Optic perineuritis (OPN) refers to a spectrum of conditions involving pathologic inflammation of the optic nerve sheath. The classic triad of OPN consists of unilateral optic neuropathy associated with pain and/or disc oedema, but the condition often mimics other optic neuropathies, resulting in delayed diagnosis and suboptimal treatment. We performed a database search of Medline and Ovid in January 2016 for articles published in any language with the keywords ‘optic perineuritis’. Sixty articles were found, published from 1956 to 2015. Two reviewers (Tai ELM and Tevaraj JMP) performed an independent screening of abstracts. Articles of interest were subsequently examined. In this review, we highlight the salient features of OPN, with particular emphasis on the clinical differences between OPN and optic neuritis. Although the majority of cases of OPN are idiopathic, investigations are required to rule out specific infectious and inflammatory causes of secondary OPN. MRI is an invaluable component of the workup, as radiographic demonstration of peri-neural inflammation is diagnostic of OPN. Corticosteroid therapy results in dramatic and rapid reversal of the signs and symptoms, but prolonged therapy with slow tapering of oral corticosteroids may be necessary to reduce the risk of relapses.
Neurofibromatosis type 2 usually presents with bilateral acoustic schwannomas. We highlight the rare presentation of neurofibromatosis initially involving third nerve. A 23-year-old Malay female presented with left eye drooping of the upper lid and limitation of upward movement for 8 years. It was associated with right-sided body weakness, change in voice, and hearing disturbance in the right ear for the past 2 years. On examination, there was mild ptosis and limitation of movement superiorly in the left eye. Both eyes had posterior subcapsular cataract. Fundoscopy showed generalised optic disc swelling in both eyes. She also had palsy of the right vocal cord, as well as the third and eighth nerve. There was wasting of the distal muscles of her right hand, with right-sided decreased muscle power. Pedunculated cutaneous lesions were noted over her body and scalp. MRI revealed bilateral acoustic and trigeminal schwannomas with multiple extra-axial lesions and intradural extramedullary nodules. Patient was diagnosed with neurofibromatosis type 2 and planned for craniotomy and tumour debulking, but she declined treatment. Neurofibromatosis type 2 may uncommonly present with isolated ophthalmoplegia, so a thorough physical examination and a high index of suspicion are required to avoid missing this condition.
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