眶尖样综合征伴继发于慢性炎性脱髓鞘性多神经病变的多发性颅神经病变。

IF 0.8 Q4 OPHTHALMOLOGY
Gabriella Schmuter, Richard N Polo, Shanlee Stevens, Celestine H Gregerson, Allison Coombs, Kevin Heinze, Abtin Tabaee, Theodore H Schwartz, Gary J Lelli, Kyle J Godfrey
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引用次数: 0

摘要

慢性炎症性脱髓鞘多神经病变(CIDP)是一种罕见的自身免疫性疾病,其特征是周围神经进行性脱髓鞘,导致运动和感觉缺陷。眼部受累,虽然不常见,但可表现为眼麻痹、乳头水肿、视神经病变或眼球突出。本文报告一位49岁男性难治性CIDP患者,眼眶广泛受累,包括双侧脑神经突出和多颅神经扩大,导致眼眶尖样综合征和压迫性视神经病变。患者的病情难以通过静脉注射免疫球蛋白、皮质类固醇、血浆置换和利妥昔单抗进行治疗,最终需要手术减压。术后早期视力症状有所改善。该病例强调了多学科方法在复杂颅神经受累的CIDP病例管理中的重要性,并强调了进一步研究其病理生理学和治疗优化的必要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Orbital apex-like syndrome with multiple cranial neuropathies secondary to chronic inflammatory demyelinating polyneuropathy.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune disorder characterized by progressive demyelination of peripheral nerves, leading to motor and sensory deficits. Ophthalmologic involvement, though uncommon, may manifest as ophthalmoplegia, papilledema, optic neuropathy, or proptosis. This report presents a 49-year-old male with refractory CIDP and extensive orbital involvement, including bilateral proptosis and enlargement of multiple cranial nerves, resulting in an orbital apex-like syndrome and compressive optic neuropathy. The patient's condition was refractory to medical management with intravenous immunoglobulin, corticosteroids, plasma exchange, and rituximab, and he ultimately required surgical decompression. Improvement in visual symptoms was noted in the early post-operative period. This case highlights the importance of a multidisciplinary approach in the management of complex CIDP cases with significant cranial nerve involvement and underscores the need for further research into its pathophysiology and treatment optimization.

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来源期刊
CiteScore
2.40
自引率
9.10%
发文量
136
期刊介绍: Orbit is the international medium covering developments and results from the variety of medical disciplines that overlap and converge in the field of orbital disorders: ophthalmology, otolaryngology, reconstructive and maxillofacial surgery, medicine and endocrinology, radiology, radiotherapy and oncology, neurology, neuroophthalmology and neurosurgery, pathology and immunology, haematology.
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