Atypical Neuroimaging Manifestations of Linear Scleroderma "en coup de sabre"

Abstract

Linear scleroderma "en coup de sabre" is a subset of localized scleroderma with band-like sclerotic lesions typically involving the fronto-parietal regions of the scalp. Patients often present with neurologic symptoms. On imaging, patients may have lesions in the cerebrum ipsilateral to the scalp abnormality. Infratentorial lesions and other lesions not closely associated with the overlying scalp abnormality, such as those found in the cerebellum, have been reported, but are extremely uncommon. We present a case of an 8-year-old boy with a left fronto-parietal "en coup de sabre" scalp lesion and describe the neuroimaging findings of a progressively enlarging left cerebellar lesion discovered incidentally on routine magnetic resonance imaging. Interestingly, the patient had no neurologic symptoms given the size of the mass identified.

Keywords: Cerebellum; Linear Scleroderma “en coup de sabre”; Localized scleroderma; MRI; Morphea.

Fig 1

Initial brain MRI exam. (A) Axial T2-weighted image, (B) axial FLAIR image showing subtle 10 x 4 mm focus of T2 prolongation in the white matter of the left cerebellar hemisphere and (C) mild diffuse leptomeningeal enhancement was present overlying the left posterior cerebellar hemisphere.

Fig 2

Coronal 2-D reformatted CT image with contrast demonstrates focal scalp thinning within the left fronto-parietal region and thinning of the underlying frontal and parietal bones.

Fig 3

Repeat MRI fourteen months later. (A) Axial T2-weighted image, (B) axial FLAIR image showing new, extensive, patchy areas of T2 prolongation in the white matter of the left cerebellar hemisphere and (C) post contrast axial T1-weighted image demonstrating persistent mild diffuse leptomeningeal enhancement (small arrow) with new areas of nodular parenchymal enhancement (large arrow) in the left cerebellar hemisphere.

Fig 4

Third MRI at age eight. (A) Axial T2- weighted image, (B) axial FLAIR image showing continued increase in size of the patchy areas of T2 prolongation in the left cerebellar hemisphere, (C) axial diffusion weighted image, (D) axial ADC map show corresponding increased diffusivity of the parenchymal lesions, (E) small foci of increased magnetic susceptibility on GRE T2* images are present and (F) post contrast axial T1-weighted image showing persistent leptomeningeal enhancement with more confluent nodular parenchymal enhancement.