Background: Macular star results from deposits of hard exsudate in Henle’s fiber layer radiating out in a starlike pattern in patients with infectious optic neuritis or neuroretinitis. More rarely it is found in other conditions such as retinal occlusive vascular disease, anterior ischemic optic neuropathy and hypertensive retinopathy. It has been well established that its finding rules out multiple sclerosis. Neuromyelitis optica (NMO) is an autoimmune disease in which aquaporin 4 (AQP-4) is the target antigen. AQP-4 is upregulated in optic nerves and inner retinal vessels. Objective: To report the finding of macular star in NMO. Case report: An 18-year-old white female with a long history of anemia, thromobocytopenia, and livedo reticularis developed tetraparesis and sphincter disturbance in December 2008. Spinal MRI showed a T2- lesion extending from middle cervical to middle thoracic levels. Brain MRI disclosed moderate brain atrophy. Cerebrospinal fluid analysis was unrevealing. She was given IV methylprednisolone with full recovery. New attacks of myelitis three and six months later showed poor response to IV methylprednisolone and cyclophosphamide. In June 2009 she complained of intermittent diplopia and in January 2010 bilateral blurring of vision. Best corrected visual acuity was 20/30 OD and 20/60 OS. Ophthalmoscopy revealed bilateral optic nerve edema with macular star. Fundi fluorescein angiography disclosed optic nerve edema with leakage but no abnormality. Optical coherence tomography revealed striking irregular thickening of the retinal nerve fiber layer. A comprehensive laboratory work-up for infectious diseases and autoantibodies was negative.Initially the patient was NMO-IgG seronegative but a second test two months later yielded a positive result. Conclusion: Inflammatory cell infiltration into the vessel walls of the optic nerve and retina has been pathologically documented in NMO patients. These changes might have played a role in the pathogenesis of the macular star in the present patient. Surprisingly however, macular star had not been previously reported in NMO patients. As it usually takes two or more weeks to develop the initial examination may fail to reveal any macular abnormality. A serial and careful ophthalmoscopic examination may prove worthy to better define the frequency and meaning of macular star in NMO patients.
Read More: Macular star: a new finding in neuromyelitis optica