Scleroderma "en coup de sabre": pathological evidence of intracerebral inflammation

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Date: Mar. 2001
Publisher: BMJ Publishing Group Ltd.
Document Type: Article
Length: 2,125 words

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Linear scleroderma "en coup de sabre" (LScs) is associated with neurological complications, the pathogenesis of which is uncertain. A 27 year old woman is reported on who developed epilepsy and focal neurological signs in association with LScs. Brain MRI demonstrated predominantly ipsilateral relapsing and remitting grey and white matter lesions. Analysis of CSF and pathology obtained at brain biopsy provides evidence of an inflammatory process which may be amenable to immunosuppressive treatment. (J Neurol Neurosurg Psychiatry 2001;70:382-385)

Keywords: scleroderma "en coup de sabre"; central nervous system inflammation; progressive facial hemiatrophy

Linear scleroderma "en coup de sabre" (LScs) denotes linear scleroderma of the frontoparietal area of the head. It has been reported in association with ipsilateral intracerebral lesions, epilepsy, and ocular complications. Ipsilateral white matter lesions have been seen on CT and MRI in patients with LScs, [1-5] but there are only two reports of neuropathology. [4,5] We report a unique case of LScs where intracerebral lesions which correlate with the clinical course have arisen and resolved spontaneously as recorded with MRI. Neuropathological findings, intrathecal production of IgG, and the response to immunosuppressive treatment, provide evidence for a potentially treatable primary intracerebral inflammatory process occurring in association with linear scleroderma. We discuss these findings in relation to the overlapping and sometimes coexistent condition of progressive facial hemiatrophy (PFHA, also named Parry-Romberg syndrome) in which characteristically the lower half of the face becomes unilaterally a trophic.

Case report

In 1989 a 27 year old right handed woman presented with a 3 month history of weakness in her left hand. Aged 15 she had presented with typical LScs affecting the right frontotemporal area of her scalp. This had subsequently progressed over several years but had been static for 5 years. She had been under regular dermatology review and received no treatment. There was no other significant medical or family history. On examination, there was mild pyramidal weakness of the left arm with exaggerated tendon reflexes. There were no other manifestations of connective tissue disease.

Brain MRI initially showed extensive abnormalities in the right hemisphere comprising multiple high signal foci on T2 weighted images involving grey matter, and superficial and deep white matter. There was enhancement with gadolinium on T1 weighted images and mild focal atrophy. The constituents of CSF were normal but electrophoresis showed local synthesis of IgG with an oligoclonal distribution.

Six months later she presented with secondarily generalised seizures. A repeat MRI examination showed partial resolution of the previous lesions but a new area of high signal in the right frontal lobe. Routine laboratory studies and a comprehensive autoantibody screen were negative.

Over the next 3 years her epilepsy remained well controlled on anticonvulsant drugs. Brain MRI in 1992 showed partial resolution of the previous right hemispheric lesions. In 1993 she began to experience difficulties with handwriting, dysarthria, and jaw spasm. Repeat MRI demonstrated a large new high signal lesion on T2 weighted images in the left thalamus, with marked enhancement on T1 weighted images and a new lesion in...

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Gale Document Number: GALE|A78049066