Neuroimaging findings in scleroderma
            <i>en coup de sabre</i>

Appenzeller, Simone; Montenegro, Maria Augusta; Dertkigil, Sérgio; Sampaio-Barros, Percival Degrava; Marques-Neto, João Francisco; Sâmara, Adil Muhib; Andermann, F.; Cendes, Fernando

doi:10.1212/01.wnl.0000124518.25087.18

Cited by 83 publications

(80 citation statements)

References 16 publications

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“…2 Unlike most forms of localized scleroderma, which lack extracutaneous manifestations, a subset of linear scleroderma referred to as en coup de sabre has been associated with several neurologic abnormalities. [3][4][5][6][7][8][9] Many of the epidemiologic studies of scleroderma have come from dermatology and rheumatology centers, where referral bias influences estimates of the incidence, prevalence, and severity of this disease. Systemic scleroderma is rare in the pediatric population, and localized scleroderma has been estimated to be at least 10 times more common than systemic disease.…”

Section: Discussionmentioning

confidence: 99%

“…5 Abnormalities on MRI and CT studies may be seen in patients with linear scleroderma en coup de sabre even in the absence of neurologic disease. 6,7 Radiologic abnormalities are predominately ipsilateral to the skin disease but may be contralateral. 4,8 Neuroradiologic abnormalities including cerebral atrophy, whitematter lesions, intraparenchymal calcification, meningeocortical alterations, and skull atrophy have all been described in the literature.…”

Section: Discussionmentioning

confidence: 99%

“…4,8 Neuroradiologic abnormalities including cerebral atrophy, whitematter lesions, intraparenchymal calcification, meningeocortical alterations, and skull atrophy have all been described in the literature. 4,6,7,9,18 Resolution and recurrence of radiologic lesions may correlate clinically with neurologic disease activity. 4,9 Just as imaging may be abnormal in patients without neurologic symptoms, normal imaging can be seen despite the presence of neurologic disease as demonstrated by 2 of our patients as well as the case of a patient with facial and oculomotor nerve palsy and grand mal seizures described by Gambichler et al 19 The lack of specific and/or sensitive radiologic findings for the associated neurologic disease contributes to the difficulty in counseling these patients regarding prognosis.…”

Section: Discussionmentioning

confidence: 99%

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Linear Scleroderma en coup de sabre With Associated Neurologic Abnormalities

Holland¹,

Steffes²,

et al. 2006

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Linear scleroderma represents a unique form of localized scleroderma that primarily affects the pediatric population, with 67% of patients diagnosed before 18 years of age. When linear scleroderma occurs on the head, it is referred to as linear scleroderma en coup de sabre, given the resemblance of the skin lesions to the stroke of a sabre. Here we describe 3 pediatric patients with linear scleroderma en coup de sabre who presented with neurologic abnormalities before or concurrent with the diagnosis of their skin disease. Our patients' cases highlight the underrecognized relationship between neurologic complications and linear scleroderma en coup de sabre and illustrate the importance of a thorough skin examination in patients with unexplained neurologic disease. S CLERODERMA IS A rare disease of unknown etiology in which increased collagen deposition occurs and results in dermal thickening. Involvement may be diffuse (systemic sclerosis) or localized to the skin (localized scleroderma). Linear scleroderma represents a unique form of localized scleroderma that primarily affects the pediatric population, with 67% of patients diagnosed before 18 years of age. 1 Linear scleroderma frequently occurs on the limbs but also may develop in the frontoparietal area of the forehead and scalp. When linear scleroderma occurs on the head, it is referred to as linear scleroderma en coup de sabre, given the resemblance of the skin lesions to the stroke of a sabre. Neurologic abnormalities have been described in association with linear scleroderma en coup de sabre. Typically, characteristic skin involvement is recognized before the development of neurologic disease. We describe 3 pediatric patients with linear scleroderma en coup de sabre who presented with neurologic abnormalities before or concurrent with the diagnosis of their skin disease. Their cases illustrate the variability in neurologic manifestations of linear scleroderma en coup de sabre as well as the importance of a careful skin examination in patients with unexplained neurologic disease. CASE REPORTS CASE 1.A 5-year-old girl presented with new-onset partial complex seizures and skin lesions. An epileptiform pattern in the left posterotemporal area was detected by electroencephalogram (EEG), and she was started on anticonvulsants. Imaging by computed tomography (CT) and MRI was normal. Enlargement of the skin lesions prompted a referral to dermatology, and examination revealed a linear, indurated plaque with alopecia extending from her left eyebrow into the hair-bearing scalp. Gray-brown atrophic plaques were also present on the midline of her scalp and on her neck extending from her left mandible to the anterior shoulder. A diagnosis of linear scleroderma en coup de sabre was made based on the characteristic skin changes observed. Her anti-single-stranded DNA level was slightly elevated at 159 /ml; her antinuclear antibodies (ANA), rheumatoid factor, complete blood count, anti-extractable nuclear antigens, and anti-double-stranded DNA levels were all withi...

