The patient relapsed 3 months later with headache and a transient ischaemic attack while on prednisolone 30 mg/day. Labyrinthine failure had developed. Prednisolone was increased to 1 mg/kg/day and methotrexate was introduced at 15 mg/week. Within 3 months, she had developed left anterior optic neuritis with retinochoroidal oedema causing reduced visual acuity (6/24) and colour vision (15/17 Ishihara). On MRI brain scan, there was progression of ischaemic changes. Topical steroid was given and methotrexate was increased and continued at 20 mg/week. Acuity and colour vision improved to normal. Prednisolone was tapered slowly to 10-15 mg/day. Neither the keratitis nor the uveitis has recurred to date. The patient has been stable for 2 years.
CommentCS presents almost equally to ophthalmology and ENT and median time to second organ involvement is 1 month. 5 At least one serious outcome (blindness, deafness, vasculitis, aortic insufficiency, or death) occurs in 63% of cases. 2 In all, 10% of patients have neurologic involvement; 2,5 although widespread CNS ischaemia has been reported in only two individuals. 5 About 30% of CS is atypical and more likely to be associated with systemic features and a poorer prognosis. 3,4 Our patient had an atypical ophthalmic mode of presentation, which probably represented acute on chronic angle closure glaucoma. Furthermore, and in keeping with her atypical CS, she had severe, widespread neurologic involvement with CNS ischaemia, optic neuritis, and peripheral neuropathy.The most common serious complication of CS is deafness, which may be ameliorated if oral steroids are started within 2 weeks of onset. 3 Ocular inflammation frequently responds to topical steroids. Systemic vasculitis usually responds to oral steroids, although aortitis may require aortic valve replacement.The role of other immunosuppressants is not established and, owing to the rarity of CS, may depend upon the collected experience of case reports. Three patients with diagnosed CS, who underwent methotrexate therapy experienced stabilised hearing and were able to reduce or discontinue steroids. 6 Our experience supports the use of methotrexate in steroidresistant patients, especially those with CNS involvement.We recommend that all patients with CS, especially atypical cases, should have regular ENT and ophthalmic assessments and that neurologic symptoms require urgent assessment. Such collaboration will help ensure appropriate and timely therapy.