Tolosa-Hunt症候群の1例

TOLOSA-HUNT SYNDROME : REPORT OF A CASE

I．はじめに

　Tolosa-Hunt （T-H）症候群は，一側性の有痛性眼球運動麻痺を主症状とし，海綿静脈洞周辺の限局性の非特異的肉芽腫炎症がその原因とされ，ステロイドが有効なものである。この疾患は，1954年Tolosa^23）によつて報告され，その後Hunt^6）によつてその臨床概念が確立された。木症候群は，最近本邦でも報告されているが，臨床症状をひき起こす海綿静脈洞の閉塞とその再開通を確認した報告は稀であり，また全身の炎症症状も稀である。最近，われわれは，眼窩静脈造影にて，一側海綿静脈洞の完全閉塞を認めたT-H症候群でステロイド治療により臨床症状の改善と海綿静脈洞の一部再開通を認めた一症例を経験したので文献的考察を加えて報告する。

Tolosa Hunt syndrome is characterized by unilateral painful ophthalmoplegia due to localized nospecific granulomatous inflammation in the cavernous sinus. Steroid therapy is effective on this syndrome. After Tolosa first reported an autoposied case of this syndrome in 1954, Hunt et al. outlined criteria for diagnosis of the syndrome. Many such cases have recently been recognized in Japan. The purpose of this communication isto report a case of this syndrome, in which the narrowing of the carotid syphon and occulusion of the cavernous sinus during early course of his illness and the recanalization of the cavernous sinus on recovery of clinical symptoms were demonstrated.

Case: A 23-year-old male was admitted to the neurological ward at Jichi Medical School Hospital because of sustained pain in the right orbit and diplopia on right lateral gaze for three weeks duration.

General physical examination was normal except for the face. The blood pressure was 108/60 mmHg and the temperature was 37.1℃. In the right eye, tenderness, photophobia and slightly hyperemic conjunctiva were noted. Superficial temporal scalp veins were bilaterally engorged.

Neurological examination revealed visual acuity of 0.06 bilaterally which had been unchanged and was thought due to miopia and coned cornea. Complete abducens paralysis of the right eye and hypesthesia of the first and second devisions of the right trigeminal nerve were present. There was no meningeal sign. Other neurological examina-tions were normal.

Laboratory examination: The white-cell count was 6900 with normal differentials, the hematocrit was 41.8%, the platelet count was 18.8×10⁴, the erythrocyte sedimentation rate was 8 mm/hr and the blood coagulability was nomal. The CRP, TPHA, LE prep. and antinuclear antibody were all negative. The thyroid function and the glucose talerance tests were normal. Tuberculin skin test was equivocal. The cerebrospinal fluid contained 6/3 monocytes per cubic millimeter ; the glucose was 76 mg/di and the protein 32 mg/di. The sero-logical, bacterial and viral examination of cerebro-spinal fluid were all negative. X-ray films of the mastoids, nasal sinuses and Skull were normal. A CT scan failed to show any abnormalities. A carotid angiography revealed a narrowing of the right internal carotid artery. An orbital phlebo-graphy demonstrated nonfilling of third segment of the right inferior petrosal sinus as well as cavernous sinus.

Under the diagnosis of Tolosa Hunt Syndrome, 60 mg per day of prednisolone was initiated. An gradual improvement of clinical symptoms and signs followed and almost complete recovery occurred within three weeks. On the 58th day of steroid therapy, another orbital phlebography showed recanalization of the right cavernous sinus.