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 Table of Contents     
CASE REPORT
Year : 2011  |  Volume : 26  |  Issue : 1  |  Page : 44-45  

Tc99m-MDP bone scintigraphy in Engelmann-Camurati disease


1 Department of Nuclear Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Endocrinology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Web Publication7-Sep-2011

Correspondence Address:
Bhagwant Rai Mittal
Department of Nuclear Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh - 160 012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-3919.84615

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   Abstract 

Engelmann-Camurati disease (ECD) is a rare bone disorder characterized by autosomal dominant inheritance. It usually presents in early childhood and is associated with symmetrical diaphyseal sclerosis. We report a 20-year-old female with scintigraphic findings characteristic of ECD. She was treated with corticosteroids and showed marked clinical improvement.

Keywords: Bone scintigraphy, Engelmann-Camurati disease, progressive diaphyseal dysplasia


How to cite this article:
Harisankar CB, Kamleshwaran KK, Bhattacharya A, Singh B, Bhadada S, Mittal BR. Tc99m-MDP bone scintigraphy in Engelmann-Camurati disease. Indian J Nucl Med 2011;26:44-5

How to cite this URL:
Harisankar CB, Kamleshwaran KK, Bhattacharya A, Singh B, Bhadada S, Mittal BR. Tc99m-MDP bone scintigraphy in Engelmann-Camurati disease. Indian J Nucl Med [serial online] 2011 [cited 2019 Dec 8];26:44-5. Available from: http://www.ijnm.in/text.asp?2011/26/1/44/84615


   Introduction Top


Engelmann-Camurati disease (ECD) is an autosomal dominant bone disorder caused by mutations in the TGF1 gene [1] and is characterized by progressive bilaterally symmetrical diaphyseal sclerosis of the long bones. It presents prenatally or early during childhood with severe deformities and dwarfism and is characterized by marked thickening of the cortices limited to the diaphyseal regions of tubular bones, occurring on both periosteal and endosteal surfaces. [2] It usually presents in young children with gait disturbance and limb pain and deformity. Autosomal dominant transmission has been noted. [3] Scintigraphically, ECD presents as an increased tracer uptake in the diaphyses of the long bones with sparing of the metaphyses and epiphyses, with corresponding sclerosing dysplastic changes in X-rays. [4],[5],[6] This pattern of involvement of the diaphyses with sparing of the metaphyses and epiphyses is characteristic of ECD. [7]


   Case Report Top


We report a 20-year-old female, who presented with generalized weakness, dull aching pain in the legs and difficulty in waking. Neurologic work up was within normal limits. Bone marrow examination, performed to exclude malignancy, revealed normal bone marrow. Bone scintigraphy was diagnostic of ECD [Figure 1]. She was treated with corticosteroids and physiotherapy and showed marked improvement clinically.
Figure 1: Whole body bone scintigraphy images taken 3 hours after intravenous injection of 20 mCi of 99m-Tc-Methylene diphosphonate show increased symmetrical tracer uptake in diaphyses of bilateral femurs and tibiae. Increased tracer uptake is also noted in the proximal humeri. Scoliosis is also noted

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   References Top

1.Park SJ, Yoon CS, Park HW, Choi JR, Chung JS, Lee KA. The first Korean case of Camurati-Engelmann disease (progressive diaphyseal dysplasia) confirmed by TGFB1 gene mutation analysis. J Korean Med Sci 2009;24:737-40.  Back to cited text no. 1
    
2.Fallon MD, Whyte MP, Murphy WA. Progressive diaphyseal dysplasia (Engelmann's disease): Report of a sporadic case of the mild form. J Bone Joint Surg Am 1980;62:465-72.  Back to cited text no. 2
    
3.Sparkes RS, Graham CB. Camurati-Engelmann disease. Genetics and clinical manifestations with a review of the literature. J Med Genet 1972;9:73-85.  Back to cited text no. 3
    
4.Damiá Ade B, Morón CC, Pérez PA, Molina TC, Mora MM, Fatou AF, et al. Bone scintigraphy in Engelmann-Camurati disease. Clin Nucl Med 2010;35:559-60.  Back to cited text no. 4
    
5.Momose M, Yoshida K, Yanagisawa S, Kadoya M. Camurati-Engelmann disease on a 99m Tc-HMDP bone scan. Eur J Nucl Med Mol Imaging 2008;35:2143.  Back to cited text no. 5
    
6.Narang D, Bharati B, Bhattacharya A, Mittal BR. Radionuclide bone scintigraphy in Engelmann-Camurati disease. Arch Dis Child 2004;89:737.  Back to cited text no. 6
    
7.Inaoka T, Shuke N, Sato J, Ishikawa Y, Takahashi K, Aburano T, et al. Scintigraphic evaluation of pamidronate and corticosteroid therapy in a patient with progressive diaphyseal dysplasia (Camurati-Engelmann disease). Clin Nucl Med 2001;26:680-2.  Back to cited text no. 7
    


    Figures

  [Figure 1]



 

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