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Alzheimer Disease Part One: Each item below contains a question or incomplete statement followed by suggested responses. Select the one best response to each question. 1. A 75-year-old woman with suspected normal-pressure hydrocephalus undergoes lumbar puncture. Forty milliliters of fluid are removed. Three hours later, she is able to walk unassisted and turns well. Spinal fluid would be expected to show which of the following? a. No abnormalities b. Elevated protein c. Low protein d. Atypical lymphocytes e. Low glucose 2. A physician believes that her patient has Alzheimer’s disease. Which of the following is most characteristic of the brain in patients with Alzheimer’s disease? a. Neuronal loss in the cerebral cortex b. Demyelination in the cerebral cortex c. Posterior column degeneration d. Neuronal loss in the cerebellar cortex e. Pigmentary degeneration in the hippocampus 3. An 80-year-old man has had a gradual memory decline over the past 10 years. A reversible cause of dementia cannot be found, and PET scan supports the diagnosis of Alzheimer’s disease. In the dementia associated with Alzheimer’s disease, the EEG will usually show which of the following? a. Spike-and-wave discharges b. Periodic frontal lobe discharges c. Focal slowing d. Generalized background slowing e. An isoelectric record 4. A 55-year-old man has a steep decline in his cognitive abilities over a 3-month period. Initial testing is nondiagnostic. He continues to progress and develops myoclonus and a left hemiparesis. Eventually, he dies of an aspiration about 8 months after the onset of symptoms. In the diseases that cause dementia, myoclonus is usually most evident in which of the following? a. Alzheimer’s disease b. Creutzfeldt-Jakob disease c. Parkinson’s disease d. Huntington’s disease e. Pick’s disease 5. A 29-year-old mentally retarded woman living in an institution has had a subacute to chronic decline in memory. Testing for reversible causes of dementia is nondiagnostic. The brain of the adult with trisomy 21 (Down syndrome) exhibits many of the histopathologic features of which of the following? a. Tay-Sachs disease b. Friedreich’s disease c. Pick’s disease d. Parkinson’s disease e. Alzheimer’s disease 6. An 80-year-old man has a history of 2 years of progressive gait disturbance and incontinence, which had been attributed to old age and prostatism. Within the past 3 months, he has been forgetful, confused, and withdrawn. His gait is short-stepped, and he turns very slowly, almost toppling over. He has a history of head trauma from 30 years ago. His CT scan is shown below. Which of the following is the most likely diagnosis? a. Alzheimer’s disease b. Creutzfeldt-Jakob disease c. Progressive multifocal leukoencephalopathy (PML) d. Normal-pressure hydrocephalus e. Chiari malformation 7. An 82-year-old man has 6 months of worsening memory loss. His family is concerned, and he is taken to a physician. Following an extensive evaluation and neuropsychological testing, he is diagnosed with dementia. Which of the following is the most common cause of dementia in the general population? a. Epilepsy b. Vascular disease c. Alzheimer’s disease d. Parkinson’s disease e. Head trauma 8. A patient undergoes ventriculoperitoneal shunt placement for hydrocephalus. He is discharged 2 days later, his gait and cognition much improved. The following morning, his wife finds him lying in bed, very confused and complaining of a headache. He is unable to walk. The surgeon who performed the procedure is concerned that these new symptoms are due to which of the following? a. Chemical meningitis b. Subdural hematoma c. Epidural hematoma d. Seizures e. Bacterial ventriculitis 9. A 67-year-old man has a history of progressive memory loss for 2 years. His examination is otherwise normal. A diagnosis of Alzheimer’s disease is made. Which of the following medications may result in some cognitive improvement? a. Donepezil b. L-dopa c. Risperidone d. Prednisone e. Vitamin B12 10. Language testing is most likely to uncover which of the following deficits in a patient with Alzheimer’s disease? a. No abnormalities b. Mutism c. Conduction aphasia d. Transcortical sensory aphasia e. Transcortical global aphasia 11. A 73-year-old man steps out of the shower on a Saturday evening and is unable to remember that he and his wife have tickets to a play. He asks her repeatedly, “Where are we going?” He appears bewildered, but is alert, knows his own name, speaks fluently, and has no motor deficits. He has no history of memory disturbance, and after 8 h returns to normal. What the most likely diagnosis is? a. Transient global amnesia b. Normal-pressure hydrocephalus c. Alzheimer’s disease d. Parkinson’s disease e. Creutzfeldt-Jakob disease 12. A 50-year-old woman began having double vision and blurry vision 3 months ago and has since had diminishing interaction with her family, a paucity of thought and expression, and