pathology of cns degenerations - in class quiz

CNS Degenerative disorders…

1

…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

52y male, dementia, depression with choreiform movements.

Image of his brain(B) compared with normal (A) ?Diagnosis

A B

A. B. C. D. E.

0% 0%

100%

0%0%

A. Wernicke’s enceph.

B. Parkinsons disease

C. Alzheimers disease

D. Pick’s disease

E. Huntington’s disease

38y male progressive weakness, spasticity, fasciculations and loss of muscle mass. Image shows section of spinal cord stained for myelin. Diagnosis?

1 2 3 4 5

20%

80%

0%0%0%

1. Multiple sclerosis

2. Amyotrophic Lateral Sclerosis

3. Motor neurone disease

4. Syringomyelia

5. Spinal cord infarction.

38y recurrent limb weakness 9m, MRI ? Diagnosis

1 2 3 4 5

100%

0% 0%0%0%

1. Multiple sclerosis

2. Parkinsons

3. Alzheimer’s

4. Huntington’s

5. Pick’s disease

58y male personality change, depression, with chorea. ? Diagnosis

1 2 3 4 5

0%

30%

10%

60%

0%

1. Multiple sclerosis

2. Parkinsons

3. Alzheimer’s

4. Huntington’s

5. Pick’s disease

A. B. C. D. E.

0%

20%

0%0%

80%

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. 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. Huntington’s disease.

B. Alzheimer’s disease.

C. Pick’s disease.

D. Parkinson’s disease

E. Multiinfarct dementia.

A 56-year-old man is rushed to the emergency room after collapsing while shoveling snow. The patient has no pulse on admission but is resuscitated. Laboratory studies show elevated serum levels of cardiac-specific proteins, and ECG confirms a transmural infarct of the left ventricle. The patient expires 2 weeks later of cardiac tamponade. Examination of the patient's brain at autopsy would most likely reveal necrosis of Purkinje cells and selective loss of neurons in which of the following regions?

A. B. C. D. E.

0%

20%

70%

0%

10%

A. Frontal lobes

B. Hippocampus

C. Hypothalamus

D. Occipital lobes

E. Thalamus

A 28 year old woman with ataxia, diplopia on lateral gaze and flashes of light on eye movement. CSF analysis shows increased proteins, lymphocytes and oligoclonal IgG bands and normal glucose. Image shows gross specimen of brain with lesions typical of this disease. What is the most likely diagnosis?

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. B. C. D. E.

11%

0%

22%

56%

11%

A. Parkinson’s disease

B. MCA infarct.

C. Lacunar Infarcts.

D. Multiple sclerosis.

E. Viral encephalitis.

38y female recurrent limb weakness. Image shows white mater stained for myelin.? Diagnosis

A. B. C. D. E.

100%

0% 0%0%0%

A. Multiple sclerosis

B. Parkinsons

C. Alzheimer’s

D. Huntington’s

E. Pick’s disease

72y Male progressive severe dementia, Image shows amyloid stain of his brain. ? Diagnosis

1 2 3 4 5

0% 0% 0%0%

100%

1. Pick’s disease.

2. Huntington’s disease.

3. Alzheimer’s disease.

4. Multi-infarct dementia.

5. Senile dementia.

38y fem recurrent tremor & limb weakness progressive over 13 years. ? Diag

1 2 3 4 5

89%

0% 0%0%

11%

1. Multiple sclerosis

2. Hypertensive-stroke.

3. Alzheimer’s

4. Huntington’s

5. Pick’s disease

A. B. C. D. E.

0%

33%

0%0%

67%

A 48 year old man with a year long history of worsening jerky, choreo-athetoid movements of all limbs, bradykinesia and rigidity. Image shows coronal section of a brain specimen showing typical features of his disease. What is the most likely diagnosis?

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?

A. Picks disease.

B. Lewy body disease.

C. Huntington’s disease.

D. Multiple sclerosis.

E. Amyotropic lateral sclerosis.

A 60-year-old man with a 15-year history of diabetes mellitus type II complains of deep burning pain and sensitivity to touch over his hands and fingers. Nerve conduction studies show slow transmission of impulses and diminished muscle stretch reflexes in both ankles and knees. Sensations to vibrations and light touch are also markedly diminished. Laboratory analysis of CSF shows no biochemical abnormalities. Which of the following is the most likely type of peripheral nerve disease in this patient?

A. B. C. D. E.

36%

55%

0%0%

9%

A. Autonomic neuropathy

B. Distal polyneuropathy

C. Inflammatory neuropathy

D. Mononeuropathy

E. Paraproteinemic polyneuropathy

58y M, Progressive bradykinesia, dementia & Hallucinations ? Diagnosis

A. B. C. D. E.

10%

40%

50%

0%0%

A. Alzheimer’s

B. Parkinson’s

C. Motor neurone disease

D. Syringomyelia

E. Lewy body disease

A 35-year-old man with Down syndrome dies of acute lympho-blastic leukemia. Gross examination of the patient's brain at autopsy shows mild microcephaly and underdevelopment of the superior temporal gyri. Histologic examination would most likely show which of the following neuropathologic changes?

