title: Localized scleroderma en coup de sabre in the Neurology Clinic.
abstract: 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.
authors.affiliation: Neurology Department, Hospital de Braga, Portugal.
authors.forename: João
authors.initials: J
authors.lastname: Pinho
authors.affiliation: Neurology Department, Hospital Privado de Braga, Lugar da Igreja Nogueira, 4715-196 Braga, Portugal. Electronic address: joaomrocha@gmail.com.
authors.forename: João
authors.initials: J
authors.lastname: Rocha
authors.affiliation: Neurology Department, Hospital de Braga, Portugal.
authors.forename: Filipa
authors.initials: F
authors.lastname: Sousa
authors.affiliation: Dermatology Department, Hospital de Braga, Portugal.
authors.forename: Cristiana
authors.initials: C
authors.lastname: Macedo
authors.affiliation: Neuroradiology Department, Hospital de Braga, Portugal.
authors.forename: João
authors.initials: J
authors.lastname: Soares-Fernandes
authors.affiliation: Neurology Department, Hospital de Braga, Portugal; School of Health Sciences, Life and Health Sciences Research Institute (ICVS), University of Minho, Portugal.
authors.forename: João
authors.initials: J
authors.lastname: Cerqueira
authors.affiliation: Neurology Department, Hospital de Braga, Portugal.
authors.forename: Ricardo
authors.initials: R
authors.lastname: Maré
authors.affiliation: Neurology Department, Hospital de Braga, Portugal.
authors.forename: Esmeralda
authors.initials: E
authors.lastname: Lourenço
authors.affiliation: Neurology Department, Hospital de Braga, Portugal.
authors.forename: João
authors.initials: J
authors.lastname: Pereira
mesh_terms: D000293:Adolescent; D000328:Adult; D001284:Atrophy; D001921:Brain / Q000000981:diagnostic imaging; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012594:Scleroderma, Localized / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology / Q000628:therapy*; D012867:Skin / Q000473:pathology; D014057:Tomography, X-Ray Computed
keywords: Magnetic resonance imaging; Neurological manifestations; Scleroderma en coup de sabre; Scleroderma, Localized