Tolosa-Hunt syndrome—症例報告とclinical criteriaに関する検討

TOLOSA-HUNT SYNDROME:REPORT OF A CASE AND COMMENT ON ITS NEW CLINICAL CRITERIA

I．はじめに

　反復する片側性頭痛・眼球痛発作，複視または視力障害を主徴とするTolosa-Hunt症候群は^11,21），Ⅲ・Ⅳ・Ⅵ脳神経群，Ⅴ脳神経第1枝及びⅡ脳神経が相互に接近する解剖学的部位—海綿静脈洞から上眼窩裂，眼窩先端部に至る部位—に発生する非特異的炎症性疾患として注目されているが，いまだ本邦における報告は少ない。

　最近著者等は脊髄腔内steroid投与により短期間に著効を示したTolosa-Hunt syndromeの1例を経験したので，本症候群の臨床的事項，とくにclinical criteriaを中心に若干の文献的考察を加え報告する。

A case of Tolosa-Hunt syndrome was presented and its clinical features were discussed in relation to the new clinical criteria proposed by Hunt in 1976.

The patient was a 23-year-old woman. Her chief complaints were right frontotemporal and retro-orbital remittent pain, transient right ptosis, double vision and visual disturbance.

On examination, papilledema, mydriasis, upper-nasal visual field defect and loss of visual acuity were noticed on the right side. The right third, fourth and sixth cranial nerves and the first branch of the right fifth cranial nerve were found to be impaired.

A right parasellar tumor was suspected at first, but it was excluded subsequently by carotid angio-graphy, orbital venography, brain scintigraphy, pneumoencephalography and CSF examination. Blood and urine examinations as well as otolar-yngological examination were nothing particular and syphilis was excluded. Under the diagnosis of Tolosa-Hunt syndrome, she was treated by intra-thecal administration of 8 mg of Dexamethason twice per week. In 3 weeks headache, right retro-orbital pain and sensory disturbance of the right forhead disappeared rapidly, although right mydri-asis and slight diplopia on upward gaze persisted.

During the follow-up period of 27 months, the patient's condition has remained the same and there has been no recurrence of previous symptoms.

The findings and clinical course of this case fit well to the new criteria of Tolosa-Hunt syndrome recently propose by Hunt. In this connection the new criteria of the disease was introduced and discussed on its various aspects.