A. B. C. D. E.

20%

10% 10%

60%

0%

A. AA amyloidosis

B. Lewy body dementia

C. Leukemic infiltrates

D. Neurofibrillary tangles

E. Old healed infarcts.

A. B. C. D. E.

0%

30% 30%

0%

40%

A 35 year old HIV +ve man with Kaposisarcoma has a 3 day history of headache and confusion followed by seizures and hemiparesis. CT scan showed multiple ring-enhancing lesions. Image shows Gross appearance and MRI of a brain typical of this disorder. What is the most likely diagnosis?

A. Multiple Aspergilloma.

B. Cerebral TB lesions.

C. Multiple Abscesses.

D. Multiple sclerosis plaques.

E. Cerebral metastases.

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?.

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 2 3 4 5

0% 0% 0%

10%

90%

1. Multiinfarct dementia.

2. Parkinsons disease.

3. Multiple sclerosis

4. Motor neurone disease.

5. Lacunar infarcts.

78y M, Dementia, memory loss and spacial disorientation. Cortical biopsy. ? Diagnosis

1 2 3 4 5

100%

0% 0%0%0%

Cortical biopsy - β amyloid immunostain

1. Alzheimer’s

2. Parkinson’s

3. Lewy body disease

4. Huntington’s disease

5. Pick’s disease

78y, Dementia, Aphasia, Image shows Hippocampus biopsy stained with anti tau stain. ? Diagnosis

A. B. C. D. E.

9%

0%

55%

0%

36%

A. Multiple sclerosis

B. Parkinsons

C. Alzheimer’s

D. Multi-infarct dementia

E. Pick’s disease

78y Male progressive severe dementia, Image shows amyloid stain of his brain.? Identify structure A

1 2 3 4 5

10%

90%

0%0%0%

1. Neurofibrillary tangles.

2. Amyloid plaques.

3. Amyloid Angiopathy

4. Astrocytes - Gliosis.

5. Neuronophagia.

58y female vocalist difficulty remembering songs, depression, dementia & gait abnormality. Image shows brain biopsy. What is the most likely diagnosis?

1 2 3 4 5

0% 0%

90%

10%

0%

1. Alzheimer’s disease.

2. Pick’s disease.

3. Huntington’s disease.

4. Lewy body disease.

5. Creutzfeldt-Jakob disease.

CJD: Spongiform degeneration (vacuoles) no inflammation - Slowly progressive 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. Prion diseases: Kuru, CJD, Scrapie, and bovine spongiform encephalopathy (mad cow disease).

30y F, 7 day history of mild tremor in her arms and impaired balance when walking. Vital signs are normal. Her symptoms disappear the following week, but recures several times over next 4 years gradually developed weakness requiring assistance when walking. Neurologic examination reveals ataxia, dysarthria, decreased vibratory sensation, increased deep tendon reflexes, and a +ve 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 (shown). Which of the following histopathologic findings would be expected in the lesions seen in the patient's brain?

A. B. C. D. E.

0% 0% 0%0%

100%

A. Astrogliosis

B. Lewy bodies

C. Myelin loss.

D. Neuritic plaques

E. Neuronophagia.

What is the best clinical description for the image?

1 2 3 4 5

17% 17%

67%

0%0%

1. 83y man – dementia, memory loss and disorientation.

2. 43y man -abnormal movements who committed suicide

3. 56y woman -progressive paralysis

4. 60y man -tremor, rigidity and dementia

5. 73y woman –Dementia aphasia but intact memory.

What is the best clinical description for the image?

1 2 3 4 5

14%

0% 0%

71%

14%

1. 83y man – dementia, memory loss and disorientation.

2. 43y man -abnormal movements who committed suicide

3. 56y woman -progressive paralysis

4. 60y man -tremor, rigidity and dementia

5. 73y woman –Dementia aphasia intact memory.

ADHDALSPDPick

CNS Degenerative disorders…

A. B. C. D. E.

0% 0% 0%0%0%

A 56 year old man develops 2 year history of increasing hand and forearm weakness and clumsiness. Later develops dysarthria and dysphagia. A year later he dies of pneumonia. Image shows two sections of spinal cord with arrows showing the pathology. What is the most likely diagnosis?

A. Parkinsonism.

B. Multiple Sclerosis.

C. Vitamin B12 deficiency (CDC)

D. Motor Neurone Disease (MND)

E. Amyotrophic Lateral Sclerosis (ALS)

A. Briefly describe pathological features seen in the image?

B. Explain the symptoms? What is the prognosis?

C. List one microscopic feature?

MND .. many subtypes:• Amyotrophic Lateral Sclerosis.• Progressive muscle atrophy (LMN) • Primary lateral sclerosis (UMN)• Progressive bulbar palsy

CNS Degenerative disorders…

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CPC-3.6– KFP Questions: Dementia – Definition, Clinical, types. Primary:

Alzheimer's, Pick's, Parkinson’s & Diffuse Lewy Body Dementia, Huntington's.

Secondary:CVA, Infections (meningitis), Neoplasms,

hydrocephalus, Drugs and toxins, metabolic, vitamin def. (e.g. B1, B2, B12).

Demyelinating disorders:Multiple sclerosis, MND,

The only real mistake is the one from which we learn nothing!

JOHN POWELL: