Neurofibromatosis Type 2 Presenting with Oculomotor Ophthalmoplegia and Distal Myopathy.

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.

Atypical Presentation of Idiopathic Bilateral Optic Perineuritis in a Young Patient.

A previously healthy 27-year-old Malay male presented with acute onset of painless, severe blurring of vision in his right eye. It was associated with headache and vomiting for the past week. Relative afferent pupillary defect was present in the right eye, with reduced optic nerve function. Patient also had bilateral generalised optic disc swelling, splinter haemorrhages, and tortuous vessels. Initial examination was suggestive of either optic neuritis or raised intracranial pressure. Typical features of bilateral optic perineuritis (OPN) such as tram track and doughnut sign were observed on magnetic resonance imaging. Connective tissue and infective screening were negative. He was diagnosed with bilateral optic perineuritis and treated with high dose intravenous corticosteroids followed by a three-month course of oral steroids. His vision and optic nerve function recovered to baseline levels.

Dengue related maculopathy and foveolitis

A 24 year-old Malay lady presented with high grade fever, myalgia, generalized rashes, severe headache and was positive for dengue serology test. Her lowest platelet count was 45 × 10(9) cells/L. She complained of sudden onset of painlessness, profound loss of vision bilaterally 7 days after the onset of fever. On examination, her right eye best corrected vision was 6/30 and left eye was 6/120. Her anterior segment examination was unremarkable. Funduscopy revealed there were multiple retinal haemorrhages found at posterior pole of both fundi and elevation at fovea area with subretinal fluid. Systemic examination revealed normal findings except for residual petechial rashes. She was managed conservatively. Her vision improved tremendously after 2 months. The retinal hemorrhages and foveal elevation showed sign of resolving. Ocular manifestations following dengue fever is rare. However, bilateral visual loss can occur if both fovea are involved.

Recurrence of multiple myeloma with soft tissue plasmacytoma presenting as unilateral proptosis.

PURPOSE: To describe a rare case of soft tissue plasmacytoma of the orbit presenting with proptosis. RESULTS: A 57 year-old Malay lady presented with right eye proptosis of 3 weeks' duration. It was associated with pain, redness and progressive blurring of vision. The patient was diagnosed to have multiple myeloma a year previously when she was investigated for backache, and had been treated with eight cycles of Pamidronate chemotherapy. On examination visual acuity was 6/60 in the right eye with impaired optic nerve function. Severe proptosis with chemosis and complete ophthalmoplegia were found. The corneal and anterior chamber examinations were normal. Fundoscopy revealed a slightly hyperaemic disc with presence of choroidal folds infero-temporally. The left vision and eye examination findings were normal. Fine needle biopsy and MRI showed recurrence of multiple myeloma with soft tissue plasmacytoma of the extraconal region compressing the right optic nerve. She was managed with orbital radiotherapy, resulting in resolution of proptosis, but without improvement in vision. CONCLUSION: Proptosis may be an indicator of recurrence of multiple myeloma in patients thought to be in remission.