Alshalchy Ali, Al-Taie Rania H, Al-Badri Sajjad G, Bani Saad Mohammed A, Bani-Saad Ali A, Ismail Mustafa
Department of Surgery, College of Medicine, University of Baghdad, Baghdad, IRQ.
Department of Surgery, College of Medicine, University of Mustansiriyah, Baghdad, IRQ.
Cureus. 2024 Sep 25;16(9):e70197. doi: 10.7759/cureus.70197. eCollection 2024 Sep.
Garcin syndrome is a rare neurological condition characterized by progressive unilateral involvement of multiple cranial nerves, without typical intracranial hypertension. It is often linked with aggressive malignancies and invasive infections; hence, it presents significant diagnostic and therapeutic challenges. Despite the advances in medical technology, the prognosis still remains poor, and there is limited literature on comprehensive reviews regarding its etiology, diagnosis, and management. A Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)-based systematic review was performed in order to compile updated evidence related to Garcin syndrome. The PubMed and Scopus databases were comprehensively searched using the search terms "Garcin syndrome" AND ("etiology" OR "diagnosis" OR "treatment" OR "management"). Information was obtained from case reports and focused on common etiologies, clinical presentations, diagnostic methodologies, treatment protocols, and outcomes. The review discussed very diverse etiologies of Garcin syndrome. The most common among these were skull base tumors, metastatic lesions, and invasive infections like mucormycosis. Most of these were multiple cranial nerve (CN) involvements in which CN V (trigeminal nerve), CN VII (facial nerve), or CN XII (hypoglossal nerve) involvement was common. Advanced imaging, especially MRI, played a very crucial role in diagnosis, showing the presence of extensive bony destruction with the involvement of cranial nerves. Treatment was varied according to the etiology, ranging from chemotherapy and radiotherapy in neoplastic cases to active surgical intervention supported by antifungal therapy in infected cases. Garcin syndrome is a clinical challenge due to its diverse etiologies and complex management profile. While early diagnosis and intervention are emphasized, the prognosis remains grave, especially in cases presenting with metastatic disease or immunocompromised states. Future research should focus on better, more sensitive diagnostic modalities and the investigation of newer therapeutic approaches for Garcin syndrome patients.
加欣综合征是一种罕见的神经系统疾病,其特征为多组颅神经进行性单侧受累,且无典型的颅内高压。它常与侵袭性恶性肿瘤和侵袭性感染相关;因此,它带来了重大的诊断和治疗挑战。尽管医学技术有所进步,但其预后仍然很差,关于其病因、诊断和管理的综合综述的文献有限。为了汇编与加欣综合征相关的最新证据,进行了一项基于系统评价和Meta分析的首选报告项目(PRISMA)的系统评价。使用搜索词“加欣综合征”以及(“病因”或“诊断”或“治疗”或“管理”)对PubMed和Scopus数据库进行了全面搜索。从病例报告中获取信息,并关注常见病因、临床表现、诊断方法、治疗方案和结果。该综述讨论了加欣综合征非常多样的病因。其中最常见的是颅底肿瘤、转移性病变和毛霉菌病等侵袭性感染。这些大多是多组颅神经受累,其中以第V对颅神经(三叉神经)、第VII对颅神经(面神经)或第XII对颅神经(舌下神经)受累最为常见。先进的影像学检查,尤其是磁共振成像(MRI),在诊断中起着至关重要的作用,显示存在广泛的骨质破坏并伴有颅神经受累。治疗根据病因而异,肿瘤病例采用化疗和放疗,感染病例采用抗真菌治疗支持下的积极手术干预。加欣综合征因其多样的病因和复杂的管理情况而成为临床挑战。虽然强调早期诊断和干预,但其预后仍然严峻,尤其是在出现转移性疾病或免疫功能低下状态的病例中。未来的研究应专注于更好、更敏感的诊断方法以及对加欣综合征患者新治疗方法的研究。