Mutsukura Kazuo, Tsuboi Yoshio, Imamura Akiko, Fujiki Fujio, Yamada Tatsuo
5th Department of Internal Medicine, Fukuoka University School of Medicine, 7-45-1 Nanakuma, Johnan-ku, Fukuoka 814-0180, Japan.
No To Shinkei. 2004 Mar;56(3):231-5.
Garcin syndrome is characterized by an unilateral cranial nerves involvement without sensory or motor long-tract disturbances. It is usually caused by tumor infiltrating in the skull base with osteolytic changes on radiological study. We report a case of 64-year-old man with history of alcohol overintake, who admitted local hospital, because of right periorbital edema and facial swelling. He noted right ptosis 2 weeks prior to admission. Neurological examination revealed right multiple cranial nerves involvement including II, III, IV, V, and VI cranial nerves. MR imaging of the brain showed marked paranasal sinusitis and abnormal infiltration of right orbital fat. Orbital apex syndrome related to paranasal sinusitis was diagnosed, and antibiotics was administered. But a few days after admission, he developed a right VII, IX, X cranial nerve palsy. He was transferred to our hospital because of acute development of left hemiparesis and deteriorated consciousness. MR imaging of the brain showed right internal carotid artery (ICA) occlusion, and infarction in right middle cerebral artery (MCA)'s territory. The diagnostic biopsy of the paranasal sinus showed mucorales hyphae, indicating that the pathological diagnosis was mucormycosis. Despite of antibiotic therapy included of amphotericin-B administration and strict control of diabetic mellitus, his sinusitis was gradually spread. His condition progressively deteriorated, and finally died of sepsis. Post-mortem examination revealed a widespread mucor infiltration in the dura mater without skull bone invasion. This case presented with unilateral multiple cranial nerve involvements (Garcin syndrome) followed by left hemiparesis associated with rhinocerebral mucormycosis. It is suggested that mucormycosis should be considered in case of Garcin syndrome without osteolysis in the skull base.
加欣综合征的特征是单侧颅神经受累,而无感觉或运动长束障碍。它通常由肿瘤浸润颅底引起,影像学检查显示有骨质溶解改变。我们报告一例64岁男性,有酒精摄入过量史,因右眶周水肿和面部肿胀入住当地医院。他在入院前2周注意到右眼上睑下垂。神经系统检查发现右侧多条颅神经受累,包括第II、III、IV、V和VI颅神经。脑部磁共振成像显示明显的鼻窦炎和右眶脂肪异常浸润。诊断为与鼻窦炎相关的眶尖综合征,并给予抗生素治疗。但入院几天后,他出现了右侧第VII、IX、X颅神经麻痹。由于急性出现左侧偏瘫和意识恶化,他被转到我院。脑部磁共振成像显示右颈内动脉闭塞,右大脑中动脉供血区梗死。鼻窦诊断性活检显示毛霉目菌丝,表明病理诊断为毛霉菌病。尽管进行了包括两性霉素B治疗和严格控制糖尿病在内的抗生素治疗,他的鼻窦炎仍逐渐蔓延。他的病情逐渐恶化,最终死于败血症。尸检显示硬脑膜广泛毛霉浸润,无颅骨侵犯。该病例表现为单侧多颅神经受累(加欣综合征),随后出现与鼻脑型毛霉菌病相关的左侧偏瘫。提示在无颅底骨质溶解的加欣综合征病例中应考虑毛霉菌病。