AII-type: Select the most appropriate answer
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1 AII-type: Select the most appropriate answer ( )1. Choose one best answer for the following pathologic pictures. A. choroid cyst B. choroid papilloma C. pontine glioma D. ependymoma E. metastatic tumor of squamous cell carcinoma ( )2. A 29 year-old male patient had fever, malaise, and a feeling of weakness lasting 1 day. Flaccid paralysis of the lower extremities without sensory or bulbar involvement was noted in 10 days. The pathology of spinal cord was shown in figure. Which is the best diagnosis? A. acute spinal cord compression B. subacute sclerosing panencephalitis C. poliomyelitis D. Japanese envephalitis E. tropical spastic paraparesis
2 ( )3. A 50 year-old female complained of headache at night with vomiting. The brain CT was shown in figure. Which is the best diagnosis? A. metastatic lesion B. astrocytoma C. meningioma D. oligodendroglioma E. osteoma ( )4. 76 year-old male had a large cerebellar tumor which pathologic findings are showed in the following figures. Which is the best diagnosis? A. hemangioblastoma B. astrocytoma C. metastatic tumor D. mengioma E. ependymoma
3 ( )5. Choose the vessel supplying the lesion in the brain stem. A. anterior inferior cerebellar artery B. superior cerebellar artery C. posterior spinal artery D. anterior spinal artery E. posterior inferior cerebellar artery
4 ( )6. A 27 year-old teacher, Mr. Lin, had progressive weakness of intrinsic muscles of his feet and hand since the childhood. Some of his family member got the similar problem as shown in the following pedigree. He received a nerve biopsy. The electromicroscopic examination is shown in the following also. Which one is not correct? A. Charcot-Marie-Tooth disease, type I is the most likely diagnosis. B. The molecular genetic changes may be mutations of PMP22, Po or connexin 32. C. This is an autosomal dominant inheritance. D Steroid can improve the patient s weakness E. The onion formation is made of Schwann cell proliferation
5 ( )7. From the following pathological findings, which disease is the most appropriate diagnosis? A. Muscular dystrophy B. Charcot-Marie-Tooth disease C. Spinal muscular atrophy D. Polymyositis E. Mitochondrial disorde H&E stain ATPase ph 9.4 stain ( )8. From the following pathological findings, which disease is the most appropriate diagnosis? A. Mitochondrial myopathy B. Nemaline rod myopathy C. Polymyositis D. Central core myopathy E. Centronuclear myopathy Gomori trichrome
6 ( )9. From the following pathological findings, which disease is the most appropriate diagnosis? A. Inclusion body myositis B. Mitochondrial myopathy C. Ragged red myopathy D. Amyloidosis E. Polymyositis Congo red stain Congo red stain (Polarized light) ( )10. From the following pathological findings, which disease is the most appropriate diagnosis? A. Glycogen storage disease B. Lipid storage disease C. Polymyositis D. Congenital myopathy E. Periodic paralysis H&E Muscle biopsy stained with PAS
7 ( )11. From the following pathological findings, which disease is the most appropriate diagnosis? A. Glycogen storage disease B. Inclusion body myositis C. Polymyositis D. Congenital myopathy E. Periodic paralysis Gomori trichrome Congo red stained muscle fibers
8 ( )12. From the following pathological findings, which disease is the most appropriate diagnosis? A. Chronic inflammatory demyelinating polyneuropathy (CIDP) B. Acute inflammatory demyelinating polyneuropathy (AIDP) C. Fabry disease D. Amyloidosis E. Charcot-Marie-Tooth disease Toluidine blue Electron micrograph ( ) year-old woman developed proximal weakness and dysphagia. Nodules as shown in this figure were found on knees and elbow. There were also arthritis and skin rash. Which is the most likely diagnosis? A. Subacute bacterial endocarditis B. Polymyositis C. Dermatomyositis D. Inclusion body myositis E. Steroid myopathy