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Linear Scleroderma en coup de sabre With Associated Neurologic Abnormalities

Holland¹,

Steffes²,

et al. 2006

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“…6,12,[18][19][20][21][22] In 3 of our 4 patients, no alterations were detected, suggesting a bias toward description in the literature of patients with more severe neurologic abnormalities. Nevertheless, MRI abnormalities have been noted in the absence of clinical neurologic symptoms, 12,18,23 suggesting that patients with ECDS may deserve brain imaging as a component of evaluation.…”

Section: Discussionmentioning

confidence: 99%

Headaches as a Presenting Symptom of Linear Morphea en Coup de Sabre

et al. 2014

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Linear morphea en coup de sabre (ECDS) is a form of localized scleroderma that predominantly affects the pediatric population, with a median age of 10 years at presentation. The existence of neurologic findings in association with ECDS has been well described in the literature. Here we describe 4 patients with ECDS who presented with headaches, which were typical migraines in 3 of the patients. The headaches preceded the onset of cutaneous findings by at least 6 months. Our patients' cases emphasize both the importance of recognizing headaches as a harbinger of ECDS and the necessity of performing thorough cutaneous examination in patients with unexplained headaches or other neurologic disease. Pediatrics 2014;134:e1715-e1719 AUTHORS:

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“…Huntington's disease is an autosomal dominant neurodegenera-nosis (29) . Localized scleroderma invariably affects the head, presenting as a linear, usually frontoparietal lesion (scleroderma "en coup de sabre") ( Figure 15), with progressive facial hemiatrophy or Parry-Romberg syndrome where the atrophy extends beyond the skin to involve the subcutaneous cellular tissue, muscles and bones.…”

Section: "Boxcar Ventricle Sign" In Huntington's Diseasementioning

confidence: 99%

Sinais em neurorradiologia: parte 2

et al. 2011

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The use of signs for interpretation of images in neuroradiology is extremely useful. Some signs are quite specific and, in some cases, pathognomonic. In this second part of their essay, the authors describe 15 additional neuroradiological signs. Main characteristics of imaging findings will be approached and the significance of their role in the clinical practice will be discussed. Keywords: Radiological signs; Neuroradiology; Computed tomography; Magnetic resonance imaging.O uso de sinais na interpretação de imagens na neurorradiologia é extremamente útil. Muitos sinais são bastante específicos e em alguns casos, patognomônicos. Nesta segunda parte os autores descreverão 15 sinais neurorradiológicos adicionais. Serão novamente abordadas as principais características de imagem de cada um e sua importân-cia na prática clínica. Unitermos: Sinais radiológicos; Neurorradiologia; Tomografia computadorizada; Imagem por ressonância magnética. Abstract "High heel foot print sign" in the skull baseThe high heel foot print sign (Figure 1) is useful in the understanding of the intricate anatomy of the skull base and represent two relevant foramina. The anterior aspect of the high heel footprint represents the foramen ovale (FO), and the posterior aspect (the heel itself) the foramen spinosum (FS). The mandibular nerve, one of the three branches of the trigeminal nerve, is the main FO component (1) . Also, the otic Figure 1. Axial CT image demonstrating normal appearance of the skull base. The high heel foot print sign is evidenced. The small arrow indicates the foramen spinosum, and the large arrow, the foramen ovale. Anteriorly to the high heel footprint is the Vesalius foramen (venous foramen) (arrowhead). ganglion, the accessory meningeal artery, the lesser petrosal nerve and the emissary veins are found in this foramen. The middle meningeal artery is in the FS (2) , and the absence of such artery is related to the persistent stapedial artery (3) . "Lemon sign" in spina bifidaThe lemon sign (Figure 2) is an useful sign in the detection of spina bifida and is commonly associated with hydrocephalus and Arnold-Chiari II malformation (4) . Such sign can be found in up to 98% of cases of spina bifida (5) . The lemon sign is not exclusive to spina bifida and can be observed in cases of encephalocele, thanatophoric dys-

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Neuroimaging findings in scleroderma en coup de sabre

Cited by 83 publications

References 16 publications

Linear Scleroderma en coup de sabre With Associated Neurologic Abnormalities

Linear Scleroderma en coup de sabre With Associated Neurologic Abnormalities

Headaches as a Presenting Symptom of Linear Morphea en Coup de Sabre

Sinais em neurorradiologia: parte 2

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