双侧动眼神经神经鞘瘤表现为上睑下垂和眼动神经麻痹-一种罕见的神经纤维瘤病的表现

P. Saggar, Vineet Saggar
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摘要

神经纤维瘤病2 (NF2)是一种罕见的常染色体显性遗传病,其特征是多发性神经系统肿瘤的发展。双侧前庭神经鞘瘤的存在是一个决定性的特征,但NF2患者也会发生其他颅、脊髓、周围神经鞘瘤、颅和脊髓脑膜瘤以及白内障。其他相关的颅内病变是非肿瘤性钙化,最常见的是脉络膜丛受累。小脑和大脑皮质钙化也可见。虽然已知有除第8神经鞘瘤以外的神经鞘瘤发生,但第三神经鞘瘤是NF-2的罕见表现。我们报告一例罕见的双侧眼动神经鞘瘤合并巨大的第5神经鞘瘤,表现为单侧上睑下垂和第三神经麻痹。
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Bilateral Oculomotor Nerve Schwannomas Presenting With Ptosis And Occulomotor Nerve Palasy- A Rare Presentation Of Neurofibromatosis-2
Neurofibromatosis 2 (NF2) is a rare autosomal dominant disorder characterized by the development of multiple nervous system tumors. The presence of bilateral vestibular schwannomas is a defining feature, but patients with NF2 also develop other cranial, spinal, peripheral schwannomas, cranial and spinal meningiomas, and cataracts. Other associated intracranial lesions are non neoplastic calcifications with involvement of the Choroid plexus being most common. Cerebellar andcerebral cortical calcifications may also be seen .Though schwanomas other than 8th nerve schwannomas are known to occur but third nerve schwannomas are a rare presentation of NF-2.We at our institution report a rare case of bilateral occulomotor nerve schwannomas associted with massive 5th nerve schwannoma which presented with unilateral ptosis and third nerve palsy.
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