9 ( )14. Which one of the following descriptions is most suitable for the wave forms inside the circle during sleep? A. Mostly appears in sleep stage I B. Originates from thalamus C. Always follows a sharp waves D. Indicates a pathological process for most of the conditions E. None of the above is correct
10 ( )15. The 58-year-old patient was a case of adenocarcinoma of lung which had been diagnosed 8 months ago and received operation and chemotherapy. The patient suffered from acute onset of pain over both legs and acute urine retention. The lumbosacral MRI was shown. Which of the following is less likely presented? A. Early sphincter dysfunction (urinary incontinence) B. Predisposing symmetric presentation C. Tendon reflex is usually normal at knee and absent at ankle D. Spastic paraplegia E. Impotence T1 with gadolinium T1 with gadolinium
11 ( )16. A 21-year-old female patient had an episode of involuntary movement of right limbs for about 1-2 minutes. Then she passed out. The duration is about 5 minutes. The following pictures are the clinical features of this patient. Her disease may be caused by A. Mutation in an oncogene B. Mutation in a tumor suppressor gene C. Chemical injury D. Nutritional dificiency E. Drug-induced side effect
12 ( )17. The 42-year-old male was quite well in the past. He suffered from vomiting twice on & Transient conscious lapse without convulsion was noted on the morning of Episodic chewing, tongue protruding, visual hallucination and strange behavior (wandering, irritability, murmuring) developed on the evening of Then high fever was noted since the midnight. Brain MRI was shown. Which is the most likely diagnosis? A. Herpes simplex infection B. Varicella zoster infection C. Subacute sclerosing panencephalitis D. Neurosyphilis E. Tumor matastasis
13 ( )18. A 20 year-old man has mental retardation, right exotropia and kyphoscoliosis from childhood. Severe muscle wasting of limbs has been noted for one year. His appearance and CT scan of cervical spine are shown in figures. Which is the best diagnosis? A. Lipid storage disease B. Glycogen storage disease C. Mitochondrial disease D. Niemann-Pick disease E. Might associated with severe sensorimotor demyelinating neuropathy Mid-back of the patient
14 ( )19. A 12 year-old girl has scoliosis and wasting of four limbs since chilhood. No family history can be traced. Her clinical manifestations is not responsive to steroid. Her nerve conduction study and sural nerve biopsy are showed as below: Motor conduction study Nerves Distal latency (onset) (ms) NCV (m/s) Amplitude (mv) F-latency (ms) R median n Non-pick up R ulnar n Non-pick up R peroneal n Non-pick up L peroneal n Non-pick up R tibial n Non-pick up Her sensory conduction study showed no response at bilateral sural, median, ulnar and radial nerves. What is the most likely diagnosis? A. Peripheral myelin protein-22 duplication (CMT1A) B. Peripheral myelin protein-22 deletion (HNPP) C. Peripheral myelin protein Zero point mutation (CMT1B) D. Congenital insensitive to pain and anhidrosis (HSAN IV) E. Connexin 32 point mutation (CMTX)
15 ( ) year-old young man has numbess of both feet and mild weakness for uncertain years. His elder brother has same problem. They both are house-painters ( 油漆工 ). His nerve conduction study is showed as below: Motor conduction study Nerves Distal latency (onset) (ms) NCV (m/s) Amplitude (mv) F-latency (ms) R median n L median n R ulnar n Across elbow L ulnar n Across elbow R peroneal n Across knee L peroneal n Across knee R tibial n L tibial n Sensory conduction study Nerves Distal latency (onset) (ms) NCV (m/s) Amplitude (µv) R sural n L sural n R radial n L radial n R median n L median n R ulnar n L ulnar n What is the most likely diagnosis? A. Arsenic intoxication B. Sensory chronic inflammatory demyelinating polyneuropathy C. Lead poison D. Hereditary neuropathy with liability to pressure palsy E. Charcot-Marie-Tooth disease X-linked
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