神经性眼肌强直

目的探讨神经性眼肌强直的发病原因、临床特点及诊断与治疗。方法报告神经性眼肌强直4例并结合复习相关文献,对本病的病因、诊断及治疗进行深入分析和讨论。结果例1,66岁,女性,内直肌痉挛,多年前,因垂体肿瘤接受过放疗。 痉挛发生于凝视第一眼位,并可由偏心注视引发。例2,72岁,男性,多年前接受放射性二氧化钍脊髓造影,发生慢性蛛网膜炎。偏心注视引起第三和第六神经支配的肌肉痉挛。这2例病人经卡马西平治疗,神经性眼肌强直停止。例3,47岁,男性,偏心注视时一侧直肌强直。几年前,因脑干成神经管细胞瘤接受放疗。因癫痫用卡马西平治疗,卡马西平用量减小,发生痉挛;用量增加.痉挛停止。例4,21岁,男性,颈部,头部,脑部先天性血管瘤反复发作,几年前部分摘除,上斜肌强直。向下注视引起痉挛并可自行缓解。结论神经性眼肌强直可出现干颅内病变治疗后数月到数年。典型的颅内病变的治疗是颅内肿瘤的放疗,但其它原因造成的颅内神经损伤也能引起眼肌神经性强直。由第三,四,六神经支配的眼外肌痉挛直接造成斜视和复视,持续几秒到几分钟。痉挛可自发产生,也可由偏心注视刺激相关颅神经诱发。神经放射性检查有时可显示海绵窦内或周围异常,但通常不显示原发肿瘤的复发。这种综合征极少自愈,但卡马西平治疗效果显著。

The Spectrum of Ocular Neutomyotonia

Objective To probe into the causes, clinical features, diagnosis and treatment of ocular neuromyotonia (ONM). Methods To deeply analyze and discuss the causes, clinical features, diagnosis an treatment of this disease by reporting 4 cases of ONM and reviewing references concerned. Results A 66 year-old woman developed spasms of a medial rectus muscle many years after irradiation for a pituitary tumor. The spasms occurred in the primary position of gaze and were induced by eccentric gaze. A 72 year-old man had chronic arachnoiditis after myelography with radioactive thorium dioxide many years ago. Spasms of muscles innervated by a third nerve and a sixth nerve were produced by eccentric gaze. Treatment with carbamazepine stopped the ONM in both patients. A 47 year-old man had ONM of a lateral rectus muscle when he looked in eccentric gaze several years after irradiation for a cerebellar medulloblas-toma. The spasms occurred when treatment for seizures with carbamazepine was decreased, and resolved with the dosage was increased. A 21 year-old man had recurrence of a congenital hemangioma of the neck, face and brain that caused ONM of a superior oblique muscle many years after it had been partially excised. Downward gaze caused the spasms, and they resolved spontaneously. Conclusion ONM begins months to years following treatment for intracranial lesions. Typically, the treatment is radiation for an intracranial tumor, but other causes of damage to the cranial nerves can produce the syndrome. Spasm of extraocular muscles innervated by the third, fourth or sixth cranial nerves produce strabismus and diplopia, lasting from a few seconds to a few minutes. The spasms are spontaneous or induced by eccentric gaze that stimulates the affected cranial nerve. Neuroradiologic tests might show abnormalities in or near the cavernous sinus, but usually do not show recurrence of the original tumor. The syndrome rarely resolves spontaneously, but responds well to treatment with carbamazepine.