Also known as painful ophthalmoplegia syndrome, Hunter syndrome.
The etiology and mechanism are unknown. It may be related to abnormal immune mechanism. Pathologically, the cavernous sinus of Shanghai was filled with granulation tissue, which damaged ⅲ, ⅳ, ⅴ 1 and 856438+0 cranial nerves.
Clinical manifestations are characterized by Ⅲ, Ⅳ, Ⅴ1,Ⅴ cranial nerve damage, eye pain and headache, which are unilateral and recurrent. Adrenocortical hormone therapy is effective.
The incidence of male and female is equal, which is more common in 30 ~ 40 years old. At first, it was intractable pain in orbit and orbit, and after a few days, ophthalmoplegia appeared. The main manifestations are oculomotor nerve paralysis (Ⅲ), trochlear paralysis (Ⅳ), abduction paralysis (Ⅵ) and paralysis of the first branch of trigeminal nerve (Ⅴ1), and sympathetic nerve fibers and optic nerve around arteries can also be damaged. It can relieve itself after a few days or weeks, and it can recur after months or years. In some cases, after 1 ~ 6 months, the symptoms can disappear even without treatment, but it may also leave lasting damage to the oculomotor nerve, optic nerve or retina. Cerebral angiography showed that the siphon of internal carotid artery was narrow and the superior ophthalmic vein and cavernous sinus were occluded, but no external lesions of cavernous sinus were found by cerebral angiography and surgical exploration.
ELAPRASETM can be used in enzyme replacement therapy for Hunter's syndrome. ELAPRASETM is the first drug approved for the treatment of Hunter's syndrome. Once a week, the drug can supplement the deficiency of enzyme level in patients with Hunter's syndrome and achieve the purpose of treating diseases.