Unilateral papiledema as an atypical presentating sign of idiopathic intracranial hyperthension

Este trabalho descreve o caso de uma paciente de 56 anos que se apresentou com cefaléia, edema unilateral de papila e constrição do campo visual nasal. Os exames laboratoriais e de neuroimagem, o acompanhamento clínico e resposta ao tratamento permitiram o diagnóstico de hipertensão intracraniana idiopática. Chamamos a atenção que o papiledema unilateral pode ser uma forma de apresentação incomum da síndrome e discutimos o seu diagnóstico diferencial e tratamento.We describe a 56-year-old female patient that presented with headache, unilateral optic disc edema and nasal visual field constriction. Laboratory and imaging investigation, clinical findings and response to treatment lead to the diagnosis of idiopathic intracranial hyperthension. We emphasize that unilateral papiledema can be an unusual clinical presentation of the syndrome and discuss its differential diagnosis and treatment

The Photopic Negative Response: An Objective Measure of Retinal Ganglion Cell Function in Patients With Leber's Hereditary Optic Neuropathy

Purpose: The photopic negative response (PhNR) is a slow negative component of a flash photopic full-field ERG that has been shown to be specific for retinal ganglion cell (RGC) activity. Direct evaluation of RGC function is desirable in patients with Leber's hereditary optic neuropathy (LHON) in which the loss of central acuity can make it difficult to monitor patients with standard metrics. The purpose of this study was to evaluate the use of PhNR as an objective noninvasive clinical metric in LHON.Methods: Full-field photopic ERG recordings were collected in subjects with the mt.11778G>A/ND4 LHON mutation using a red on blue stimulus. The PhNR was identified using a computer-based automated detection system, and data were manually examined to remove movement artifacts.Results: The PhNR amplitude was compared between controls (n = 13), carriers (n = 17), and affected (n = 6). Mean PhNR amplitude decreased significantly across groups (P < 0.0001). Post hoc Tukey's test revealed a significant decrease in PhNR amplitude between carriers and controls (P < 0.05) and between carriers and affected (P < 0.01).Conclusions: We are able to demonstrate that the PhNR amplitude is significantly decreased in patients affected by LHON compared to carriers in a well-described pedigree. Surprisingly, there was also a decrease in PhNR in carriers, suggesting potential subclinical RGC dysfunction in some carriers. This is important in patients affected with LHON who typically have a dense central scotoma. The PhNR may be a useful objective outcome measure for future clinical trials