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Dementia 02

Topic: Imaging

Created on Thursday, February 22 2007 by jdmiles

Last modified on Thursday, February 22 2007.

A 70 year-old male presents to your office accompanied by his mother, who provides most of the history.
The mother states that for quite some time now, the patient developed a tremor, has fallen several times, has been increasingly slow getting around, has been seeing visions of fuzzy white rattlesnakes, has had dramatic fluctuations in his level of arousal, and has been having progressively worsening memory problems.
Which of the following pathology findings is MOST characteristic of this patient's disease?

A) Inclusions comprised primarily of Huntingtin

B) Bunina bodies

C) Inclusions comprised primarily of nacho cheese

D) Amyloid plaques

E) Inclusions comprised primarily of alpha-synuclein

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This question was created on February 22, 2007 by jdmiles.
This question was last modified on February 22, 2007.

ANSWERS AND EXPLANATIONS

A) inclusions comprised primarily of Huntingtin

This answer is incorrect.

This patient's history is consistent with dementia with Lewy bodies (DLB). Patients with DLB develop Parkinsonian symptoms and dementia symptoms at approximately the same time. Other key clinical features include recurrent visual hallucinations and fluctuations in mental status. Lewy bodies are eosinophilic cytoplasmic inclusions comprised mainly of alpha-synuclein. Huntingtin is a protein associated with Huntington chorea. (See References)

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B) Bunina bodies

This answer is incorrect.

This patient's history is consistent with dementia with Lewy bodies (DLB). Patients with DLB develop Parkinsonian symptoms and dementia symptoms at approximately the same time. Other key clinical features include recurrent visual hallucinations and fluctuations in mental status. Neuronal aggregates and Bunina bodies are seen in amyotrophic lateral sclerosis. (See References)

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C) inclusions comprised primarily of nacho cheese

This answer is incorrect.

This patient's history is consistent with dementia with Lewy bodies (DLB). Patients with DLB develop Parkinsonian symptoms and dementia symptoms at approximately the same time. Other key clinical features include recurrent visual hallucinations and fluctuations in mental status. Lewy bodies are eosinophilic cytoplasmic inclusions comprised mainly of alpha-synuclein, and contain little or no cheese of any kind. (See References)

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D) amyloid plaques

This answer is incorrect.

This patient's history is consistent with dementia with Lewy bodies (DLB). Patients with DLB develop Parkinsonian symptoms and dementia symptoms at approximately the same time. Other key clinical features include recurrent visual hallucinations and fluctuations in mental status. Amyloid plaques are seen in DLB, but widespread Lewy bodies are a more defining feature. (See References)

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E) inclusions comprised primarily of alpha-synuclein

This answer is correct.

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References:

1. Victor, M., and Ropper, A.H. (2001). Adams and Victor's Principles of Neurology, 7th Edition. McGraw-Hill, New York.
2. DeKosky, S.T., Kaufer, D.I., and Lopez, O.L. (2004). The Dementias. In Bradley, W.G., Daroff, R.B., Fenichel, G.M., and Jankovic, J. (Eds.). Neurology in Clinical Practice, 4th Edition. Butterworth Heinemann, Philadelphia. Pp. 1901-1951
3. Prayson, R.A., and Goldblum, J.R. (Eds.) (2005). Neuropathology. Elsevier, Philadelphia.

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imaging
Dementia 02
Question ID: 02220701
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 02/22/2007
Modified: 02/22/2007
Estimated Permutations: 0