[Usher syndrome: about a case]. / Le syndrome d'Usher: à propos d'une observation.

Usher syndrome is a genetic disease resulting in double sensory deprivation (auditory and visual) called deafblindness. We report the case of a 50-year old patient, born to consanguineous parents, presenting with congenital deafness associated with normal vestibular function and pigmentary retinopathy responsible for decreased bilateral visual acuity occurred at the age of 16 years. This association composes Usher syndrome type 2, a rare autosomal recessive disorder. Cataract surgery allowed visual acuity improvement in this patient.

[Role of oral antiviral therapy in the treatment of acute retinal necrosis]. / Intérêt de la thérapie antivirale par voie orale dans le traitement des nécroses rétiniennes aigues.

Acute retinal necrosis syndrome (ARNS) is a rare but devastating uveitic syndrome with devastating visual outcome (visual prognosis ++). It should be diagnosed as early as possible because of its severity and its risk of bilateralization. This is a rare entity caused by the group of herpes viruses. In immunocompromised patients the complications of RNA syndrome often lead to loss of visual acuity. After the discovery of this disease using polymerase chain reaction (PCR) and immune load coefficient (ILC) usually by puncturing the cornea to evacuate the aqueous humour, the confirmation of this diagnosis allows for faster optimization of disease management decreasing the time required to confirm the diagnosis. ARN syndrome is associated with a very poor prognosis. Recent studies have shown that oral antiviral (valaciclovir, famciclovir and valganciclovir) and intravitreal therapies without initial intravenous treatment are an effective treatment for RNA. OBSERVATION: We report the clinical features of a 39-year-old young patient admitted to our emergency department with visual loss. Ophthalmologic examination objectified unilateral acute retinal necrosis. The patient was treated with oral antiviral therapy (valacyclovir) associated with corticosteroids and evolution was very favorable. CONCLUSION: the prognosis of acute retinal necrosis syndrome or RNA is usually severe. The patient should be treated as early as possible in order to limit bilateralization and the occurrence of complications. This study confirms that oral antiviral (valacyclovir, famciclovir and valganciclovir) and intravitreal therapies without initial intravenous treatment are an effective treatment for RNA.