Indian Journal of Forensic Medicine & Toxicology

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Camurati Engelmann’s Disease (CED): A Case Report


Affiliations
1 G.G.S.Medical College & Hospital (BFUHS) Faridkot, Punjab, India
2 Maharaja Agrasen Medical College, Agroha, Hisar, India
     

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Camurati Engelmann's disease is rare and is characterized by severe limb pain, proximal muscle weakness and hyperostosis of long bones and the skull. We report a case of Camurati Engelmann's disease with decreased muscle mass and weakness. There was increased lumbar lordosis, calcaneovalgus of both feet and enlargement of shafts of femur and tibia. Diagnosis was established on X-rays which showed hyperostosis of both femur and tibia and sclerosis of skull bones. The child was put on corticosteroids and analgesics and there was alleviation of symptoms after two follow-ups.

Keywords

Camurati-engelmann’s Disease, Lumbar Lordosis, Hyperostosis, Corticosteroids.
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  • Clawson DK, Loop JW. Progressive diaphyseal dysplasia (Engelmann’s disease). J Bone Joint Surg 1964; 46A: 143- 150.

  • Kinoshita, Saito, Tomita et al. Domain specific mutations in TGFb1 result in Camurati-Engelmann’s disease. Nature Genet 200; 26: 19-20.

  • Lennon EA, Schechter MM, Homabrook RW. Engelmann’s disease. Report of a case with review of the literature. J Bone Joint Surg 1961; 43B: 273-284.

  • Wilson FC, Hundley JD. Progressive diaphyseal dysplasia. Review of the literature and report of seven cases in one family. J Bone Joint Surg 1973; 55: 461-474.

  • Yoshudia H, Mimo A, Kiyosawa N. Muscular changes in Engelmann’s disease. Arch Dis Child 1980; 55: 716-719.

  • Ribbing S. Hereditary, multiple, diaphyseal sclerosis. Acta Radiol 1949; 31: 522-536.

  • Crisp AJ, Brenton DP. Engelmann’s disease of bone – A systemic disorder? Ann Rheu Dis 1982; 41: 183-188.

  • Gird any BR. Engelmann’s disease (progressive diaphyseal dysplasia) – A non-progressive familial form of muscular dystrophy with characteristic bone changes. Clin Orthop 1959; 14: 102-109.

  • Seeger LL, Hewel KC, Yao L et al. Ribbling disease (multiple diaphyseal sclerosis): Imaging and differential diagnosis. Am J Roentgon 1996; 167: 689-694.

  • Minford AMB, Hardy GJ, Forsythe WI, Fitton JM, Rowe VL. Engelmann’s disease and the effect of corticosteroids: A case report. J Bone Joint Surg 1981; 63B: 597-600.

  • Gray AC, Wallace R, Crowe M. Engelmann’s disease: A 45-year follow-up. J Bone Joint Surg 1996; 78B: 488-491.


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  • Camurati Engelmann’s Disease (CED): A Case Report

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Authors

Amit Thakur
G.G.S.Medical College & Hospital (BFUHS) Faridkot, Punjab, India
Vishali Kotwal
G.G.S.Medical College & Hospital (BFUHS) Faridkot, Punjab, India
Rahul Agrawal
Maharaja Agrasen Medical College, Agroha, Hisar, India
Manpreet Kaur Bajwa
Maharaja Agrasen Medical College, Agroha, Hisar, India

Abstract


Camurati Engelmann's disease is rare and is characterized by severe limb pain, proximal muscle weakness and hyperostosis of long bones and the skull. We report a case of Camurati Engelmann's disease with decreased muscle mass and weakness. There was increased lumbar lordosis, calcaneovalgus of both feet and enlargement of shafts of femur and tibia. Diagnosis was established on X-rays which showed hyperostosis of both femur and tibia and sclerosis of skull bones. The child was put on corticosteroids and analgesics and there was alleviation of symptoms after two follow-ups.

Keywords


Camurati-engelmann’s Disease, Lumbar Lordosis, Hyperostosis, Corticosteroids.

References