Neurologic manifestations of localized scleroderma

Kister, Ilya; Inglese, Matilde; Laxer, Ronald M.; Herbert, Joseph

doi:10.1212/01.wnl.0000334474.88923.e3

Cited by 143 publications

(179 citation statements)

References 46 publications

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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: 97%

“…5 The relationship between linear morphea ECDS and neurologic findings is well established, with neurologic symptoms described in 18% to 47% of reported cases. 1,6 Neurologic involvement most commonly manifests as complex partial seizures, [7][8][9] but also as hemiparesis, muscle weakness, personality changes, intellectual deterioration, and headaches. [10][11][12][13][14] Headaches associated with ECDS have been poorly characterized in the literature, and only a minority have been classified as migraine headache.…”

Section: Discussionmentioning

confidence: 99%

“…24,25 Indeed, microvascular alterations with vasculitis and fibrosis have been noted in both the affected skin and cerebral tissue. 6,10,16 The pathogenesis of migraine headache is a topic of debate;…”

Section: Discussionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

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

et al. 2014

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“…Abnormal MRI findings are observed in 90% of cases and include hyperintensity on T2-weighted images of the corpus callosum, subcortical regions, deep gray matter and brainstem; and most of times are ipsilateral. Focal atrophy that is the main dermatological finding may also be observed in the cerebral parenchyma (30) .…”

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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“…Many theories about PRS have emerged throughout the years, attributing this syndrome to widely varying etiologies such as infection, trauma, sympathetic nervous system dysfunction, vascular abnormalities, inflammatory conditions, and autoimmune disorders, but at this time, a specific etiology remains uncertain. [1][2][3][4][5][6][7][8][9][13][14][15][16][17]20,25 Changes of cutaneous PRS may be subtle on imaging studies, but 20% of these patients will have intracranial manifestations that may not correspond to the severity of soft-tissue involvement or neurologic symptoms. 13,18,20 Radiologic examinations may identify clinically occult intracranial involvement, facilitate the exclusion of other differential diagnostic considerations, 15 and aid in monitoring disease progression.…”

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confidence: 99%

Parry Romberg Syndrome: 7 Cases and Literature Review

Wong

Phillips

Hagiwara

et al. 2015

American Journal of Neuroradiology

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SUMMARY:Parry Romberg syndrome is a rare progressive hemiatrophy of the face that typically occurs in children and young adults and has a peculiar progression that ceases without apparent cause after a highly variable period. Only a subset of patients with Parry Romberg syndrome will develop secondary neurologic or ophthalmologic symptoms, and prognosis is highly variable. Inconsistency in the pattern of atrophy and the development of associated symptoms in patients with Parry Romberg syndrome has made it challenging to diagnose, prognosticate, and treat. The precise etiology of this disease remains unknown, but some authors have implicated sympathetic cervical ganglion dysfunction, abnormal embryogenesis, autoimmune and inflammatory mechanisms, or vasculopathy as potential causes. We present 7 cases of Parry Romberg syndrome and their associated clinical and imaging findings with specific attention to the radiographic characteristics of this disease. ABBREVIATION: PRS ϭ Parry Romberg syndrome

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Neurologic manifestations of localized scleroderma

Cited by 143 publications

References 46 publications

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

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

Sinais em neurorradiologia: parte 2

Parry Romberg Syndrome: 7 Cases and Literature Review

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