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Myopathies 04

Topic: Pathology

Created on Thursday, November 29 2007 by jdmiles

Last modified on Thursday, November 29 2007.

The image above shows an electron micrograph of a skeletal muscle biopsy.

Which of the following statements about this condition is most accurate?


 
        A) This is polymyositis
 
        B) This disorder can be reliably diagnosed with electrodiagnostic testing
 
        C) This is dermatomyositis
 
        D) CPK level in this patient is likely to be normal
 
        E) 25% of patients with this condition have other autoimmune diseases
 

 


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This question was created on November 29, 2007 by jdmiles.
This question was last modified on November 29, 2007.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) This is polymyositis

This answer is incorrect.


This EM photomicrograph shows the inclusions typical of inclusion body myositis (IBM). IBM is the most common muscle disorder in patients over 50 years of age. It is characterized by distal and proximal weakness, classically
showing preferential involvement of the quadriceps and long finger flexors. Dysphagia is common, affecting up to 40% of patients. CPK level is usually normal or slightly elevated. It is more common in males than in females, and 15% of patients with IBM also have other autoimmune diseases. Both sporadic and familal forms exist, however the familial form is quite rare. IBM is progressive, and half of these patients are wheelchair bound within 10 years. IBM does not appear to be an inflammatory disease, and does not respond to steroids or immunosuppressive therapies.  (See References)

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B) This disorder can be reliably diagnosed with electrodiagnostic testing

This answer is incorrect.


This EM photomicrograph shows the inclusions typical of inclusion body myositis (IBM).  IBM is the most common muscle disorder in patients over 50 years of age.  It is characterized by distal and proximal weakness, classically
showing preferential involvement of the quadriceps and long finger flexors.  Dysphagia is common, affecting up to 40% of patients.  CPK level is usually normal or slightly elevated.  It is more common in males than in females, and 15% of patients with IBM also have other autoimmune diseases.  Both sporadic and familal forms exist, however the familial form is quite rare.  IBM is progressive, and half of these patients are wheelchair bound within 10 years.  IBM does not appear to be an inflammatory disease, and does not respond to steroids or immunosuppressive therapies.

IBM has variable findings on EMG and NCS, so electrodiagnostic testing is not reliably diagnostic.

  (See References)

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C) This is dermatomyositis

This answer is incorrect.


This EM photomicrograph shows the inclusions typical of inclusion body myositis (IBM). IBM is the most common muscle disorder in patients over 50 years of age. It is characterized by distal and proximal weakness, classically
showing preferential involvement of the quadriceps and long finger flexors. Dysphagia is common, affecting up to 40% of patients. CPK level is usually normal or slightly elevated. It is more common in males than in females, and 15% of patients with IBM also have other autoimmune diseases. Both sporadic and familal forms exist, however the familial form is quite rare. IBM is progressive, and half of these patients are wheelchair bound within 10 years. IBM does not appear to be an inflammatory disease, and does not respond to steroids or immunosuppressive therapies.  (See References)

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D) CPK level in this patient is likely to be normal

This answer is correct.


This EM photomicrograph shows the inclusions typical of inclusion body myositis (IBM). IBM is the most common muscle disorder in patients over 50 years of age. It is characterized by distal and proximal weakness, classically
showing preferential involvement of the quadriceps and long finger flexors. Dysphagia is common, affecting up to 40% of patients. CPK level is usually normal or slightly elevated. It is more common in males than in females, and 15% of patients with IBM also have other autoimmune diseases. Both sporadic and familal forms exist, however the familial form is quite rare. IBM is progressive, and half of these patients are wheelchair bound within 10 years. IBM does not appear to be an inflammatory disease, and does not respond to steroids or immunosuppressive therapies.  (See References)

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E) 25% of patients with this condition have other autoimmune diseases

This answer is incorrect.


This EM photomicrograph shows the inclusions typical of inclusion body myositis (IBM). IBM is the most common muscle disorder in patients over 50 years of age. It is characterized by distal and proximal weakness, classically
showing preferential involvement of the quadriceps and long finger flexors. Dysphagia is common, affecting up to 40% of patients. CPK level is usually normal or slightly elevated. It is more common in males than in females, and 15% of patients with IBM also have other autoimmune diseases. Both sporadic and familal forms exist, however the familial form is quite rare. IBM is progressive, and half of these patients are wheelchair bound within 10 years. IBM does not appear to be an inflammatory disease, and does not respond to steroids or immunosuppressive therapies.  (See References)

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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. (ISBN:0070674973) Advertising:
2. Prayson, R.A., and Goldblum, J.R. (Eds.) (2005). Neuropathology. Elsevier Churchill Livingstone, Philadelphia. (ISBN:0443066582) Advertising:
3. Preston, D.C., and Shapiro, B.E. (2005). Electromyography and Neuromuscular Disorders: Clinical-Electrophysiologic Correlations, 2nd Edition. Elsevier, Philadelphia. (ISBN:075067492X) Advertising:
4. Hermanns, B., Molnar, M., and Schröder, J.M. (2000). "Peripheral neuropathy associated with hereditary and sporadic inclusion body myositis: confirmation by electron microscopy and morphometry." J Neurol Sci, 179(S 1-2) 92-102. (PMID:11054491)
5. Rose, M.R., McDermott, M.P., Thornton, C.A., Palenski, C., Martens, W.B., and Griggs, R.C. (2001). "A prospective natural history study of inclusion body myositis: implications for clinical trials." Neurology, 57(3) 548-50. (PMID:11502935)
6. 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)
7. Hengstman, G.J., van Engelen, B.G., ter Laak, H.J., and Gabreëls-Festen, A.A. (2001). "Familial inclusion body myositis with histologically confirmed sensorimotor axonal neuropathy." J Neurol, 247(11) 882-4. (PMID:11151424)
8. Brannagan, T.H., Hays, A.P., Lange, D.J., and Trojaborg, W. (1998). "The role of quantitative electromyography in inclusion body myositis." J Neurol Neurosurg Psychiatry, 63(6) 776-9. (PMID:9416815)
9. Houser, S.M., Calabrese, L.H., and Strome, M. (1998). "Dysphagia in patients with inclusion body myositis." Laryngoscope, 108(7) 1001-5. (PMID:9665246)
10. [edited by] Bashar Katirji... [et al.]. . Boston : Butterworth-Heinemann, c2002 (ISBN:0750671696)
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pathology
Myopathies 04
Question ID: 112907082
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 11/29/2007
Modified: 11/29/2007
Estimated Permutations: 356400

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