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Inclusion Body Myositis 01

Topic: Pathology

Created on Wednesday, May 2 2007 by jdmiles

Last modified on Wednesday, May 2 2007.

A 66 year-old right handed male presents with a 3-year history of worsening weakness. He complains of difficulty walking up stairs. Past medical history and family history are unremarkable. Exam is normal except for symmetric weakness in the quadriceps and finger flexors.

Muscle biopsy is likely to reveal which of the following findings?


 
        A) Membrane-bound aggregations of glycogen
 
        B) Tubulofilamentous intranuclear and intracytoplasmic inclusions in electron microscopy
 
        C) Tubuloreticular inclusions in endothelial cells
 
        D) Nemaline rods
 
        E) Ragged red fibers
 

 


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This question was created on May 02, 2007 by jdmiles.
This question was last modified on May 02, 2007.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) membrane-bound aggregations of glycogen

This answer is incorrect.


This patient has a history and exam typical of inclusion body myositis (IBM). IBM is the most common inflammatory muscle disease presenting in people over the age of 50. Pathological findings on light microscopy include rimmed vacuoles, inflammatory cells in muscle fibers, and atrophic muscle fibers. Intracytoplasmic and intranuclear inclusions consisting of tubulofilamentous material are seen on electron micrsocopy. Membrane-bound collections of glycogen are typical of Pompe disease, but not of IBM.  (See References)

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B) tubulofilamentous intranuclear and intracytoplasmic inclusions in electron microscopy

This answer is correct.


This patient has a history and exam typical of inclusion body myositis (IBM). IBM is the most common inflammatory muscle disease presenting in people over the age of 50. Pathological findings on light microscopy include rimmed vacuoles, inflammatory cells in muscle fibers, and atrophic muscle fibers. Intracytoplasmic and intranuclear inclusions consisting of tubulofilamentous material are seen on electron micrsocopy.  (See References)

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C) tubuloreticular inclusions in endothelial cells

This answer is incorrect.


This patient has a history and exam typical of inclusion body myositis (IBM). IBM is the most common inflammatory muscle disease presenting in people over the age of 50. Pathological findings on light microscopy include rimmed vacuoles, inflammatory cells in muscle fibers, and atrophic muscle fibers. Intracytoplasmic and intranuclear inclusions consisting of tubulofilamentous material are seen on electron micrsocopy. Tubuloreticular inclusions in endothelial cells are typical in dermatomyositis, but not in IBM.  (See References)

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D) nemaline rods

This answer is incorrect.


This patient has a history and exam typical of inclusion body myositis (IBM). IBM is the most common inflammatory muscle disease presenting in people over the age of 50. Pathological findings on light microscopy include rimmed vacuoles, inflammatory cells in muscle fibers, and atrophic muscle fibers. Intracytoplasmic and intranuclear inclusions consisting of tubulofilamentous material are seen on electron micrsocopy. Nemaline rods are characteristic of nemaline myopathy, but not of IBM.  (See References)

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E) ragged red fibers

This answer is incorrect.


This patient has a history and exam typical of inclusion body myositis (IBM). IBM is the most common inflammatory muscle disease presenting in people over the age of 50. Pathological findings on light microscopy include rimmed vacuoles, inflammatory cells in muscle fibers, and atrophic muscle fibers. Intracytoplasmic and intranuclear inclusions consisting of tubulofilamentous material are seen on electron micrsocopy. Ragged red fibers are not characteristic of IBM.  (See References)

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References:

1. Prayson, R.A., and Goldblum, J.R. (Eds.) (2005). Neuropathology. Elsevier Churchill Livingstone, Philadelphia.
2. Lotz, B.P., Engel, A.G., Nishino, H., Stevens, J.C., and Litchy, W.J. (1989). "Inclusion body myositis. Observations in 40 patients." Brain, 112 ( Pt 3) 727-47. (PMID:2543478)
3. Preston, D.C., and Shapiro, B.E. (2005). Electromyography and Neuromuscular Disorders: Clinical-Electrophysiologic Correlations, 2nd Edition. Elsevier, Philadelphia.
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pathology
Inclusion Body Myositis 01
Question ID: 050207057
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 05/02/2007
Modified: 05/02/2007
Estimated Permutations: 2400

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