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
{"title":"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。","authors":"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","doi":"10.1080/01676830.2025.2562356","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":47421,"journal":{"name":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","volume":" ","pages":"1-6"},"PeriodicalIF":0.8000,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Orbital apex-like syndrome with multiple cranial neuropathies secondary to chronic inflammatory demyelinating polyneuropathy.\",\"authors\":\"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\",\"doi\":\"10.1080/01676830.2025.2562356\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":47421,\"journal\":{\"name\":\"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery\",\"volume\":\" \",\"pages\":\"1-6\"},\"PeriodicalIF\":0.8000,\"publicationDate\":\"2025-09-25\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1080/01676830.2025.2562356\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"OPHTHALMOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1080/01676830.2025.2562356","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"OPHTHALMOLOGY","Score":null,"Total":0}
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.
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.