João Pinho1, João Rocha2, Filipa Sousa1, Cristiana Macedo3, João Soares-Fernandes4, João Cerqueira5, Ricardo Maré1, Esmeralda Lourenço1, João Pereira1. 1. Neurology Department, Hospital de Braga, Portugal. 2. Neurology Department, Hospital Privado de Braga, Lugar da Igreja Nogueira, 4715-196 Braga, Portugal. Electronic address: joaomrocha@gmail.com. 3. Dermatology Department, Hospital de Braga, Portugal. 4. Neuroradiology Department, Hospital de Braga, Portugal. 5. Neurology Department, Hospital de Braga, Portugal; School of Health Sciences, Life and Health Sciences Research Institute (ICVS), University of Minho, Portugal.
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.
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.