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Lipoatrophic—Lipodystrophic Syndromes

The Spectrum of Findings on MR Imaging

A. Premkumar1, C. Chow1, P. Bhandarkar1, V. Wright1, N. Koshy1, S. Taylor2 and E. Arioglu2

1 Department of Diagnostic Radiology, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bldg. 10, Rm. 1C660, 10 Center Dr. MSC 1182, Bethesda, MD 20892-1182.
2 National Institute of Diabetes, Digestive and Kidney Diseases, National Institutes of Health, Bldg. 10, Rm. 9S213 10, Center Dr. MSC 1829, Bethesda, MD 20892-1829.



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Fig. 2D. Familial partial lipodystrophy, or Dunnigan-Kobberling syndrome, in 26-year-old woman. Axial MR imaging section obtained through thighs shows no subcutaneous fat and minimal intra- and intermuscular fat. Fat in bone marrow is preserved.

 


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Fig. 4E. Acquired general lipodystrophy, or Lawrence syndrome, in young girl. Axial T1-weighted MR image (400/9; number of exitations, 2; matrix, 256x192) obtained through thighs shows absence of fat in subcutaneous and intramuscular regions but preservation of fat in bone marrow.

 


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Fig. 1A. Congenital generalized lipoatrophy in 17 year-old boy. (Imaging parameters for A—C are TR/TE, 400/9; number of excitations, 2; matrix, 256x192.) Axial T1-weighted MR imaging section obtained through upper abdomen shows increased signal intensity of liver due to fatty infiltration.

 


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Fig. 1B. Congenital generalized lipoatrophy in 17 year-old boy. (Imaging parameters for A—C are TR/TE, 400/9; number of excitations, 2; matrix, 256x192.) Axial MR imaging section obtained through mid abdomen shows complete absence of subcutaneous and intraabdominal fat.

 


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Fig. 1C. Congenital generalized lipoatrophy in 17 year-old boy. (Imaging parameters for A—C are TR/TE, 400/9; number of excitations, 2; matrix, 256x192.) Axial MR imaging section obtained through mid thigh shows no subcutaneous, intramuscular, or bone marrow fat.

 


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Fig. 2A. Familial partial lipodystrophy, or Dunnigan-Kobberling syndrome, in 26-year-old woman. Photographs show increased adipose tissue in patient's face and neck (A) and decreased fat in gluteal region and lower extremities (B).

 


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Fig. 2B. Familial partial lipodystrophy, or Dunnigan-Kobberling syndrome, in 26-year-old woman. Photographs show increased adipose tissue in patient's face and neck (A) and decreased fat in gluteal region and lower extremities (B).

 


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Fig. 2C. Familial partial lipodystrophy, or Dunnigan-Kobberling syndrome, in 26-year-old woman. Axial T1-weighted MR image (TR/TE, 400/9; number of exitations, 2; matrix, 256x192) of mid abdomen shows no subcutaneous fat and increase in intraabdominal fat.

 


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Fig. 3A. New familial partial lipodystrophy syndrome in 30-year-old woman. (Imaging parameters for A and B are TR/TE, 400/9; number of exitations, 2; matrix, 256x192.) Axial T1-weighted MR image of mid abdomen shows decrease in subcutaneous adipose tissue and relative increase in intraabdominal adipose tissue.

 


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Fig. 3B. New familial partial lipodystrophy syndrome in 30-year-old woman. (Imaging parameters for A and B are TR/TE, 400/9; number of exitations, 2; matrix, 256x192.) Axial T1-weighted MR image reveals increased subcutaneous fat in thighs.

 


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Fig. 4A. Acquired general lipodystrophy, or Lawrence syndrome, in young girl. Photographs show patient before (A, taken at 3 years 8 months) and after (B, taken at 6 years 10 months) loss of fat that was preceded by painful febrile panniculitis. Generalized fat loss developed in 16 weeks.

 


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Fig. 4B. Acquired general lipodystrophy, or Lawrence syndrome, in young girl. Photographs show patient before (A, taken at 3 years 8 months) and after (B, taken at 6 years 10 months) loss of fat that was preceded by painful febrile panniculitis. Generalized fat loss developed in 16 weeks.