unsteadiness of gait. Her whole body appears to jump in the presence of a loud noise. An MRI scan and routine CSF examination are unremarkable. What the most likely diagnosis is? a. Transient global amnesia b. Normal-pressure hydrocephalus c. Alzheimer’s disease d. Parkinson’s disease e. Creutzfeldt-Jakob disease 13. A 2-year-old girl developed normally until the past year. She has since become unable to speak or otherwise communicate with her parents, sits in a chair, and makes nearly continuous wringing movements with her hands. She also has episodes of breath holding alternating with hyperventilation. What the most likely diagnosis is? a. Vitamin B12 deficiency b. Hypothyroidism c. Huntington’s disease d. Rett syndrome e. Multi-infarct dementia 14. A 17-year-old girl develops mild dementia, tremor, and rigidity. Her father died in his fourth decade of life of a progressive dementing illness associated with jerking (choreiform) limb movements. On exposure to L-dopa, she becomes acutely agitated and has jerking limb movements. What the most likely diagnosis is? a. Vitamin B12 deficiency b. Hypothyroidism c. Huntington’s disease d. Rett syndrome e. Multi-infarct dementia 15. A 62-year-old man has had 2 years of progressive memory loss and inappropriate behavior. He has been delusional. More recently, he has developed tremors, myoclonus, dysarthria, and unsteadiness of gait. The CSF shows a lymphocytic pleocytosis, protein of 150, and positive VDRL. What the most likely diagnosis is? a. Huntington’s disease b. Rett syndrome c. Multi-infarct dementia d. General paresis e. Temporal lobe epilepsy 16. A 44-year-old woman from Africa presents with inattentiveness, poor concentration, and lethargy. She has paranoid delusions. There is mild proximal weakness and ataxia. On general exam, she has edema, coarse and pale skin, and macroglossia. On reflex examination, she has delayed relaxation of the ankle reflexes. What the most likely diagnosis is? a. Vitamin B12 deficiency b. Hypothyroidism c. Huntington’s disease d. Rett syndrome e. Multi-infarct dementia 17. A 54-year-old woman presents with 6 months of progressive memory loss. She has limited vertical eye movements, and on examination she has rhythmic, synchronous grimacing and eye closure movements (oculomasticatory myorhythmia). Jejunal biopsy reveals PAS-positive cells. What the likely organism that caused the disease is? a. HTLV-I b. Tropheryma whippelii c. Treponema pallidum d. JC virus e. Prion protein 18. A 35-year-old intravenous drug abuser presents with inability to control his left hand. He reports that at times he will button his shirt with his right hand, only to find that his left hand is unbuttoning the shirt against his control. He has a history of thrush. He is alert and oriented. MRI shows an increased T2 signal affecting the subcortical white matter of the right parietal lobe without enhancement. What the likely organism that caused the disease is? a. HTLV-I b. Tropheryma whippelii c. Treponema pallidum d. JC virus e. Prion protein 19. A previously healthy 24-year-old man presents with 3 days of headaches and fever, followed by hallucinations, speech disturbance, and lethargy. He has a mild right hemiparesis. Spinal fluid is bloody, and MRI shows abnormal signal, with enhancement, in the left anterior temporal lobe. What the likely organism that caused the disease is? a. HTLV-I b. Tropheryma whippelii c. Herpes simplex virus d. JC virus e. Prion protein 20. Which is not the structure in the pathology of Alzheimer's disease? a. Senile plaque b. Cholinergic neuron loss c. Neuronal fiber entanglement d. Lewy body e. Vascular amyloidosis Part Two: Fill in the blanks 1. The diagnosis of Alzheimer's disease is based on . 2. In the early stage of Alzheimer's disease, the main manifestation is . 3. The grading scale for Alzheimer's disease diagnosis is . 4. The gross assessment scale for Alzheimer's disease diagnosis is . 5. The scale used for Alzheimer's disease and vascular dementia is . 6. The Psychiatric Rating Scale for Alzheimer's disease diagnosis is . 7. Granular degeneration of neurons was more common in . 8. The effective method for clinical diagnosis of Alzheimer's disease is . 9. The most common type of dementia in the population is . 10. Compared with vascular dementia and Alzheimer's disease, the more important cognitive impairment is . 11. The identification of Louis's dementia and Alzheimer's disease is mainly . 12. The characteristic pathological changes of Alzheimer's disease are 13. , The are 14. . main , The on clinical , manifestations Alzheimer's disease . diagnosis of , . , of Alzheimer's disease 15. Senile plaques are extracellular deposits of mainly rely in the grey matter of the brain. 16. The include principles , of treatment for Alzheimer's disease . 17. The characteristic clinical manifestation of Alzheimer's disease is . 18. Alzheimer's disease can be divided into and . 19. The types of dementia that need to be identified with Alzheimer's disease are , , . 