Pathology of CNS Degenerations - In class Quiz

1. Pathology of CNS Degenerations - In class Quiz CNS Degenerative disorders… …To leave the world a better place. To know even one life has breathed easier because you have lived… that is success..! -- Ralph Waldo Emerson 1

2. 52y male, dementia, depression with choreiform movements.Image of his brain(B) compared with normal (A) ?DiagnosisA. Wernicke’s enceph. A BB. Parkinsons diseaseC. Alzheimers diseaseD. Pick’s diseaseE. Huntington’s disease 100% 0% 0% 0% 0% A. B. C. D. E. 52y male, dementia, depression with choreiform movements.Image of his brain(B) compared with normal (A) ?DiagnosisA. Wernicke’s enceph. A BB. Parkinsons diseaseC. Alzheimers diseaseD. Pick’s diseaseE. Huntington’s disease 100% 0% 0% 0% 0% A. B. C. D. E.

3. 38y male progressive weakness, spasticity, fasciculations and loss of muscle mass. Image shows section of spinal cord stained for myelin. Diagnosis?1. Multiple sclerosis2. Amyotrophic Lateral Sclerosis3. Motor neurone disease4. Syringomyelia5. Spinal cord infarction. 80%20% 0% 0% 0% 1 2 3 4 5 38y male progressive weakness, spasticity, fasciculations and loss of muscle mass. Image shows section of spinal cord stained for myelin. Diagnosis?1. Multiple sclerosis2. Amyotrophic Lateral Sclerosis3. Motor neurone disease4. Syringomyelia5. Spinal cord infarction. 80%20% 0% 0% 0% 1 2 3 4 5

4. 38y recurrent limb weakness 9m, MRI ?Diagnosis1. Multiple sclerosis2. Parkinsons3. Alzheimer’s4. Huntington’s5.100% Pick’s disease 0% 0% 0% 0% 1 2 3 4 5 38y recurrent limb weakness 9m, MRI ?Diagnosis1. Multiple sclerosis2. Parkinsons3. Alzheimer’s4. Huntington’s5.100% Pick’s disease 0% 0% 0% 0% 1 2 3 4 5

5. 58y male personality change, depression, with chorea. ? Diagnosis1. Multiple sclerosis2. Parkinsons3. Alzheimer’s4. Huntington’s5. Pick’s disease 60% 30% 10%0% 0%1 2 3 4 5 58y male personality change, depression, with chorea. ? Diagnosis1. Multiple sclerosis2. Parkinsons3. Alzheimer’s4. Huntington’s5. Pick’s disease 60% 30% 10%0% 0%1 2 3 4 5

6. A 65 year old woman with 3y history of emotional disturbances, irritability,irresponsible and difficulty with activities of daily living. Now she has difficulty talking.Image shows appearance of her brain. What is the most likely diagnosis?A. Huntington’s disease.B. Alzheimer’s disease.C. Pick’s disease.D. Parkinson’s diseaseE. Multiinfarct dementia. 80% 20% 0% 0% 0% A. B. C. D. E.A. Briefly discuss pathogenesis of this condition?B. List 3 gross and Microscopic feature of this disease?C. List 3 differentiating features of Alzheimers & this disease?. A 65 year old woman with 3y history of emotional disturbances, irritability,irresponsible and difficulty with activities of daily living. Now she has difficulty talking.Image shows appearance of her brain. What is the most likely diagnosis?A. Huntington’s disease.B. Alzheimer’s disease.C. Pick’s disease.D. Parkinson’s diseaseE. Multiinfarct dementia. 80% 20% 0% 0% 0% A. B. C. D. E.A. Briefly discuss pathogenesis of this condition?B. List 3 gross and Microscopic feature of this disease?C. List 3 differentiating features of Alzheimers & this disease?.

7. A 56-year-old man is rushed to the emergency room aftercollapsing while shoveling snow. The patient has no pulseon admission but is resuscitated. Laboratory studies showelevated serum levels of cardiac-specific proteins, and ECGconfirms a transmural infarct of the left ventricle. The patientexpires 2 weeks later of cardiac tamponade. Examination ofthe patients brain at autopsy would most likely revealnecrosis of Purkinje cells and selective loss of neurons inwhich of the following regions? 70% A. Frontal lobes B. Hippocampus C. Hypothalamus 20% D. Occipital lobes 10%0% 0% E. ThalamusA. B. C. D. E. A 56-year-old man is rushed to the emergency room aftercollapsing while shoveling snow. The patient has no pulseon admission but is resuscitated. Laboratory studies showelevated serum levels of cardiac-specific proteins, and ECGconfirms a transmural infarct of the left ventricle. The patientexpires 2 weeks later of cardiac tamponade. Examination ofthe patients brain at autopsy would most likely revealnecrosis of Purkinje cells and selective loss of neurons inwhich of the following regions? 70% A. Frontal lobes B. Hippocampus C. Hypothalamus 20% D. Occipital lobes 10%0% 0% E. ThalamusA. B. C. D. E.

