MCQ Book Questions

Q60882. A male neonate is noted at birth to have paralysis of the lower limbs. The infant fails to thrive and expires. The brainstem and cerebellum are examined at autopsy (shown in the image). What is the diagnosis?

Q60883. A female neonate is noted to have a pronounced enlargement of her head (shown in the image). She develops convulsions. MRI reveals excessive accumulation of cerebrospinal fl uid, ventricular enlargement, and atrophy of the cerebral cortex. This developmental birth defect was most likely caused by which of the following mechanisms of disease?

Q60884. A 50-year-old man presents with a “staggering” gait and “light- ning pain” in his hands and legs. His past medical history is signifi cant for an aortic aneurysm and aortic insuffi ciency. Neurologic examination reveals impaired senses of vibration, as well as touch and pain in the lower extremities. The patient subsequently dies of pneumonia. Autopsy discloses oblitera- tive endarteritis of meningeal blood vessels and atrophy of the posterior columns of the spinal cord. What is the appropriate diagnosis?

Q60885. A 76-year-old man is admitted to the hospital for evaluation of progressive memory loss and disorientation. The pupils are small but react normally to light. Muscle tone is normal. A lumbar puncture returns clear, colorless CSF under normal pressure. An electroencephalogram shows diffuse slowing. A CT scan of the brain reveals moderate atrophy. Which of the following is the most likely diagnosis?

Q60886. Which anatomic structure/region produced the CSF that accumulated in the brain of the neonate described in Question 4?

Q60887. A 3-year-old girl who has been mentally retarded since birth is killed in a drowning accident. The superior surface of the brain at autopsy is shown in the image. What is the diagnosis for this gross deformity of the brain?

Q60888. A 35-year-old woman complains of urinary incontinence and blurred vision for 2 months. A funduscopic examina- tion shows no abnormalities. Two months later, the patient develops double vision and numbness in the fi ngers of her left hand. MRI shows scattered plaques in the patient’s brain and spinal cord. Over the next several months, some of these plaques diminish in size, while others appear in new loca- tions. These plaques would most likely show selective loss of which of the following proteins?

Q60889. A 30-year-old woman presents with an 8-day history of mild tremor in her arms and impaired balance when walking. Vital signs are normal. Her symptoms disappear the following week. About 18 months later, the patient experiences another episode of weakness and requires assistance when walking. Neurologic examination reveals ataxia, dysarthria, decreased vibratory sensation in her legs, absent abdominal refl exes, increased deep tendon refl exes, and a Babinski sign on the left. Fifteen years after the onset of symptoms, the patient becomes bedridden and dies. A coronal section of the patient’s brain at autopsy is stained for myelin with luxol fast blue (shown in the image). Which of the following histopathologic fi ndings would be expected in these plaques?

Q60890. What key feature distinguishes normal proteins from their pathologic forms in the brain of the patient described in Ques- tion 10?

Q60891. An 88-year-old woman with Alzheimer disease dies of conges- tive heart failure. Examination of the brain at autopsy shows bilateral atrophy of the gyri, particularly in the frontal and hip- pocampal cortex. What additional fi nding might be expected in the brain of this patient?

Q60892. 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 clini- cal symptoms. His mother died in a psychiatric institute, and his maternal grandfather committed suicide. MRI shows bilat- eral cerebral atrophy and enlargement of the lateral ventricles. Marked atrophy would also be expected in which of the fol- lowing regions of this patient’s brain?

Q60893. Which of the following gene abnormalities would be expected in the patient described in Question 13?

Q60894. A 60-year-old man with a history of smoking and chronic bronchitis complains of diffi culty walking. On examination, the patient appears stiff and stooped, shows an expression- less face, and speaks in a monotonous voice. A tremor of his fi ngers is apparent but ceases when he tries to reach for some- thing. The patient dies 3 years later of metastatic lung cancer. At autopsy, the substantia nigra of the patient (right) differs from that of a normal brain (left). This pathologic fi nding is associated with which of the following biochemical changes?

Q60895. A 60-year-old chronic alcoholic was found in a state of mental confusion. Physical and neurologic examinations reveal hori- zontal diplopia, strabismus, amblyopia, nystagmus, ataxia, and peripheral neuropathy. The patient subsequently devel- ops lobar pneumonia and expires. Examination of the brain at autopsy shows calcifi cation and brownish discoloration of atrophic mammillary bodies. Petechiae in the quadrigeminal plate and periaqueductal regions of the midbrain are also observed. Which of the following best explains the pathogen- esis of these clinical and pathologic fi ndings?

Q60896. A 15-year-old boy is rushed to the emergency room after suffering a tonic-clonic seizure 4 weeks after a spelunking expedition. The boy appears irritable and agitated, and his parents state that he has diffi culty swallowing fl uids. Lumbar puncture shows numerous lymphocytes. The patient becomes delirious, slips into a coma, and expires. At autopsy, the brain stem shows infi ltrates of lymphocytes around small blood ves- sels and evidence of neuronophagia. Some neurons contain eosinophilic inclusions. What is the proper name for these neuronal inclusions?

Q60897. A 45-year-old woman is rushed to the emergency room following an automobile accident. Ten hours after admission, the patient complains of a severe headache and blurred vision. An X-ray fi lm of the cranium shows a fracture of the temporal- parietal bone. Despite emergency craniotomy, the patient dies. Which of the following pathologic fi ndings would be expected at autopsy?

Q60898. A 68-year-old woman complains of diffi culty getting out of a chair. On examination, the patient shows reduced facial expression, a resting tremor, cogwheel rigidity, and bradyki- nesia (slowness of voluntary movements). The patient dies of congestive heart failure 10 years later. Microscopic exami- nation of brain tissue at autopsy is shown in the image. The spherical, eosinophilic inclusions in the cytoplasm of this pigmented neuron are composed of which of the following proteins?

Q60899. A 50-year-old woman presents with a 3-month history of easy fatigability, a smooth sore tongue, numbness and tingling of the feet, and weakness of the legs. The hemoglobin is 5.6 g/dL, WBC count is 5,100/mL, and platelets are 240,000/mL. A hematologic evaluation reveals a megaloblastic anemia that is not reversed by folate therapy. Peripheral neuropathy in this patient is most likely associated with which of the following pathologic fi ndings?

Q60900. An 18-year-old man suffers massive trauma in a motorcycle accident. A CT scan shows multiple intracerebral hemorrhages. The patient expires after 6 months in a coma. At autopsy, there are cystic cavities within the frontal and temporal lobes, cor- responding to the areas of prior hemorrhage. These cavities were formed in large measure due to the phagocytic activity of which of the following cell types?

Q60901. A 22-year-old boxer suffers a concussion during a boxing match and is rushed to the emergency room. According to his trainer, the blow defl ected his head upward and posteriorly. Loss of consciousness in this patient presumably occurred because of a functional paralysis of neurons in which of the following anatomic regions of his brain?