AJR
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Abstract Freely available
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Robertson, P. L.
Right arrow Articles by Goldblatt, J.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Robertson, P. L.
Right arrow Articles by Goldblatt, J.
Social Bookmarking
Add to CiteULike   Add to Complore   Add to Connotea   Add to Del.icio.us   Add to Digg   Add to Reddit   Add to Technorati  
What's this?
Hotlight (NEW!)
Right arrow
What's Hotlight?

Semiquantitative Assessment of Skeletal Response to Enzyme Replacement Therapy for Gaucher's Disease Using the Bone Marrow Burden Score

Patricia L. Robertson1, Mario Maas2 and Jack Goldblatt3

1 Department of Radiology, The University of Melbourne, Royal Melbourne Hospital, Grattan St., Parkville, Melbourne, Victoria 3050, Australia.
2 Department of Radiology, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
3 School of Paediatrics and Child Health, University of Western Australia, Perth, WA, Australia.


Figure 1
View larger version (98K):
[in this window]
[in a new window]
[as a PowerPoint slide]
  		Fig. 1A —Pretreatment MR images in 44-year-old woman with newly diagnosed Gaucher's disease. Sagittal T1-weighted (A) and T2-weighted (B) images of lumbar spine show bone marrow of lower signal intensity than disk and presacral fat. Score for signal intensity was 4 and for distribution was 3 since fat around basivertebral veins was replaced. Lumbar bone marrow burden (BMB) score was thus 7.

 

Figure 2
View larger version (116K):
[in this window]
[in a new window]
[as a PowerPoint slide]
  		Fig. 1B —Pretreatment MR images in 44-year-old woman with newly diagnosed Gaucher's disease. Sagittal T1-weighted (A) and T2-weighted (B) images of lumbar spine show bone marrow of lower signal intensity than disk and presacral fat. Score for signal intensity was 4 and for distribution was 3 since fat around basivertebral veins was replaced. Lumbar bone marrow burden (BMB) score was thus 7.

 

Figure 3
View larger version (126K):
[in this window]
[in a new window]
[as a PowerPoint slide]
  		Fig. 1C —Pretreatment MR images in 44-year-old woman with newly diagnosed Gaucher's disease. Coronal T1-weighted (C) and T2-weighted (D) images of femurs were scored 5 for signal intensity and 3 for extent of involvement because of infiltration of epiphyses around knee, giving total femoral BMB score of 8. Note shortening of right femur because of previous infarction and collapse of right femoral head (more readily seen in Fig. 2A, 2B, 2C, 2D).

 

Figure 4
View larger version (130K):
[in this window]
[in a new window]
[as a PowerPoint slide]
  		Fig. 1D —Pretreatment MR images in 44-year-old woman with newly diagnosed Gaucher's disease. Coronal T1-weighted (C) and T2-weighted (D) images of femurs were scored 5 for signal intensity and 3 for extent of involvement because of infiltration of epiphyses around knee, giving total femoral BMB score of 8. Note shortening of right femur because of previous infarction and collapse of right femoral head (more readily seen in Fig. 2A, 2B, 2C, 2D).

 

Figure 5
View larger version (106K):
[in this window]
[in a new window]
[as a PowerPoint slide]
  		Fig. 2A —Images of same woman with Gaucher's disease shown in Figure 1A, 1B, 1C, 1D obtained 2.5 years later, after she had undergone 20 months of enzyme replacement therapy. Sagittal T1-weighted (A) and T2-weighted (B) images of spine show considerable return of signal to marrow so that signal intensity score is now 1 and distribution score 3, giving lumbar bone marrow burden (BMB) score of 4.

 

Figure 6
View larger version (104K):
[in this window]
[in a new window]
[as a PowerPoint slide]
  		Fig. 2B —Images of same woman with Gaucher's disease shown in Figure 1A, 1B, 1C, 1D obtained 2.5 years later, after she had undergone 20 months of enzyme replacement therapy. Sagittal T1-weighted (A) and T2-weighted (B) images of spine show considerable return of signal to marrow so that signal intensity score is now 1 and distribution score 3, giving lumbar bone marrow burden (BMB) score of 4.

 

Figure 7
View larger version (145K):
[in this window]
[in a new window]
[as a PowerPoint slide]
  		Fig. 2C —Images of same woman with Gaucher's disease shown in Figure 1A, 1B, 1C, 1D obtained 2.5 years later, after she had undergone 20 months of enzyme replacement therapy. Coronal T1-weighted (C) and STIR (D) images of femurs show return of signal to femoral epiphyses but because of persisting heterogeneous pattern and residual T2 signal intensity score remained 5 with distribution score of 1, giving overall femoral BMB score of 6.

 

Figure 8
View larger version (146K):
[in this window]
[in a new window]
[as a PowerPoint slide]
  		Fig. 2D —Images of same woman with Gaucher's disease shown in Figure 1A, 1B, 1C, 1D obtained 2.5 years later, after she had undergone 20 months of enzyme replacement therapy. Coronal T1-weighted (C) and STIR (D) images of femurs show return of signal to femoral epiphyses but because of persisting heterogeneous pattern and residual T2 signal intensity score remained 5 with distribution score of 1, giving overall femoral BMB score of 6.

 

Figure 9
View larger version (113K):
[in this window]
[in a new window]
[as a PowerPoint slide]
  		Fig. 3A —Images of same woman with Gaucher's disease shown in Figures 1A, 1B, 1C, 1D and 2A, 2B, 2C, 2D obtained 4 years after commencement of enzyme replacement therapy. Signal intensity on sagittal T1-weighted (A) and T2-weighted (B) images of spine is now normal giving lumbar bone marrow burden (BMB) score of 0.

 

Figure 10
View larger version (138K):
[in this window]
[in a new window]
[as a PowerPoint slide]
  		Fig. 3B —Images of same woman with Gaucher's disease shown in Figures 1A, 1B, 1C, 1D and 2A, 2B, 2C, 2D obtained 4 years after commencement of enzyme replacement therapy. Signal intensity on sagittal T1-weighted (A) and T2-weighted (B) images of spine is now normal giving lumbar bone marrow burden (BMB) score of 0.

 

Figure 11
View larger version (136K):
[in this window]
[in a new window]
[as a PowerPoint slide]
  		Fig. 3C —Images of same woman with Gaucher's disease shown in Figures 1A, 1B, 1C, 1D and 2A, 2B, 2C, 2D obtained 4 years after commencement of enzyme replacement therapy. Coronal T1-weighted (C) and STIR (D) images of femurs where signal intensity in marrow is now closer to normal in areas not affected by infarction or fibrosis so that signal intensity score is 2 and distribution score 1, giving femoral BMB score of 3.

 

Figure 12
View larger version (129K):
[in this window]
[in a new window]
[as a PowerPoint slide]
  		Fig. 3D —Images of same woman with Gaucher's disease shown in Figures 1A, 1B, 1C, 1D and 2A, 2B, 2C, 2D obtained 4 years after commencement of enzyme replacement therapy. Coronal T1-weighted (C) and STIR (D) images of femurs where signal intensity in marrow is now closer to normal in areas not affected by infarction or fibrosis so that signal intensity score is 2 and distribution score 1, giving femoral BMB score of 3.

 

Add to CiteULike CiteULike   Add to Complore Complore   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Digg Digg   Add to Reddit Reddit   Add to Technorati Technorati    What's this?



HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Copyright © 2007 by the American Roentgen Ray Society.