 


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Fig. 4C. Acquired general lipodystrophy, or Lawrence syndrome, in young girl. Axial T1-weighted MR imaging sections (TR/TE, 400/9; number of exitations, 2; matrix, 256x192) obtained through abdomen reveal fatty liver (C) and loss of subcutaneous and intraabdominal fat (D).

 


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Fig. 4D. Acquired general lipodystrophy, or Lawrence syndrome, in young girl. Axial T1-weighted MR imaging sections (TR/TE, 400/9; number of exitations, 2; matrix, 256x192) obtained through abdomen reveal fatty liver (C) and loss of subcutaneous and intraabdominal fat (D).

 


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Fig. 5A. Acquired partial dystrophy, or Barraquer-Simons' syndrome, in 60-year-old woman. (Imaging parameters for A—C are TR/TE, 400/9; number of exitations, 2; matrix, 256x192.) Axial T1-weighted MR imaging section of mid abdomen reveals loss of subcutaneous fat and relative increase in intraabdominal fat.

 


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Fig. 5B. Acquired partial dystrophy, or Barraquer-Simons' syndrome, in 60-year-old woman. (Imaging parameters for A—C are TR/TE, 400/9; number of exitations, 2; matrix, 256x192.) Axial T1-weighted MR imaging section through pelvis shows increased subcutaneous fat posteriorly with loss of fat anteriorly.

 


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Fig. 5C. Acquired partial dystrophy, or Barraquer-Simons' syndrome, in 60-year-old woman. (Imaging parameters for A—C are TR/TE, 400/9; number of exitations, 2; matrix, 256x192.) Axial T1-weighted MR imaging section reveals increased subcutaneous fat is also present in thighs.

 


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Fig. 6A. Acquired partial lipodystrophy in 56-year-old man. (Imaging parameters of A and B are TR/TE, 400/9; number of exitations, 2; matrix, 256x192.) Axial T1-weighted MR imaging section obtained through abdomen shows loss of subcutaneous fat and increased intraabdominal fat.

 


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Fig. 6B. Acquired partial lipodystrophy in 56-year-old man. (Imaging parameters of A and B are TR/TE, 400/9; number of exitations, 2; matrix, 256x192.) Axial T1-weighted MR imaging section obtained through mid thigh reveals minimal subcutaneous fat and minimal (B) or no (C) intramuscular fat.

 


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Fig. 6C. Acquired partial lipodystrophy in 56-year-old man. (Imaging parameters of A and B are TR/TE, 400/9; number of exitations, 2; matrix, 256x192.) Axial T1-weighted MR imaging section obtained through mid thigh reveals minimal subcutaneous fat and minimal (B) or no (C) intramuscular fat.

 


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Fig. 7A. Dermatomyositis-associated lipodystrophy in 19-year-old woman. Photograph of patient with juvenile dermatomyositis-associated lipodystrophy depicts major loss of fat in extremities.

 


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Fig. 7B. Dermatomyositis-associated lipodystrophy in 19-year-old woman. Axial T1-weighted MR image (TR/TE, 400/9; number of exitations, 2; matrix, 256 x 192) of abdomen shows no subcutaneous fat and increased intraabdominal fat.

 


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Fig. 7C. Dermatomyositis-associated lipodystrophy in 19-year-old woman. Axial T1-weighted MR imaging section (400/9; number of exitations, 2; matrix, 256 x 192) obtained through thighs shows asymmetrical distribution of subcutaneous fat, with more fat deposited anteromedially.

 


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Fig. 8A. Lipodystrophy of unknown origin in 57-year-old man. (Imaging Parameters of A-C are TR/TE, 400/9; number of exitations, 2; matrix, 256 x 192.) Axial MR image shows increase in subcutaneous fat relative to intraabdominal fat.

 


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Fig. 8B. Lipodystrophy of unknown origin in 57-year-old man. (Imaging Parameters of A-C are TR/TE, 400/9; number of exitations, 2; matrix, 256 x 192.) Axial MR image reveals little gluteal fat and "marbling" of gluteus muscle.

 


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Fig. 8C. Lipodystrophy of unknown origin in 57-year-old man. (Imaging Parameters of A-C are TR/TE, 400/9; number of exitations, 2; matrix, 256 x 192.) Axial MR image reveals decrease in subcutaneous fat in thighs, with slight asymmetry in distribution of fat.

 

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