Long-term visual prognosis for patients with suprasellar meningiomas

Objetivo: Determinar o prognostico visual de longo prazo (maior ou igual a dez anos) de pacientes com meningiomas supra-selares. Metodos: Analise retrospectiva de casos Resultados: Vinte pacientes com meningiomas supra-selares foram avaliados e seguidos pelo tempo medio de 16,6 anos. Todos apresentavam queixas visuais no exame inicial. Dois pacientes nao foram tratados e apresentaram piora progressiva da visao em ambos os olhos; 18 foram submetidos a tratamento cirurgico para resseccao da lesao. Houve taxa de mortalidade de 5 por cento, incidencia de morbidade neurologica de 17 por cento, e 44 por cento de morbidade visual. Oito (44 por cento) dos pacientes tratados apresentaram evidencias de recidiva tumoral (tempo medio de 10,7 anos); cinco destes foram submetidos a nova cirurgia. Cinco pacientes tambem foram submetidos a radioterapia convencional, dois deles somente apos evidencias de recorrencia. A acuidade visual final melhorou em 39 por cento dos pacientes, permaneceu estavel em 22 por cento e piorou em 39 por cento. A AV foi melhor ou igual ao exame inicial em 61 por cento dos pacientes tratados, ou em 89 por cento dos pacientes tratados que nao apresentaram recidiva tumoral. No exame final, 72 por cento dos pacientes apresentaram acuidade visual de 20/40 ou melhor em pelo menos um dos olhos. Os outros parametros de avaliacao da funcao visual geralmente acompanharam a evolucao da acuidade visual. Conclusoes: O grupo de pacientes acompanhados clinicamente apresentou resultados visuais de longo prazo bastante inferiores aos do grupo tratado. No grupo de pacientes tratados cirurgicamente os resultados visuais foram melhores quando nao houve complicacoes intra-operatorias e/ou recidiva tumoralBV UNIFESP: Teses e dissertaçõe

The Pupil Light Reflex in Leber's Hereditary Optic Neuropathy: Evidence for Preservation of Melanopsin-Expressing Retinal Ganglion Cells

PURPOSE. To investigate the pupillary light reflex (PLR) of patients with severe loss of vision due to Leber's Hereditary Optic Neuropathy (LHON) in the context of a proposed preservation of melanopsin-expressing retinal ganglion cells (mRGCs).METHODS. Ten LHON patients (7 males; 51.6 +/- 14.1 years), with visual acuities ranging from 20/400 to hand motion perception and severe visual field losses, were tested and compared with 16 healthy subjects (7 males; 42.15 +/- 15.4 years) tested as controls. PLR was measured with an eye tracker and the stimuli were controlled with a Ganzfeld system. Pupil responses were measured monocularly, to 1 second of blue (470 nm) and red (640 nm) flashes with 1, 10, 100, and 250 cd/m(2) luminances. the normalized amplitude of peak of the transient PLR and the amplitude of the sustained PLR at 6 seconds after the flash offset were measured. in addition, optical coherence topography (OCT) scans of the peripapillary retinal nerve fiber layer were obtained.RESULTS. the patient's peak PLR responses were on average 15% smaller than controls (P < 0.05), but 5 out of 10 patients had amplitudes within the range of controls. the patients' sustained PLRs were comparable with controls at lower flash intensities, but on average, 27% smaller to the 250 cd/m(2) blue light, although there was considerable overlap with the PLR amplitudes of control. All patients had severe visual field losses and the retinal nerve fiber layer thickness was reduced to a minimum around the optic disc in 8 of the 10 patients.CONCLUSIONS. the PLR is maintained overall in LHON patients despite the severity of optic atrophy. These results are consistent with previous evidence of selective preservation of mRGCs.Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)International Foundation for Optic Nerve DiseaseUniv São Paulo, Inst Psicol, São Paulo, BrazilUniv São Paulo, Nucleo Neurociencias & Comportamento, São Paulo, BrazilBudapest Univ Technol & Econ, Dept Mech Opt & Eng Informat, Budapest, HungaryIRCCS Ist Sci Neurol Bologna, Bologna, ItalyUniv Bologna, Dept Biomed & Neuromotor Sci DIBINEM, Bologna, ItalyStudio Oculist Azeglio, Bologna, ItalyUniversidade Federal de São Paulo UNIFESP, Escola Paulista Med, Dept Oftalmol, São Paulo, BrazilUniv So Calif, Keck Sch Med, Dept Ophthalmol, Doheny Eye Inst, Los Angeles, CA 90033 USAColumbia Univ, Dept Psychol, New York, NY 10027 USAColumbia Univ, Dept Ophthalmol, New York, NY 10027 USAUniversidade Federal de São Paulo UNIFESP, Escola Paulista Med, Dept Oftalmol, São Paulo, BrazilFAPESP: 08/58731-2Web of Scienc

Natural History of Conversion of Leber's Hereditary Optic Neuropathy. A Prospective Case Series

Purpose: To illustrate the natural history of Leber's hereditary optic neuropathy (LHON). Design: Prospective observational case series. Participants: The Soave-Brazil pedigree of m.11778G>A/ND4 mitochondrial DNA LHON mutation. Methods: A prospectively acquired database of the Soave-Brazil pedigree was reviewed. Data from 285 individuals were included in the database over a 15-year period. The pedigree was reviewed for unaffected mutation carriers who converted to affected status, 6 patients with LHON were identified. The medical records were reviewed 1 year preconversion to 1 year postconversion for visual acuity (logarithm of the minimum angle of resolution [logMAR]), Humphrey Visual Field (HVF) mean deviation (MD), and retinal nerve fiber layer (RNFL) thickness, as measured by Cirrus (Carl Zeiss, Oberkochen, Germany) optic coherence tomography (OCT). The RNFL thickness values were normalized for age. Visual acuity, HVF, and processed RNFL data from each of the 12 eyes were then sorted into 2-month time periods relative to the date of conversion, within which they were averaged. Main Outcome Measures: The main outcome measures were visual acuity, HVF MD, and RNFL thickness. Results: Decreased visual acuity preceded conversion by up to 2 months and then declined up to 8 months postconversion. Decrease in HVF MD occurred at least 4 months preceding conversion, after which values decreased until plateau at 6 to 8 months. Average RNFL thickness was above normal baseline thickness in all 4 quadrants as measured by OCT at the time of conversion. Increase in RNFL thickness preceded conversion as early as 4 to 6 months, peaked at conversion, and decreased until individual plateaus. The temporal quadrant was first to be involved, then the inferior and superior quadrants, and the nasal quadrant showed the latest and least changes. Conclusions: Subclinical changes preceded the date of conversion and may reflect the complicated nature of identifying the date of conversion in LHON. Early increases in RNFL preceding conversion suggest that structural changes precede clinically significant vision loss. Asynchronous quadrant involvement supports a previously published mathematical model. The natural history of LHON is not a subacute process, as previously believed, but progresses more slowly, taking up to 8 months to plateau