Leber's idiopathic stellate neuroretinitis: A clinical case

In 1916, Leber's idiopathic stellate neuroretinitis (LISN) was described by Theodore Leber as a rare disease characterized by optic disc swelling associated with a macular star. This fundus appearance can have multiple causes but the etiology of Leber's idiopathic stellate neuroretinitis remains unknown.

A 40 year-old man consulted for a progressive decline in visual acuity and a blurred vision in his left eye. Corrected Visual acuity of the left eye was hand motion, Funduscopy of the left eye revealed a stellate maculopathy with loss of foveolar depression and a normal optic disc. The angiography confirmed an optic disc oedema. Laboratory investigations were normal. No infectious nor inflammatory etiology was found. Brain imaging was normal. Patient received 3 days of intravenous methylprednisolone at 10mg/kg/D for 3 days in a row and an oral relay was started with a progressive degression over 2 weeks. The evolution after treatment was satisfactory, the visual acuity 3 weeks after the intravenous injection of corticoids increased to 2/10.

Leber's idiopathic stellate neuroretinitis (LISN) is a disorder characterized by disc oedema, peripapillary and macular hard exudates and, often, the presence of vitreous cells. The changes in the optic nerve are the primary cause of reduced vision in this condition. The more common treatable causes must be excluded wich are cat scratch disease (CSD) and vascular disease. 50% of cases have no identifiable cause and are labeled idiopathic neuroretinitis. There is no consensus regarding optimal treatment. The prognosis of Leber's idiopathic stellate neuroretinitis is good in most cases.

The cause of neuroretinitis must be aggressively pursued before a diagnosis of lebre's idiopathic neuroretinis can be retained in order to formulate an appropriate treatment strategy.