Clinical presentations of X-linked retinoschisis in Taiwanese

X-linked retinoschisis (XLRS, OMIM 312700) is one of the most common causes of juvenile macular degeneration that affects males early in life. The major symptom is early onset of visual loss, and the typical presentations include radiating cystic changes of the macula with or without periph-eral retinoschisis. Some patients may present with vitreous hemorrhage and retinal detachment. In 1898, Hass first described two brothers with typical radiating cystic maculopathy and peripheral choroidal atrophy as “Veränderungen der Retina und Choroidea (English: Changes of the retina and choroid) ” [1]. Since then, the disease has been diagnosed based on ophthalmoscopy and described in the literature under a wide variety of names according to different clinical manifestations and etiologies. In the 1960s, abnormal electroretinograms (ERGs) wa