Garcin症候群を呈した鼻脳ムコール症

Garcin Syndrome in a Patient with Rhinocerebral Mucormycosis

　要旨　症例は64歳でアルコール多飲歴のある男性。右眼球の発赤と腫脹のため近医に搬送され，細菌性副鼻腔炎による眼窩先端症候群が疑われたが，下部脳神経障害が加わりGarcin症候群への進展が認められた。さらに，右内頸動脈閉塞と脳梗塞を併発し当院に転院した。経鼻生検でムコール菌を確認。アンホテリシンB投与後一時軽快が得られたが，脳膿瘍を併発し死亡した。剖検においてムコール菌の硬膜浸潤が認められた。Garcin症候群を呈した鼻脳ムコール症の報告例はきわめて稀であるとともに，臨床経過，剖検所見より，ムコール菌の頭蓋底部の硬膜を介した連続的な進展が機序として考えられた。

　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.

（Received : November 25, 2003）