نتایج جستجو برای: neurofibromatosis nf2

تعداد نتایج: 7346  

2015
Jan Kalimullah Abdul Malik Amir Humza Sohail Rai Dilawar Shahjehan Sabeehuddin Siddique Muhammad Ehsan Bari

BACKGROUND Neurofibromatosis type 2 (NF2) is an autosomal dominant syndrome with a frequency of 1 in 25,000 live births and a penetrance of almost 100% by the sixth decade of life. The main tumors occurring in NF2 patients are bilateral vestibular schwannomas, other peripheral, cranial and spinal nerve schwannomas, intracranial and intraspinal meningiomas, ependymomas, and gliomas. CASE DESCR...

Journal: :Cancer research 1999
K Ueki C Wen-Bin Y Narita A Asai T Kirino

Mutations of NF2, the gene for neurofibromatosis 2, are detected in 20-30% of sporadic meningiomas, and almost all mutations lead to loss of merlin expression. However, loss of heterozygosity (LOH) at chromosome 22q is found at a much higher frequency, up to 50-70%, and the possibility of another tumor suppressor gene in this region has not been excluded. Furthermore, a recent report proposed t...

Journal: :Neurology 2021

Neurofibromatosis (NF) type 1 (NF1), NF 2 (NF2), and schwannomatosis are genetic tumor predisposition syndromes with a wide variety of clinical manifestations. Effective medical treatments for these conditions remain limited. Since its founding in 2011, the Response Evaluation Schwannomatosis (REiNS) International Collaboration has worked to develop standardized, practical, clinically meaningfu...

Journal: :acta medica iranica 0
mohammad hassan khalessi

fourteen patients with bilateral low frequency sensorineural hearing loss were found with disproportionate speech discrimination scores. these patients were submmitted to a comprehensive audiological, electrophysiological, neurological, and radiological battery of investigation. the results pointed to a lesion probably in the central auditory pathway.

2015
A. O. Stemmer-Rachamimov R. Alston Stavros M Stivaros Anat O Stemmer-Rachamimov Robert Alston Scott R Plotkin Joseph B Nadol Alicia Quesnel Jennifer O’Malley Gillian A Whitfield Martin G McCabe Simon R Freeman Simon K Lloyd Neville B Wright John-Paul Kilday Ian D Kamaly-Asl Samantha J Mills Scott A Rutherford Andrew T King D Gareth Evans

The Harvard community has made this article openly available. Please share how this access benefits you. Your story matters. " Multiple synchronous sites of origin of vestibular schwannomas in neurofibromatosis Type 2. ABSTRACT Background Neurofibromatosis Type 2 (NF2) is a dominantly inherited tumour syndrome with a phenotype which includes bilateral vestibular (eighth cranial nerve) schwannom...

Journal: :JAMA dermatology 2018
Elisabeth Castellanos Adrià Plana Cristina Carrato Meritxell Carrió Inma Rosas Emilio Amilibia Francesc Roca-Ribas Cristina Hostalot Alicia Castillo Andrea Ros Ariadna Quer Juan Luis Becerra Hector Salvador Conxi Lázaro Ignacio Blanco Eduard Serra Isabel Bielsa

Importance Neurofibromatosis type 2 (NF2) is a devastating genetic condition characterized by the development of multiple tumors of the nervous system. An early diagnosis of individuals with NF2 would facilitate treatment and reduction of disease impact because most severe effects of the disease do not usually develop before adolescence. Little attention has traditionally been paid to dermatolo...

Journal: :Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia 2018
V Jayanetti A I Klistorner S L Graham M Dexter M P Flaherty K Jones F A Billson M Wilson K North J R Grigg C L Fraser

Monitoring optic nerve sheath meningiomas (ONSM) in Neurofibromatosis type 2 (NF2) patients remains difficult. Other ocular manifestations of NF2 may obscure ophthalmic assessment of optic nerve function in these patients. Serial magnetic resonance imaging (MRI) used to assess the optic nerve is not without limitations, being expensive and often requiring general anaesthetic in children, with a...

2016
Matthew L. Carlson Jamie J. Van Gompel

OBJECTIVE To report a case of a patient presenting with two separate unilateral vestibular schwannomas (VSs) without other stigmata of neurofibromatosis type 2 (NF2). STUDY DESIGN This article discusses a case report and review of the literature. SETTING Tertiary academic referral center. PARTICIPANTS A 41-year-old female was referred for evaluation of a left-sided 1.8-cm cerebellopontine...

2013
Fabio P. Nunes Vanessa L. Merker Dominique Jennings Paul A. Caruso Emmanuelle di Tomaso Alona Muzikansky Fred G. Barker Anat Stemmer-Rachamimov Scott R. Plotkin

Bevacizumab treatment can result in tumor shrinkage of progressive vestibular schwannomas in some neurofibromatosis 2 (NF2) patients but its effect on meningiomas has not been defined. To determine the clinical activity of bevacizumab against NF2-related meningiomas, we measured changes in volume of meningiomas in NF2 patients who received bevacizumab for treatment of progressive vestibular sch...

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