20. The biggest difference between frontal and temporal dementia and Alzheimer's disease is , . Part Three: Definitions 1. Dementia 2. Alzheimer's disease (AD) 3. Paraphasia 4. Cognitive deficit 5. Senile plaques Part Four: General questions 1. The classic presentation of Alzheimer’s disease. 2. Please sketch the pathogenesis of Alzheimer’s disease. 3. Please sketch drugs used in the treatment of Alzheimer’s disease. 4. The classic presentation of dementia of Lewy bodies. 5. Please sketch the causes of dementia. Part Five: Case analysis An 80-year-old man has a history of progressive gait disturbance and incontinence, which had been attributed to old age and prostatism. Within the past 3 months, he has been forgetful, confused, and withdrawn. His gait is short-stepped, and he turns very slowly, almost toppling over. He has a 30-year history of head trauma. Now please answer several questions below: 1. What’s the most probable diagnosis; 2. How to treat his disease. 3. The classic presentation of his disease. Answers Part One: Choice questions 1 to 5: aadbe 6 to 10: d c b a d 11 to 15: aedcd 16 to 20: b b d c d Part Two: Fill in the blanks 1. Pathologic examination 2. Near memory loss 3. Clinical dementia rating scale (CDR) 4. Mini mental state examination scale (MMSE) 5. Hachinski ischemic scale 6. Hamilton Depression Scale (HAMD) 7. Alzheimer's disease 8. Clinical comprehensive analysis combined with cognitive assessment 9. Alzheimer's disease 10. performing function 11. Fluctuating cognitive impairment 12. Neuronal fiber entanglement, Senile plaque 13. Memory dysfunction, Apathy and paranoia, Difficult to find words 14. Neuropsychological examination, Detailed medical history, physical examination Neuroimaging examination 15. amyloid beta 16. Dementia treatment, Symptomatic treatment 17. Cognitive impairment 18. Sporadic type, Familial type 19. front temporal dementia, Vascular dementia, Louis's dementia 20. Frontal executive dysfunction, The relative preservation of visual function and memory function Part Three: Definitions 1. Dementia: Dementia is a broad category of brain diseases that cause a long term and often gradual decrease in the ability to think and remember that is great enough to affect a person's daily functioning. 2. Alzheimer’s disease: Alzheimer's disease is a chronic neurodegenerative disease that usually starts slowly and gets worse over time. 3. Paraphasia: Paraphasia is a type of language output error commonly associated with aphasia, and characterized by the production of unintended syllables, words, or phrases during the effort to speak. 4. Cognitive deficit: Cognitive deficit is an inclusive term to describe any characteristic that acts as a barrier to the cognition process. 5. Senile plaques: Senile plaques are extracellular deposits of amyloid beta in the grey matter of the brain. Part Four: General questions 1. A. Early manifestations: Impairment of recent memory is typically the first sign of Alzheimer’s disease. Aphasia, anomia, and acalculia may develop. B. Late manifestations: In the last stages, previously preserved social graces are lost, and psychiatric symptoms, including psychosis with paranoia, hallucinations, or delusions maybe prominent. Seizures occur in some cases. 2. A. Genetic: Such as trisomy 21, amyloid precursor protein on chromosome 21, presenilin 1 and 2. B. Role of β-amyloid: Amyloid β-protein is deposited in neuritic plaques and in cerebral and meningeal blood vessels of patients with Alzheimer’s disease. C. Neurochemical: The acetylcholine-synthesizing enzyme choline acetyltransferase is markedly depleted in the cerebral cortex and hippocampus of patients. Degeneration of the nucleus basalis of Meynert and of the cholinergic septal-hippocampal tract can be found. D: Toxic. E: Infectious. 3. Cognitive dysfunction: Acetylcholinesterase inhibitor including Tacrine and Donepezil. Behavioral disturbance: ⑴ Antipsychotic: Haloperdol ⑵ Antidepressant: Nortriptyline, Desipramine, Trazodone, Venlafaxine. ⑶ Anxiolytic: Buspirone, Lorazepam. 4. The features of dementia with Lewy bodies is fluctuating cognitive ability, well-formed visual hallucinations, and signs of parkinsonism, especially rigidity and bradykinesia. 5. Causes of dementia include cerebral disorders, with extrapyramidal features, systemic disorders (cancer, infection), metabolic disorders (alcoholism, hypothyroidism, Vitamin B12 deficiency), organ failure, trauma, vascular disorders, pseudodementia. Part Five: Case analysis 1. Dementia with Lewy bodies 2. Patients of dementia with Lewy bodies may respond well to anticholinestrase drugs such as tacrine or donepezil, but are especially sensitive to extrapyramidal side effect of antipsychotic drugs, which should therefore be avoided or used with caution. 3. The features of dementia with Lewy bodies is fluctuating cognitive ability, well-formed visual hallucinations, and signs of parkinsonism, especially rigidity and bradykinesia.