70% of children have posterior fossa tumors B) 70% of those are medulloblastomas C) 70% of adults have supratentorial tumors D) 70% of those are gliomas E) 70 % of gliomas are astrocytomas 37) Characteristics of juvenile pilocytic astrocytoma include all of the following EXCEPT: A) Astrocytes elaborate a fluid to form a cyst B) Tumor cells are elongate, or bipolar C) Cerebellar hemisphere is the most common location D) Demonstration of mural tumor nodule on MRI facilitates removal E) Surgical removal is a cure, requires no follow-up 38) Glioblastoma multiforme is so named because: A) Astrocyte patterns vary markedly in different zones B) Gut cells may appear in portions of the tumor C) Capillary proliferation is variable in same tumor D) Malignant oligos and ganglion cells may be found in some areas E) All of the above
  8. 8. 8 39) Microscopy of a brain tumor shows packed “fried egg” tumor cells in a “chicken wire” vascular stroma. Fine stippling of calcium was noted on the x-ray. The most likely diagnosis is: A) Astrocytoma, grade III B) Medulloblastoma C) Choroid plexus papilloma D) Oligodendroglioma, grade I E) Glioblastoma multiforme 40) A high school girl starts burning her fingers while smoking cigarettes. A family history of von Recklinghausen disease prompts an MRI to confirm a cervical syrinx and swelling of the cord. At surgery a red mass is extracted from the center of the cord. Microscopy shows tumor cells forming rosettes around vessels or mucin-filled cavities. Diagnosis is: A) Astrocytoma B) Medulloblastoma C) Choroid plexus papilloma D) Glioblastoma E) Ependymoma 41) A 45-year-old woman is brought to the emergency room after experiencing a generalized seizure. An X-ray film of the skull reveals a hyperostotic bone lesion. A CBC is normal. A portion of the skull and the mass adherent to the dura are removed. Microscopic examination of the surgical specimen shows syncytial whorled clusters of cells. What is the appropriate diagnosis? A) Oligodendroglioma B) Meningioma C) Medulloblastoma D) Ependymoma E) Astrocytoma 42) The saccular aneurysm of the circle of Willis is due to: A) Arteriosclerosis B) Blunt trauma C) Choroid plexus thrombosis D) Defect in the media E) Skull fracture across the wall 43) Polyvascular (wickerwork) channels are: A) Attributed to high fat diets B) Birth defects C) Cancer dependent D) Due to ischemia or hypoxia E) Evidently very effective
  9. 9. 9 44) Arteriosclerosis in the non-diabetic is usually most severe at the: A) Middle cerebral artery origin B) Bifurcation of the common carotid artery C) Circle of Willis per se D) Distal branches of the arteries E) Basilar bifurcation 45) Dizzy spells in an older adult when using the left arm may be due to: A) Subclavian steal syndrome B) Basilar artery arteriosclerosis C) Closure of the left posterior cerebral artery D) Dissection of the common carotid artery E) Embolus to the left vertebral artery 46) A 95-year-old woman has a major stroke resulting in paralysis of the left side of the body. She survives six months and ultimately dies of pneumonia. Examination of the brain should show all of the following EXCEPT: A) Wallerian degeneration of the left corticospinal tracts B) Loss of most of the right motor cortex C) Marked shrinkage of the right internal capsule D) Atrophy of the left inferior olivary nucleus E) Active herniation of the uncus of the right temporal lobe 47) Rupture of a Charcot-Bouchard microaneurysm would most likely result in: A) Cavernous sinus thrombosis B) Subarchnoidal hemorrhage C) Subdural hematoma D) Hypertensive intracerebral hemorrhage E) Epidural hematoma 48) Becker muscular dystrophy is characterized by all the following except: A) Onset of clinical symptoms occurs late B) Many patients have an almost normal lifespan C) Muscle weakness is less severe D) Primarily females are affected E) It evolves over a longer period of time
  10. 10. 10 49) A female neonate is noted to have a pronounced enlargement of head. She develops convulsions. MRI reveals excessive accumulation of cerebrospinal fluid, ventricular enlargement, atresia of the aqueduct of Sylvius and atrophy of the cerebral cortex. This birth defect was most likely caused by: A) Birth trauma B) Congenital brain tumor C) Ependymitis D) Lack of the muscular wall in the anterior communicating artery E) Anencephaly 50) Veterans with damage to their trunk or extremities can have foci of extreme sensitivity and pain on touching or compression during normal movement. These are due to: A) Amputation neuromas B) Blast effects on nerves C) Compensation seeking D) Dorsal root ganglion degeneration E) Extramural sexual activity with venereal disease 27) Common complications of saccular (“berry”) aneurysms include: A) Rupture and epidural hematoma B) Thrombosis and brain infarction C) Rupture and subarachnoid hemorrhage D) Multiple foci of perivascular demyelination E) Rupture and ganglionic hemorrhages 28) Common complications of Charcot-Bouchard microaneurysms include: A) Thrombosis and brain infarction B) Rupture and epidural hematoma C) Rupture and subarachnoid hemorrhage D) Rupture and ganglionic hemorrhages E) Rupture and lobar hemorrhages 29) Signs of raised intracranial pressure include all of the following except: A) Headache B) Stiff neck C) Vomiting D) Papilledema 30) The Kernohan’s notch is a: A) Complication of foramen magnum herniations B) Complication of subfalcine herniations C) Complication of transtentorial (uncinate) herniations D) Cortical contusion contralateral to the site of impact
