Navegando por Palavras-chave "neurofibromatosis"
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- ItemSomente MetadadadosDetection of Cerebral Vasculopathy by Transcranial Doppler in Children With Neurofibromatosis Type 1(Sage Publications Inc, 2016) Sagica Fernandes Paschoal, Joelma Karin [UNIFESP]; Paschoal, Fernando Mendes, Jr.; de Lima, Fernanda Teresa [UNIFESP]; Pinho, Ricardo Silva [UNIFESP]; Pereira Vilanova, Luiz Celso [UNIFESP]; Bor-Seng-Shu, Edson; Masruha, Marcelo Rodrigues [UNIFESP]Neurofibromatosis type 1 is characterized by nerve sheath neurofibromas associated with a number of additional clinical features, including cerebrovascular disease. The aim of this study was to use transcranial Doppler as a screening method for identifying cerebral vasculopathy in children with neurofibromatosis type 1. Forty children with neurofibromatosis type 1, aged 5 to 18 years old, were examined by transcranial Doppler. Patients presenting with hemodynamic features of arterial stenosis/occlusion on transcranial Doppler underwent magnetic resonance angiography to confirm the findings. Magnetic resonance angiography was performed on 4 children who exhibited a transcranial Doppler hemodynamic pattern indicative of cerebral vasculopathy. Among these cases, 2 presented internal carotid artery stenosis/occlusion, 1 had bilateral middle cerebral artery stenosis, and 1 presented a normal magnetic resonance angiography result. Transcranial Doppler can be used routinely in the investigation of cerebrovascular disease in neurofibromatosis type 1 patients, where magnetic resonance angiography can be subsequently applied to confirm the diagnosis, further contributing to the prevention of cerebrovascular events.
- ItemAcesso aberto (Open Access)Hemifacial spasm in a patient with neurofibromatosis and Arnold-Chiari malformation: a unique case association(Academia Brasileira de Neurologia - ABNEURO, 2007-09-01) Felício, André Carvalho [UNIFESP]; Godeiro-Junior, Clecio de Oliveira [UNIFESP]; Borges, Vanderci [UNIFESP]; Silva, Sonia Maria de Azevedo [UNIFESP]; Ferraz, Henrique Ballalai [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)BACKGROUND: The association of hemifacial spasm (HFS), Chiari type I malformation (CIM) and neurofibromatosis type 1 (NF1) has not been described yet. CASE REPORT: We report the case of a 31-year-old woman with NF1 who developed a right-sided HFS. On magnetic resonance imaging (MRI) a CIM was seen without syringomyelia. The patient has been successfully treated with botulinum toxin type A injections for 5 years without major side effects. CONCLUSION:Clinical features of HFS, CMI and NF1 are highlighted together with their possible relationship. Also, therapeutic strategies are also discussed.
- ItemSomente MetadadadosMulticenter study of wound healing in neurofibromatosis and neurofibroma(Lippincott Williams & Wilkins, 2007-09-01) Miyawaki, Takeshi; Billings, Brian; Har-Shai, Yaron; Agbenorku, Pius; Kokuba, Elisa [UNIFESP]; Moreira-Gonzalez, Andrea [UNIFESP]; Tsukuno, Mari; Kurihara, Kunihiro; Jackson, Ian T.; Providence Hosp; Jikei Univ; Universidade Federal de São Paulo (UNIFESP); Lady Davis Carmel Med CtrBased on clinical experience, the senior author has become convinced that wounds produced to correct the deformities of patients with neurofibromatosis (NF-1) have produced remarkably good scars, the interesting feature being that progression to keloid or hypertrophic scar is rare. The other point noted was that this situation did not change, no matter the patient's race or skin color. There have been few reports describing or discussing this hypothesis. The purpose of this study was to investigate whether wounds produced in the patients with NF-1 produce keloid or hypertrophic scars. The patients with solitary neurofibroma were also included in this study; these were compared with the NF-1 group. This was conducted as a multicenter study. Patients with neurofibromatosis/solitary neurofibroma, who were operated on from 1990 to 2000, were evaluated by reviewing their medical charts and photographs retrospectively. The patients were treated in centers from five different countries. The analysis was undertaken based on the following points: 1) age and sex at surgery; 2) race of the patients; 3) past and family histories of hypertrophic scar and keloid; 4) surgical site(s); 5) diagnosis, NF1 or solitary neurofibroma; 6) surgical complications; 7) number of reoperations to manage the complications; 8) adjuvant therapy for the tumor; 9) depth of the tumors; and 10) incidence of malignant degeneration. A total of 101 cases with neurofibromatosis or solitary neurofibroma was analyzed. The age at surgery ranged from 1 year 6 months to 74 years; sex ratio was 47 males and 54 females. The racial distribution of the patients was 13 white, 13 black, 3 Hispanic, and 58 Asian. There was no past or family history of hypertrophic scar or keloid. The surgical sites were head and neck in 70 cases, trunk in 20 cases, upper extremities in 22 cases, and lower extremities in 20 cases. The clinical diagnosis was NF-1 in W cases, solitary neurofibroma in 35 cases, plexiform neurofibroma in four cases, and no distinct clinical diagnosis in five cases. There were no other types of neurofibiomatosis. Hematoma and white wide scar were the main postoperative complications found in six cases of NF-1. Infection was also noted in four cases. However, no patient developed hypertrophic scar or keloid in the neurofibromatosis group, whereas two cases showed hypertrophic scar in the solitary neurofibroma group. The outcome showed that the patients with NF-1 and plexiform neurofibroma, no matter the racial group, produce good scars without keloid or hypertrophic changes, whereas solitary neurofibroma has a potential to cause hypertrophic scar.