8. A 28 year old woman with ataxia, diplopia on lateral gaze and flashes of light on eyemovement. CSF analysis shows increased proteins, lymphocytes and oligoclonal IgGbands and normal glucose. Image shows gross specimen of brain with lesions typicalof this disease. What is the most likely diagnosis?A. Parkinson’s diseaseB. MCA infarct.C. Lacunar Infarcts.D. Multiple sclerosis.E. Viral encephalitis. 56% 22% 11% 11% 0% A. B. C. D. E.A. Briefly discuss etiology & pathogenesis of this condition?B. Describe the gross features shown in the image?C. List other clinical features? What is the prognosis of this condition? A 28 year old woman with ataxia, diplopia on lateral gaze and flashes of light on eyemovement. CSF analysis shows increased proteins, lymphocytes and oligoclonal IgGbands and normal glucose. Image shows gross specimen of brain with lesions typicalof this disease. What is the most likely diagnosis?A. Parkinson’s diseaseB. MCA infarct.C. Lacunar Infarcts.D. Multiple sclerosis.E. Viral encephalitis. 56% 22% 11% 11% 0% A. B. C. D. E.A. Briefly discuss etiology & pathogenesis of this condition?B. Describe the gross features shown in the image?C. List other clinical features? What is the prognosis of this condition?

9. 38y female recurrent limb weakness. Image shows white mater stained for myelin.? Diagnosis A. Multiple sclerosis B. Parkinsons C. Alzheimer’s D. Huntington’s E. Pick’s disease100% 0% 0% 0% 0%A. B. C. D. E. 38y female recurrent limb weakness. Image shows white mater stained for myelin.? Diagnosis A. Multiple sclerosis B. Parkinsons C. Alzheimer’s D. Huntington’s E. Pick’s disease100% 0% 0% 0% 0%A. B. C. D. E.

10. . 72y Male progressive severe dementia,Image shows amyloid stain of his brain.? Diagnosis1. Pick’s disease.2. Huntington’s disease.3. Alzheimer’s disease.4. Multi-infarct dementia.5. Senile dementia. 100% 0% 0% 0% 0% 1 2 3 4 5

11. . 38y fem recurrent tremor & limb weakness progressive over 13 years. ? Diag1. Multiple sclerosis2. Hypertensive-stroke.3. Alzheimer’s4. Huntington’s5. Pick’s disease89% 11% 0% 0% 0%1 2 3 4 5

12. . A 48 year old man with a year long history of worsening jerky, choreo-athetoidmovements of all limbs, bradykinesia and rigidity. Image shows coronal section of abrain specimen showing typical features of his disease. What is the most likelydiagnosis?A. Picks disease.B. Lewy body disease.C. Huntington’s disease.D. Multiple sclerosis.E. Amyotropic lateral sclerosis. 67% 33% 0% 0% 0% A. B. C. D. E.A. Briefly discuss pathogenesis of this condition? (Trineucleotide repeat dis)B. Describe the gross features shown in the image?C. what other clinical features are expected in this disease?

13. . A 60-year-old man with a 15-year history of diabetes mellitustype II complains of deep burning pain and sensitivity totouch over his hands and fingers. Nerve conduction studiesshow slow transmission of impulses and diminished musclestretch reflexes in both ankles and knees. Sensations tovibrations and light touch are also markedly diminished.Laboratory analysis of CSF shows no biochemicalabnormalities. Which of the following is the most likely typeof peripheral nerve disease in this patient? 55% A. Autonomic neuropathy B. Distal polyneuropathy 36% C. Inflammatory neuropathy D. Mononeuropathy 9% E. Paraproteinemic 0% 0% polyneuropathy A. B. C. D. E.

14. . 58y M, Progressive bradykinesia, dementia & Hallucinations ? DiagnosisA. Alzheimer’sB. Parkinson’sC. Motor neurone diseaseD. SyringomyeliaE. Lewy body disease 50% 40%10% 0% 0%A. B. C. D. E.

15. . A 35-year-old man with Down syndrome dies of acutelympho-blastic leukemia. Gross examination of the patientsbrain at autopsy shows mild microcephaly andunderdevelopment of the superior temporal gyri. Histologicexamination would most likely show which of the followingneuropathologic changes? 60% A. AA amyloidosis B. Lewy body dementia C. Leukemic infiltrates 20% D. Neurofibrillary tangles 10% 10% E. Old healed infarcts. 0% A. B. C. D. E.

16. . A 35 year old HIV +ve man with Kaposisarcoma has a 3 day history of headache andconfusion followed by seizures and hemiparesis. CT scan showed multiple ring-enhancing lesions. Image shows Gross appearance and MRI of a brain typical of thisdisorder. What is the most likely diagnosis?A. Multiple Aspergilloma.B. Cerebral TB lesions.C. Multiple Abscesses.D. Multiple sclerosis plaques.E. Cerebral metastases. 40% 30% 30% 0% 0% A. B. C. D. E.A. What does “ring enhancing” lesion mean?B. Briefly discuss etiology and pathogenesis?C. List other pathologic findings expected in the brain of a AIDS patiets?.

