J Korean Soc Radiol.  2013 Mar;68(3):197-200. 10.3348/jksr.2013.68.3.197.

Relapsing and Progressive Tumefactive Demyelinating Form of Central Nervous System Involvement in a Patient with Progressive Systemic Sclerosis

• Kim HK
• Lee HJ

• KMID: 1748450
• DOI: http://doi.org/10.3348/jksr.2013.68.3.197

Abstract

White matter hyper intensities (WMHI) on MRI are not rare in patients with progressive systemic sclerosis (PSS). In this presentation, WMHI were developed in both middle cerebellar peduncles and temporal white matter in a patient with PSS, and regressed after medication of high dose steroid. However, new lesions were developed in the subcortices of both precentral gyri, and progressed rapidly to tumefactive hyperintensity on MRI. We report an unusual relapsing and progressive tumefactive demyelinating form of central nervous system involvement in PSS.

MeSH Terms

Central Nervous System
Humans
Scleroderma, Diffuse

Figure

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  Fig. 1 A 68-year-old man with central nervous system involvement of progressive systemic sclerosis. A. Axial T2-weighted image shows multiple small white matter hyperintensities in the left pons and cerebral white matter of both hemispheres. B. Follow-up MRI obtained 2 months later shows white matter hyperintensities in both cerebellar peduncles (arrow) and left temporal lobe. C. Follow-up MRI obtained 6 months later shows regression of the white matter hyperintensities in both cerebellar peduncles; however, newly developed patchy white matter hyperintensities are seen in the subcorticies of both precentral gyri (arrow). D. Lesions were progressed into tumefactive white matter hyperintensities after one month (arrow). E. Diffusion tensor MRI showing decreased fiber connectivities in the right cerebellar peduncle (arrow). F. Follow-up imaging shows decreased white matter fiber tract in the right frontal lobe (double arrows).

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