Xia Nian-Ge, Chen Yan-Yan, Wang Xin-Shi, Xu Hui-Qin, Zheng Rong-Yuan
Department of Neurology, the First Affiliate Hospital of Wenzhou Medical University, Wenzhou City, Zhejiang, P.R. China.
Medicine (Baltimore). 2017 Nov;96(45):e8508. doi: 10.1097/MD.0000000000008508.
Garcin syndrome is characterized by the gradual involvement, and ultimately, unilateral paralysis of at least 7 and sometimes all cranial nerves, without intracranial hypertension or any long tract signs.
We report the case of a 59-year-old woman who presented with Garcin syndrome, which gradually progressed over a period of 2 years.
A left parotid gland biopsy revealed parotid gland adenoid cystic carcinoma (PGACC) with perineural invasion of a peripheral nerve bundle and lymph node metastasis.
The patient was treated 3 times with local-field palliative radiotherapy.
She died after several months.
To the best of our knowledge, this is the first report of PGACC presenting as Garcin syndrome. PGACC is a rare tumor with a high propensity for perineural spread, and it should be considered as a possible cause of Garcin syndrome.
加欣综合征的特点是至少7对颅神经逐渐受累,最终出现单侧麻痹,有时所有颅神经均受累,且无颅内高压或任何长束征。
我们报告了一例59岁女性患者,患有加欣综合征,该综合征在2年时间里逐渐进展。
左侧腮腺活检显示为腮腺腺样囊性癌(PGACC),伴有外周神经束的神经周围浸润和淋巴结转移。
患者接受了3次局部姑息性放疗。
几个月后患者死亡。
据我们所知,这是首例表现为加欣综合征的PGACC报告。PGACC是一种罕见肿瘤,具有较高的神经周围扩散倾向,应被视为加欣综合征的可能病因。