Annals of Indian Academy of Neurology
IMAGES IN NEUROLOGY
Year
: 2006  |  Volume : 9  |  Issue : 3  |  Page : 175--176

Imaging in Paget's disease of skull


Amel Antony, Anil Kumar, P Sreekumar, Haroon Marikar 
 Institute of Radiology and Imaging Sciences, Kochi, Kerala, India

Correspondence Address:
Amel Antony
Institute of Radiology and Imaging Sciences, Kochi, Kerala
India




How to cite this article:
Antony A, Kumar A, Sreekumar P, Marikar H. Imaging in Paget's disease of skull.Ann Indian Acad Neurol 2006;9:175-176


How to cite this URL:
Antony A, Kumar A, Sreekumar P, Marikar H. Imaging in Paget's disease of skull. Ann Indian Acad Neurol [serial online] 2006 [cited 2021 Jan 17 ];9:175-176
Available from: https://www.annalsofian.org/text.asp?2006/9/3/175/27663


Full Text

A 70 year old lady presented with hemifacial spasm. There was no significant past history. On physical examination there were no focal neurological deficits Fundoscopy showed no papilloedema. There were no abnormal bony prominences. Laboratory investigationos including serum biochemistry profile were unremarkable. The plain X-ray of skull, CT Scan Head and MRI Head revealed changes characteristic of Paget's disease. [Figure 1][Figure 2][Figure 3][Figure 4].

Paget's disease is a chronic skeletal disease characterized by bony expansion with variable destruction and sclerosis.[1] Sir James Paget first described this disease "osteitis deformans" in 1876. The disease is uncommon in Asians.[2]

The disease begins as a destructive process affecting the outer table and sparing the inner table. Later osteoblastic activity supervenes and is seen as sclerotic areas in a background of lysis and is seen in the image. This is described as "cotton wool appearance"in the X-ray skull. Coarsened trabeculae of increased volume is seen pathologically. Skull base is commonly involved[3] CT shows the disease extent of the disease with greater accuracy and is useful if reconstructive surgery is contemplated.

 Magnetic Resonance Imaging



Classically, yellow marrow is maintained[1] and is shown as hyperintensity in the T1 image. The areas of hypointensity in T1 weighted images represent areas of sclerosis. The inner table of skull is maintained. MRI can detect sarcomatous degeneration (a complication of Paget's disease) as destruction of inner table and extra-axial soft tissue.

References

1Smith SE, Murphey MD, Motamedi K, Mulligan ME, Resnik CS, Gannon FH. Radiologic spectrum of Paget disease of bone and its complications. Radiographics 2002;22:1191-216.
2Lopez-Abente G, Morales-Piga A, Bachiller-Corral FJ, Illera-Martin O, Martin-Domenech R, Abraira V. Identification of possible areas of high prevalence of Paget's disease of bone in Spain. Clin Exp Rheumatol 2003;21:635-8.
3Curtin HD, Rabinov J, Som PM. Skull base in Som PM curtin HD (editors). Head and Neck imaging. 4th ed. Mosby Year Book: St. Louis, MO; p. 785-864.