A. The most possible etiologies of this disease is a mitochondria disease. dominant inheritance pattern account for the diseases.

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1 AⅡ-type: Select ONE appropriate answer ( ) 1. Disturbance, slurred speech and metal deterioration since the age of 12. Currently, NE revealed marked dysarthria with relatively normal comprehension, gait difficulty with toe walking, ataxia, dystonic upper limbs and generalized hyperreflexia. The further brain MRI is performed and the T2-weight image revealed as follows. Which of the following description is CORRECT? A. The most possible etiologies of this disease is a mitochondria disease. B. In classic cases, a defect in the gene encoding pantothenate kinase 2 with autosomal dominant inheritance pattern account for the diseases. C. The spasticity and rigidity in this disease most prominent in the arm and in some instances, they begin in the bulbar muscles. D. Pathologically, presence of swollen axon fragment is a unique feature resembling those of neuroaxonal dystrophy. E. Chelating agents to reduce the iron storage is much helpful. 解答 : D 1

2 題目之出處 :Adam &Victor s principle of Neurology 9 th. Edition Chap. 37, P943 ( ) 2. Which term is the best one to describe the abnormal posture shown in the following photograph? A. Hyperekplexia B. Camptocormia C. Spasmodic torticollis D. Antecollis E. Myokymia 解答 : B 題目之出處 :Merritt s Neurology, 12th edition, p. 753 Principles of Neurology, 9th edition, p and p ( ) year-old female had left eye blurred vision then lost her visual acuity last year. She had band-like sensation over her trunk, lower limbs weakness and numbness one week ago, also she had constipation and urine retention. We check her serum with HEK-293 cells then get the image. Which is the LIKELY antibody to the image? A. 4-aminopyridine (a-ap) B. Anti-MOG antibody C. Anti-aquaporin 4 antibody 2

3 D. anti-nmda antibody E. Anti-MBP antibody 解答 : C ( ) 4. A 49-year-old male patient has insidious onset of slowly progressive distal limbs weakness for 15 years. The initial symptoms include easily got trapped on the floor, easily dropped slippers, and easily dropped keys from hands. About 8 years ago, he had decreased sensation to heat, cold and pain over bilateral distal hands and legs. Mild feet and hands deformity were also noted. He had diabetes mellitus for 2 years. His daughter had similar 3

4 symptoms since 18 years old. The DTR showed general areflexia. What is the MOST POSSIBLE tentative diagnosis? A. Chronic inflammatory demyelinating polyradiculoneuropathy B. Charcot-Marie-Tooth C. Porphyric Neuropathy D. Diabetic neuropathies E. Vasculitis Neuropathy 解答 : (B) 題目之出處 : NICP 5 th, p (2008) ( ) 5. A 56-year-old man with progressive dysphagia and dysarthria, and a prior history of proximal extremity weakness and fasciculations,particularly in the perioral area and the 4

5 tongue associated with tongue atrophy. Among the following descriptions, which one is WRONG? A. Abnormal CAG trinucleotide repeats B. Systemic manifestinations include diabetes mellitus C. Normal serum creatine kinase D. Electrodiagnostic testing reveals sensory neuronopathy E. Respiratory muscle weakness is rare 解答 : C 題目之出處 :NICP 5 th p2199~2201 5

6 ( ) 6. A 55-year-old man with hypertension was presented to emergency room due to right neck pain and dizziness. His T2-weighted brain MRI was as following. Which is the MOST POSSIBLE diagnosis? A. Atherosclerosis B. common carotid dissection C. internal carotid dissection, D. vertebral arterial dissection E. aneurysm Figure 解答 : C 題目之出處 :Neurology in Clinical Practice, 5th ed.,p.2021 ( ) 7. A 65 year-old woman who receives hemodialysis for 1 year due to chronic renal failure, suffered from sudden onset of acaroparethesia for 2 days. Her motor nerve conduction velocity study presented as below. Which of the following statements about this NCV is the MOST APPROPRIATE etiology? A. Chronic inflammatory demyelinating polyneuropathy B. Uremic polyneuropathy C. Acute inflammatory demyelinating polyneuropathy D. Mononeuropathy multiplex 6

7 E. Critical illness polyneuropathy 解答 : C ( ) 8. A 60-year-old female has had memory impairment and unsteady gait for 3 months. Her right upper and lower extremities shaked involuntarily, which were easily evoked by sounds. She became totally bedridden within 1 month. Neurological examination showed dysarthria, dysdiodochokinesia, and hypotonia. DWI MRI were shown below. What is the MOST APPROPRIATE assessment for this patient? A. Lumbar puncture and check protein B. Check serum glucose level C. Cerebral angiogram D. Brain biopsy E. Visual evoked potential 7

8 解答 : A 題目之出處 :Merritt s Neurology, 12 th edition, p. 244 ( ) 9. Which is the LIKELY diagnosis according to the following angiography? A. Moyamoya disease B. CNS Lupus C. fibromuscular dysplasia D. Left ICA Dissection E. Left P-Com aneurysm 8