17. . 38y fem, recurrent attacks of limb weakness, paraesthesia worsened over 7 years resulting in death due to respiratory failure. Image shows her brain specimen. What is the most likely Diagnosis?1. Multiinfarct dementia.2. Parkinsons disease.3. Multiple sclerosis4. Motor neurone disease.5. Lacunar infarcts. 90% 10%0% 0% 0%1 2 3 4 5

18. . 78y M, Dementia, memory loss and spacial disorientation. Cortical biopsy. ? Diagnosis 1. Alzheimer’s 2. Parkinson’s 3. Lewy body disease 4. Huntington’s disease 5. Pick’s disease100% Cortical biopsy - β amyloid immunostain 0% 0% 0% 0%1 2 3 4 5

19. . 78y, Dementia, Aphasia, Image showsHippocampus biopsy stained with anti tau stain.? DiagnosisA. Multiple sclerosisB. ParkinsonsC. Alzheimer’sD. Multi-infarct dementiaE. Pick’s disease 55% 36%9% 0% 0%A. B. C. D. E.

20. . 78y Male progressive severe dementia,Image shows amyloid stain of his brain.? Identify structure A1. Neurofibrillary tangles.2. Amyloid plaques.3. Amyloid Angiopathy4. Astrocytes - Gliosis.5. Neuronophagia. 90% 10% 0% 0% 0% 1 2 3 4 5

21. . 58y female vocalist difficulty remembering songs, depression, dementia & gait abnormality. Image shows brain biopsy. What is the most likely diagnosis? 1. Alzheimer’s disease. 2. Pick’s disease. 3. Huntington’s disease. 4. Lewy body disease. 5. Creutzfeldt-Jakob disease. 90%CJD: Spongiform degeneration (vacuoles) no inflammation - Slowlyprogressive ataxia and dementia.Prion protein (APrP amyloid) – form template to make more abnormal protein(not a living being) Infection  From contaminated brain of infected animal. 10%Prion0% diseases: Kuru, CJD, Scrapie, and bovine spongiform encephalopathy 0% 0%(mad cow disease). 1 2 3 4 5

22. . 30y F, 7 day history of mild tremor in her arms and impaired balance whenwalking. Vital signs are normal. Her symptoms disappear the following week, butrecures several times over next 4 years gradually developed weakness requiringassistance when walking. Neurologic examination reveals ataxia, dysarthria,decreased vibratory sensation, increased deep tendon reflexes, and a +veBabinski sign on the left. Fifteen years after the onset of symptoms, the patientbecomes bedridden and dies. A coronal section of the patients brain at autopsy isstained for myelin (shown). Which of the following histopathologic findings wouldbe expected in the lesions seen in the patients brain? A. Astrogliosis B. Lewy bodies C. Myelin loss. D. Neuritic plaques E. Neuronophagia. 100% 0% 0% 0% 0% A. B. C. D. E.

23. . What is the best clinical description for the image?1. 83y man – dementia, memory loss and disorientation.2. 43y man -abnormal movements who committed suicide3. 56y woman -progressive paralysis4. 60y man -tremor, rigidity and dementia5. 73y woman –Dementia aphasia but intact memory. 67%17% 17% 0% 0% 1 2 3 4 5

24. . What is the best clinical description for the image?AD1. 83y man – dementia, memory loss and disorientation.HD2. 43y man -abnormal movements who committed suicideALS 3. 56y woman -progressive paralysisPD4. 60y man -tremor, rigidity and dementiaPick 5. 73y woman –Dementia aphasia intact memory. 71% 14% 14% 0% 0% 1 2 3 4 5

25. . CNS Degenerative disorders…A 56 year old man develops 2 year history of increasing hand and forearm weaknessand clumsiness. Later develops dysarthria and dysphagia. A year later he dies ofpneumonia. Image shows two sections of spinal cord with arrows showing thepathology. What is the most likely diagnosis? MND .. many subtypes: • Amyotrophic Lateral Sclerosis.A. Parkinsonism. • Progressive muscle atrophy (LMN)B. Multiple Sclerosis. • Primary lateral sclerosis (UMN) • Progressive bulbar palsyC. Vitamin B12 deficiency (CDC)D. Motor Neurone Disease (MND)E. Amyotrophic Lateral Sclerosis (ALS) 0% 0% 0% 0% 0% A. B. C. D. E.A. Briefly describe pathological features seen in the image?B. Explain the symptoms? What is the prognosis?C. List one microscopic feature?

26. . CNS Degenerative disorders…CPC-3.6– KFP Questions: Dementia – Definition, Clinical, types. Primary:  Alzheimers, Picks, Parkinson’s & Diffuse Lewy Body Dementia, Huntingtons. Secondary:  CVA, Infections (meningitis), Neoplasms, hydrocephalus, Drugs and toxins, metabolic, vitamin def. (e.g. B1, B2, B12). Demyelinating disorders:  Multiple sclerosis, MND, 26

27. . The only real mistake is the one from which we learn nothing! JOHN POWELL: