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Espasmo hemifacial bilateral: relato de caso
Machado, Flavia Costa Nunes;Fregni, Felipe;Campos, Cynthia Resende;Limongi, Jo?o Carlos Papaterra;
Arquivos de Neuro-Psiquiatria , 2003, DOI: 10.1590/S0004-282X2003000100023
Abstract: bilateral hemifacial spasm (bhs) is a rare focal movement disorder often associated with vascular compression of both facial nerves. the contractions are usually asymmetric and asynchronous. typically, one side is affected first and there is a long but variable interval for the symptoms on the other side to occur. bhs must be differentiated from other conditions including blefarospasm, facial myokymia, facial tics, oromandibular dystonia, and hemimasticatory spasm. the most successful and non-invasive symtomatic treatment is botulinum toxin injections but microvascular decompression surgery is another therapeutic option. we report the case of a 70 years old man with bilateral hemifacial spasms and present a brief review of the literature.
Neurologic Involvement in Scleroderma en Coup de Sabre  [PDF]
Tiago Nardi Amaral,Jo?o Francisco Marques Neto,Aline Tamires Lapa,Fernando Augusto Peres,Caio Rodrigues Guirau,Simone Appenzeller
Autoimmune Diseases , 2012, DOI: 10.1155/2012/719685
Abstract: Localized scleroderma is a rare disease, characterized by sclerotic lesions. A variety of presentations have been described, with different clinical characteristics and specific prognosis. In scleroderma en coup de sabre (LScs) the atrophic lesion in frontoparietal area is the disease hallmark. Skin and subcutaneous are the mainly affected tissues, but case reports of muscle, cartilage, and bone involvement are frequent. These cases pose a difficult differential diagnosis with Parry-Romberg syndrome. Once considered an exclusive cutaneous disorder, the neurologic involvement present in LScs has been described in several case reports. Seizures are most frequently observed, but focal neurologic deficits, movement disorders, trigeminal neuralgia, and mimics of hemiplegic migraines have been reported. Computed tomography and magnetic resonance imaging have aided the characterization of central nervous system lesions, and cerebral angiograms have pointed to vasculitis as a part of disease pathogenesis. In this paper we describe the clinical and radiologic aspects of neurologic involvement in LScs. 1. Introduction Scleroderma is a rare disease of unknown etiology, characterized by thickening and hardening of skin resulting from increased collagen production. The term includes a variety of diseases, from localized scleroderma (LS) to systemic sclerosis. LS is traditionally considered to be limited to skin, subcutaneous tissue, underlying bone, and, in craniofacial subtype, nervous system involvement [1]. Recent studies, however, have described malaise, fatigue, arthralgia, and myalgia in morphea. Moreover, rheumatologic, ophthalmologic and neurologic symptoms and signs have been described in up to 20% of the patients with LS. Based on these findings LS ought to be differentiated from systemic sclerosis by the absence of sclerodactylya, Raynaud’s phenomenon, and capillaroscopic abnormalities [1]. LS incidence ranges from 0.4 to 2.7 per 100,000 people [2]. Although present in all races, the prevalence among Caucasians is increased, summing up 72 to 82% of the patients [2]. Females are primarily affected [1], and a similar distribution between children and adults occurs [1, 3]. Disease incidence peaks in the fifth decade of life in adults, whereas 90% of children are diagnosed between 2 and 14 years of age [1, 3–5]. Linear scleroderma en coup de sabre (LCsc) is a rare subset of LS. The typical presentation affects frontoparietal region, and the mean age of onset is around 13 years old [1]. In this paper, clinical presentation of LScs and its neurological
Ipsilateral Coat′s reaction in the eye of a child withen coup de sabre morphoea-A case report  [cached]
Neki A,Sharma A
Indian Journal of Ophthalmology , 1992,
Abstract: A female child with post-traumatic en coup de sabre type of morphoea (fronto-parietal circumscribed scleroderma) involving the left side of the forehead and face, who developed a Coat′s disease-like fundus picture over the following two years, is being reported.
Brain cavernomas associated with en coup de sabre linear scleroderma: Two case reports
Emily T Fain, Melissa Mannion, Elena Pope, Daniel W Young, Ronald M Laxer, Randy Q Cron
Pediatric Rheumatology , 2011, DOI: 10.1186/1546-0096-9-18
Abstract: Localized scleroderma encompasses the conditions of linear scleroderma (LS) (extremity and facial), plaque or circumscribed morphea, pansclerotic and generalized morphea. By definition, localized scleroderma involves the skin and underlying tissue. This is opposed to systemic scleroderma, which not only involves skin but includes internal damage to the lungs, gastrointestinal tract, and vascular system.Linear scleroderma is a form of localized scleroderma that primarily affects the pediatric population. Up to two-thirds of patients given this diagnosis are under the age of 18, and males and females are equally affected [1,2]. The lesions are typically sclerotic and discolored. When these lesions occur on the scalp or forehead, they are termed "en coup de sabre" [3-6]. In the en coup de sabre subtype, extracutaneous associations, mostly neurological, have been described [7]. However, internal organ involvement beyond the central nervous system (CNS) is extremely rare.Linear scleroderma en coup de sabre is typically ipsilateral and near the midline of the forehead with extension into the frontoparietal scalp. It can be associated with hair loss and significant sclerosis of the skin. Often en coup de sabre lesions coexist with Parry-Romberg Syndrome (PRS), which involves hemiatrophy of the subcutaneous tissue and bones of the face without skin or scalp involvement [3-5,8].The most commonly described brain lesions in LS en coup de sabre are intracranial calcifications, which are characteristically ipsilateral to the skin lesions, and appear as hypointense white matter lesions on T2-weighted magnetic resonance imaging (MRI) [5,6,9]. Hyperintense white matter lesions on T2-weighted MRI are also commonly seen in these patients [10]. Other neuroimaging abnormalities described in LS en coup de sabre include cerebral hemiatrophy, hyperintensities in deep white and gray matter structures, nonspecific alterations in cerebral vasculature [11], and intracranial aneurysms [12].Cer
Systemic sclerosis, localized morphea, en coup de sabre and aortic regurgitation: A rare association  [cached]
Sharma Y,Sawhney M,Srivastava S
Indian Journal of Dermatology, Venereology and Leprology , 2004,
Abstract: A 24-year-old male presented with Raynaud′s phenomenon, digital infarcts, salt and pepper pigmentation and characteristic facies. There was gastrointestinal involvement clinically, endoscopically as well radiologically. In view of these findings and the demonstration of anti-nuclear antibodies with a homogeneous pattern of immunofluorescence and ScL-70 antibodies, he was diagnosed as a case of diffuse cutaneous systemic sclerosis. In addition, the patient had plaques of localized morphea, en coup de sabre with facial hemiatrophy on the left side, and mild aortic regurgitation (detected on echocardiography). The occurrence of these rare associations of localized plaques of morphea, en coup de sabre and aortic regurgitation in a single case of systemic sclerosis is quite an exceptional and interesting occurrence.
A Rare Cause of Late-Onset Epilepsy: Linear Scleroderma en Coup de Sabre  [PDF]
Haoyue Zhu, Xiuli Shang
Case Reports in Clinical Medicine (CRCM) , 2016, DOI: 10.4236/crcm.2016.54028
Abstract: Late-Onset Epilepsy (LOE), with onset in adult life, is often attributed to cerebrovascular disease and intracranial tumor. Herein we present a LOE patient with history of Linear Scleroderma en Coup de Sabre (LScs) and abnormal cranial MRI signs. Curiously, his band-like skin lesion, presenting on the forehead, was in line with the surface projection of the intracranial focus shown in MRI. This gave a clue of the link between the skin lesion and the intracranial focus and the epilepsy. To sum up, it exposed a rare cause of LOE. Moreover, it underlined the significance of recognizing the cause to be associated with a substantially increased risk of developing epilepsy.
"Scleroderma linearis: hemiatrophia faciei progressiva (Parry-Romberg syndrom) without any changes in CNS and linear scleroderma "en coup de sabre" with CNS tumor
Beata Bergler-Czop, Anna Lis-?wi?ty, Ligia Brzezińska-Wcis?o
BMC Neurology , 2009, DOI: 10.1186/1471-2377-9-39
Abstract: We present two cases of a disease: a case of a 49-year-old woman with a typical image of hemifacial atrophy, without any changes of the nervous system and a case of a 33-year-old patient with an "en coup de sabre" scleroderma and with CNS tumor.We described typical cases of a rare diseases, hemifacial atrophy and "en coup de sabre" scleroderma. In the patient diagnosed with Parry-Romberg syndrome, with Borrelia burgdoferi infection and with minor neurological symptoms, despite a four-year case history, there was a lack of proper diagnosis and treatment.In the second patient only skin changes without any neurological symptoms could be observed and only a precise neurological diagnosis revealed the presence of CNS tumor.Hemifacial atrophy (Parry-Romberg syndrome) is a relatively rare disease. The etiology of the disease is not clear. Some authors postulate its relation with limited scleroderma linearis [1,2]. In a number of patients, neurological affection is the medium of the disease. Cory et al. [3] consider the origin of the disease to be a non-infectious, one-sided inflammatory process, connected with chronic, vasomotoric disturbances and with sympathetic nerves inflammation. Trauma appear in some patients. In some cases, the disease had of hereditary and autosomal character [1,3]. Clinically it comes to a face deformation, including skin, subcutaneous tissue and bones. A face asymmetry becomes visible. One side of a face seems smaller. The affected side eyeball is situated deeper, the mouth angle is lifted. The skin changes can include the hairy skin of the head, eyebrows and lashes, resulting in baldness focuses formation. The skin within the changes is of an increased compactness, tension and often it becomes over-colored or decolorized. Some patients suffer from headaches, which can be located in the area of trigeminal nerve innervations, and from temporary sensor disturbances [4]. Focal epilepsy on the opposite side can sometimes be diagnosed after neurologic
Lichen sclerosus et atrophicans, scleroderma en coup de sabre and Lyme borreliosis  [cached]
Serena Bonin,Nicoletta Gubertini,Giusto Trevisan
Dermatology Reports , 2011, DOI: 10.4081/dr.2011.e27
Abstract: Lichen sclerosus et atrophicans (LSA) is a chronic, inflammatory skin disease of unknown etiology, characterized by atrophy. We report a case of LSA with frontoparietal distribution, mimicking scleroderma en coup de sabre, causing scarring alopecia. The case was associated with Borrelia infection. The lesion improved with 2 cycles of antibiotic therapy with ceftriaxone 2 gr /day i.v for 21 days associated with UVA-1 therapy and local and systemic vitamin E supply (400 mg 2x/day per os for 3 months). This case stresses the importance of identifying clinical manifestations associated with Lyme disease and the use of tissue PCR to detect borrelial DNA in patients with these lesions, but characterized by negative serology for Borrelia.
Po owiczy zanik twarzy bez zmian w o rodkowym uk adzie nerwowym i twardzina en coup de sabre z guzem zewn trzmózgowym – opis przypadków
Beata Bergler-Czop,Anna Lis-?wi?ty,Ligia Brzezińska-Wcis?o
Przegl?d Dermatologiczny , 2011,
Abstract: Introduction. Progressive facial hemiatrophy (Parry-Romberg syndrome)is a relatively rare disease of unclear aetiology. Some authorspostulate its relation with linear scleroderma.Objective. To present 2 cases: facial hemiatrophy and linear sclerodermalocalized on the head with different clinical picture.Case report. We present two cases: a case of a 49-year-old woman witha typical picture of hemifacial atrophy, without any changes in the centralnervous system; and a case of a 33-year-old patient with sclerodermaen coup de sabre and extracerebral tumour. In a patient diagnosedwith Perry-Romberg syndrome, with Borrelia burgdorferi infectionand minor neurological symptoms, for the 4-year duration of thedisease the proper diagnosis and therapy have not been established. Inthe second patient only skin changes without any neurological symptoms were observed, but through investigations of the central nervoussystem revealed the presence of an extracerebral tumour.Conclusions. The presented cases, especially the second one, indicatethe necessity of detailed neurological examination in patients with progressivefacial hemiatrophy and scleroderma en coup de sabre in spite ofa lack of subjective complaints.
Bilateral linear scleroderma "en coup de sabre" associated with facial atrophy and neurological complications
Thilo Gambichler, Alexander Kreuter, Klaus Hoffmann, Falk G Bechara, Peter Altmeyer, Thomas Jansen
BMC Dermatology , 2001, DOI: 10.1186/1471-5945-1-9
Abstract: A 23 year old woman presented bilateral LSCS and facial atrophy. The patient had epileptic seizures as well as oculomotor and facial nerve palsy on the left side which also had pronounced skin involvement. Clinical features of different stages of the disease are presented.The findings of the presented patient with bilateral LSCS and facial atrophy provide further evidence for a neurological etiology of the disease and may also indicate that classic progressive facial hemiatrophy (Parry-Romberg syndrome) and LSCS actually represent different spectra of the same disease.Linear scleroderma "en coup de sabre" (LSCS) is a subtype of localized scleroderma (Tab. 1). LSCS, usually developing in the first or second decade of life, presents as band-like sclerotic lesions with more or less marked skin discoloration of the frontoparietal area. Characteristically, LSCS manifests unilaterally and does not extend below the eyebrow. The active stage usually lasts two to five years. Involutionary atrophy of skin, muscle, and even bone may occur. Neurological and ophthalmological abnormalities are not infrequent in LSCS [1-3]. We present an uncommon case of bilateral LSCS with facial atrophy and several neurological complications.A 23-year-old woman presented with band-like sclerotic lesions and cicatricial alopecia on the left frontoparietal area (upper trigeminal dermatome). In this area, there was remarkable atrophy of skin and underlying tissues. She had complete ptosis of the left eye. Veins was clearly seen through the atrophic skin in the left temporal region. There was also a remarkable subcutaneous cleft above the right eyebrow and a nearly complete loss of the left eyebrow (Figs 1,2,3,4,5). The tongue or gums were not involved. The patient refused a skin biopsy from the frontal area. Onset of the disease was reported to have been at age 7, with a small scleroderma-like lesion on the left forehead. The lesion was asymptomatic and not preceded by trauma. Over the succeeding y
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