9 解答 : A 題目之出處 :Neurology in Clinical practice, 5th edition, p.1186 ( ) 10. A 38 years old man, had history of migraine with aura, frequent transient TIA-like episode with right hemiparesis and dysarthria. His brain MRI is shown in figure (FLAIR), also had strong family history of stroke, and his blood pressure is relative high 140/92 mmhg, which the following test is your first choice? A. Oligoclonal band in CSF B. Cerebral angiography C. Tumor markers D. NOTCH3 gene test E. Skin biopsy for vascular sarcoidosis stain Figure 解答 : D 題目之出處 : Principle of Neurology, 9ed, PP:798 ( ) 11. A 65 y/o female patient presented with progressive consciousness disturbance, speechless and right limbs weakness for 3 weeks. Which is the MOST LIKELY diagnosis according to her brain image findings? A. Brain metastasis 9

10 B. Left PCA territory infarction C. Dural AV fistula complicated with sinus thrombosis and venous infarction D. Brain abscess E. posterior reversible leukoencephalopathy syndrome (PRES) 解答 : C ( ) 12. A 65-year-old male with fever, headache, seizure and altered consciousness and personality. According to the EEG, which one is the MOST APPROPRIAT treatment? A. Amphotericin B B. Acyclovir C. Trimethoprim-sulfamethoxazole 10

11 D. Corticosteroid E. Ceftriaxone 解答 : B 題目之出處 :Merritt s Neurology, 12 th edition, p ( ) 13. A 9-year-old boy with mental retardation and uncontrolled seizure. According to the distinctive clinical and EEG features, which of the following is the MOST LIKELY diagnosis? A. Childhood absence epilepsy B. West syndrome C. Lennox-Gastaut syndrome D. Juvenile myoclonic epilepsy E. Rasmussen syndrome 11

12 解答 : C 題目之出處 : Neurology in Clinical Practice, 5 th edition, p. 461 ( ) 14. The following pictures are the skin lesions of a 8-year-old boy with seizure, mental retardation. Which of the following is the MOST LIKELY diagnosis? A. Neurofibromatosis-1 B. Incontinentia pigmenti C. Sturge-Weber syndrome D. Tuberous sclerosis complex E. Neurofibromatosis-2 12

13 解答 : D 題目之出處 :Neurology in Clinical Practice, 5 th edition, p ( ) 15. Which of the statements concerning the EEG shown below is CORRECT? A. The subject is probably in stage 3 of sleep. B. The cerebral activities marked by the arrows are vertex sharp waves. C. The arrows indicate typical interictal epileptic discharges in subjects with benign Rolandic epilepsy (benign childhood epilepsy with centrotemporal spikes). D. Anticonvulsant treatment is indicated for this subject to avoid seizure attack E. Carbamazepine is the standard choice for subjects with this EEG pattern. 13

14 解答 : B ( ) 16. This is a 46-year-old man who has progressive left hand weakness for months. The EEG study showed the following feature. Among all the following description, which is the MOST LIKELY condition in this patient? A. Creutzfeldt-Jakob disease B. Uremic encephalopathy C. A tumor over right temporal and parietal lobe D. A tumor over left temporal and parietal lobe E. Hypoglycemia encephalopathy 14

15 解答 : C 題目之出處 : Neurology in Clinical Practice, 5 th edition, p. 463 ( ) 17. A 32-year-old man experienced headache while lifting heavy weight. The headache was throbbing and localized in the posterior region of the neck and bilateral parietal areas. The headache aggravated when he assumed an upright posture and alleviated when he lying down. What is the MOST LIKELY diagnosis? A. Tuberculosis B. Herpes Simplex Virus C. Spontaneous intracranial hypotension D. IICP headache E. Venous sinus thrombosis 15

16 解答 : C 題目之出處 : Acta Neurologica Taiwanica, Acta Vol 20, No 4, 2011 ( ) 18. A patient with 10-year history of progressive cerebellar ataxia, deafness, cognitive impairment, spasticity and xanthochromic CSF. Brain MRI showed the abnormality finding as the figure. Which one is CORRECT? A. Intracerebral hypotension B. Superficial siderosis C. Subarachnoid hemorrhage D. Susac syndrome E. Arteriovenous malformation 16

17 解答 : B 題目之出處 :Merritt s Neurology, 12 th ed, 2012, p.367 ( ) 19. A 62-years-old woman, non-alcoholism, presented memory loss with an amnestic syndrome and lost the way frequently for 1 month. Brain CT showed no remarkable finding. Brain MRI showed T1 hypointensity, homogenously contrast-enhancing hyperintensity and diffusion restriction in the splenium of corpus callosum. Among the following descriptions, which one is the MOST LIKELY diagnosis? A. Primary CNS lymphoma B. Extrapontine myelinosis C. Lipoma D. Multiple Sclerosis E. Marchiafava-Bignami disease 17

18 T1+Contrast DWI 解答 : (A) 題目之出處 :Merritt s Neurology, 12 th edition,p. 408 ( ) 20. A 60 year-old man presented with progressive numbness and weakness for 3 months from bilateral feet initially then spreading to bilateral upper limbs. EMG/NCV showed demyelinating sensori-motor polyneuropathy. Skull plain film was taken. Which is the MOST POSSIBLE diagnosis? A. Malignancy with paraneoplastic syndrome and bony metastasis B. Osteitis deformans (Paget disease) C. Multiple myeloma D. Neurosarcoidosis E. Achondroplasia 18

19 解答 : C 題目之出處 :Merrit s Neurology 11 TH, p