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TitleLocalized scleroderma en coup de sabre in the Neurology Clinic
Author(s)Pinho, João
Rocha, João
Sousa, Filipa
Macedo, Cristiana
Fernandes, João Soares
Cerqueira, João José
Maré, Ricardo
Lourenço, Esmeralda
Pereira, João
Scleroderma en coup de sabre
Neurological manifestation
Magnetic resonance imaging
Scleroderma, Localized
Neurological manifestations
Issue date19-Jan-2016
JournalMultiple Sclerosis and Related Disorders
CitationPinho, J., Rocha, J., Sousa, F., Macedo, C., Soares-Fernandes, J., Cerqueira, J., . . . Pereira, J. (2016). Localized scleroderma en coup de sabre in the Neurology Clinic. - 8, - 98. doi: 10.1016/j.msard.2016.05.013
Abstract(s)Background: Localized scleroderma en coup de sabre (LScs) is a form of localized scleroderma thought to be an autoimmune disorder. Central nervous system involvement is not rare and neurological manifestations include seizures, focal neurological deficits, headache and neuropsychiatric changes. Methods: Patients attending the Neurology Clinic with the final diagnosis of LScs with neurological manifestations were identified and clinical and imagiological records reviewed. Results: Five patients (0.024%) had LScs with neurological involvement, presenting with transient focal neurologic deficits, seizures, headache or migraine with aura. Neuroimaging studies confirmed localized skin depression and showed bone thinning, white matter lesions, brain calcifications, sulcal effacement and meningeal enhancement. Three patients experienced clinical improvement after immunosuppressive therapy, and in two of these patients neuroimaging findings also improved. Conclusions: Recognizing typical dermatologic changes is keystone for the diagnosis of LScs with neurological involvement. It is a diagnosis of exclusion and extensive etiological diagnostic evaluation should be performed. Treatment options, including conservative follow-up or immunosuppressive therapy, should be carefully considered.
TypeJournal editorial
Publisher version
AccessOpen access
Appears in Collections:ICVS - Artigos em revistas internacionais / Papers in international journals

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