Linear scleroderma “en coup de sabre” coexisting with plaque-morphea: neuroradiological manifestation and response to corticosteroids

• Published in: Journal of neurology, neurosurgery and psychiatry Vol. 74; no. 5; pp. 661 - 664
• Main Authors: Unterberger, I, Trinka, E, Engelhardt, K, Muigg, A, Eller, P, Wagner, M, Sepp, N, Bauer, G
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd 01.05.2003; BMJ; BMJ Publishing Group LTD; BMJ Group
• Subjects: Adult; Antibodies; Atrophy; Biological and medical sciences; Biopsy; Calcification; Case reports; central nervous system nflammation; Convulsions & seizures; Epilepsy; Female; Humans; Hypotheses; linear scleroderma “en coup de sabre”; Magnetic Resonance Imaging; Medical sciences; Methylprednisolone - administration & dosage; Methylprednisolone - therapeutic use; Nervous System Diseases - diagnosis; Nervous System Diseases - drug therapy; Nervous System Diseases - etiology; Neuroprotective Agents - administration & dosage; Neuroprotective Agents - therapeutic use; Patients; plaque-morphea; Pregnancy; Pregnancy Complications - diagnosis; Pregnancy Complications - drug therapy; Pulse Therapy, Drug; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Scleroderma; Scleroderma, Localized - complications; Scleroderma, Localized - diagnosis; Scleroderma, Localized - drug therapy; Short Report; Steroids; Tomography; Tomography, X-Ray Computed; Trauma; Human; Hemiparesis; Connective tissue disease; Corticosteroid; Skin disease; Nervous system diseases; Prognosis; Motor system disorder; Antiinflammatory agent; Nuclear magnetic resonance imaging; Linear scleroderma; Morphea scleroderma; Cerebral disorder; Case study; Chemotherapy; Treatment; Central nervous system disease; Young adult; Medical imagery; Complication; Female; Neurological disorder
• ISSN: 0022-3050; 1468-330X
• DOI: 10.1136/jnnp.74.5.661

A 24 year old woman in the 33rd week of pregnancy developed progressive neurological complications with right sided hemiparesis in association with the occurrence of linear scleroderma “en coup de sabre” (LSCS) and pre-existing plaque-morphea, already being treated by balneophototherapy. Further progression of neurological symptoms led to a caesarean section with the delivery of a healthy child. Brain magnetic resonance imaging (MRI) showed focal T2 signal increases in the left frontoparietal region directly adjacent to the area of LSCS. Cerebrospinal fluid analysis revealed oligoclonal bands, suggesting an intracerebral inflammatory process. Subsequent pulsed corticosteroid treatment led to a remission of neurological symptoms and to a marked resolution of the MRI lesions.