  11. 11. 11 31) Which of the following is the most common CNS malformation? A) Polymicrogyria B) Arnold-Chiari malformation C) Spina bifida D) Anencephaly E) Agenesis of the corpus callosum 32) Characteristic cerebrospical fluid (CSF) changes of acute pyogenic (bacterial) meningitis include all of the following except: A) Pleocytosis (neutrophils) B) Increased pressure C) Increased protein concentration D) Increased glucose concentration 33) Cerebral abscesses may be a complication of: A) Myocardial infarct B) Down syndrome C) Otitis and sinusitis D) Acute pyogenic (bacterial) meningitis 34) Lewy bodies are neuronal cytoplasmic inclusions typical of: A) Alzheimer disease B) Parkinson disease C) Rabies encephalitis D) Herpes simplex encephalitis 35) Characteristic CSF changes of viral meningitis include all of the following except: A) Pleocytosis (lymphocytes) B) Negative Gram staining C) High protein concentration D) Low sugar concentration 36) The vast majority of primary brain tumors in adults arise in the: A) Cerebral hemispheres B) Posterior fossa C) Spinal cord D) Meninges 37) The vast majority of brain tumors in children arise in the: A) Cerebral hemispheres B) Posterior fossa
  12. 12. 12 C) Spinal cord D) Meninges 38) The cells of origin of most adult CNS neoplasms are: A) Arachnoid cells B) Ependymal cells C) Astrocytes D) Oligodendrocytes E) Neuronal cells 39) Which of the following systems of neurons is primarily damaged in Parkinsonian syndromes? A) Serotoninergic neurons of the raphe nucleus B) Dopaminergic neurons of the substantia nigra C) GABAergic neurons of the striatum D) Cholinergic neurons of the nucleus basalis 40) The brain lesions caused by acute thiamine deficiency affect mainly the: A) Sommer’s sector of hippocampus B) Mammillary bodies C) Posterior columns of the spinal cord D) Substantia nigra E) Caudate nuclei 41) Intracranial schwannomas most frequently involve the: A) Optic nerve B) Hypoglossal nerve C) Olfactory nerve D) Trigeminal nerve E) Auditory nerve 42) The least differentiated variant of astrocytoma is the: A) Pilocytic astrocytoma B) Glioblastoma C) Fibrillary astrocytoma D) Gemistocytic astrocytoma E) Anaplastic astrocytoma 43) A 22-year-old woman is found to have symmetric muscular weakness and myalgias. On physical examination a lilac-colored eyelid rash is noted. A muscle biopsy reveals lymphocytes and neutrophils with destruction of individual muscle fibers. She most likely has: A) Myasthenia gravis B) Dermatomyositis C) Werdnig-Hoffman disease
  13. 13. 13 D) Pompe’s disease E) Duchenne muscular dystrophy 44) A 12-month-old girl has increased weakness involving all of her extremities. Laboratory findings are unremarkable. A muscle biopsy reveals many very small round muscle fibers, a few scattered very large fibers 2-4 times normal size and target fibers. She most likely has: A) Becker muscular dystrophy B) McArdle’s disease C) Polymyositis D) Myasthenia gravis E) Werdnig-Hoffman disease 45) Absent immunohistochemical membrane staining for dystrophin is best associated with: A) Duchenne muscular dystrophy B) Becker muscular dystrophy C) Both A and B D) Neither A nor B 46) Which of the following muscle fiber types contract slowly, is high in enzymes of the oxidative pathway and shows high resistance to fatigue? A) Type I B) Type IIa C) Type IIb D) Both B and C 47) A 33-year-old man has been found to have manifested difficulty with vision and with movement of his right arm. An MR imaging reveals periventricular plaques of demyelination in the white matter. He most likely has: A) Basal ganglia hemorrhage B) Amyotrophic lateral sclerosis C) Multiple sclerosis D) Astrocytoma E) Tuberculoma 48) A 70-year-old man has a history of progressive cognitive decline over the past 7 years. He has no movement disorder. At autopsy, histologic sections of his cerebral cortex reveal many neuritic (senile) plaques with amyloid cores. He most likely has: A) Creutzfeldt-Jacob disease B) Amyotrophic lateral sclerosis C) Parkinson disease D) Lacunar infarcts E) Alzheimer disease
  14. 14. 14 49) Following an episode of pharyngitis for less than a day, a 20-year-old woman develops a headache and then becomes obtunded over a 24-hour period. She has fever and a stiff neck; papilledema is noted on funduscopic examination. She most likely has: A) Subarachnoid hemorrhage B) Acute pyogenic (bacterial) meningitis C) Meningioma D) Cerebral abscess 50) A 47-year-old man presents with a 3 cm enhancing mass on MR imaging in the right frontal lobe. He has been having headaches for the past 3 months. A stereotaxic biopsy reveals cells resembling “fried eggs”. He most likely has: A) Astrocytoma B) Glioblastoma C) Oligodendroglioma D) Medulloblastoma
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