Characteristics of beta parapapillary atrophy in primary angle-closure suspect.

OBJECTIVE: To investigate the characteristics of beta parapapillary atrophy (ß-PPA) in patients with primary angle-closure suspect (PACS). METHODS AND ANALYSIS: In total, 215 and 259 eyes with PACS and non-PACS (NPACS), respectively, were enrolled in this observational, cross-sectional study. Stereoscopic fundus and optical coherence tomography images were used to characterise ß-PPA; the former was also used to measure the major ß-PPA parameters. Univariate and multiple logistic regression analyses were used to identify the factors correlated with the presence of ß-PPA and with ß-PPA parameters. RESULTS: The ß-PPA occurrence rates were 48.80% and 44.40% in the PACS and NPACS groups, respectively, with no significant difference between groups. Compared with that in the NPACS group, the ß-PPA area was significantly larger (p=0.005) in the PACS group, but the angular extent and maximum radial length did not differ between groups (p=0.110 and 0.657, respectively) after adjusting for age and axial length. The presence of ß-PPA was associated with older age (OR 1.057, 95% CI 1.028 to 1.088, p<0.001) and larger disc area (OR 1.716, 95% CI 1.170 to 2.517, p=0.006). A larger ß-PPA area was associated with older age (p=0.014), greater vertical cup-to-disc ratio (p=0.028), larger disc area (p<0.001) and PACS diagnosis (p=0.035). CONCLUSION: 48.80% of participants with PACS had ß-PPA, which is slightly larger than NPACS. The area of ß-PPA was larger in PACS, while the angular extent and maximum radial length did not differ between groups.

Bilateral optic atrophy in Wilson disease: A case report and literature review.

Wilson disease (WD) is an autosomal recessive disorder of copper metabolism. The clinical manifestations of WD are complex and variable, with Kayser-Fleischer ring (K-F ring) and the sunflower cataract being the most common ocular findings. Visual impairment is rare in patients with WD. We report the case of a 17-year-old female with bilateral optic atrophy associated with WD and summarize the clinical features of previously reported cases of optic neuropathy in WD, Clinicians should be aware that WD is a rare cause of optic neuropathy and that optic neuropathy in patients with WD may need to be recognized and screened.

Optic Nerve MRI T2-Hyperintensity: A Nonspecific Marker of Optic Nerve Damage.

BACKGROUND: MRI abnormalities are common in optic neuropathies, especially on dedicated orbital imaging. In acute optic neuritis, optic nerve T2-hyperintensity associated with optic nerve contrast enhancement is the typical imaging finding. In chronic optic neuropathies, optic nerve T2-hyperintensity and atrophy are regularly seen. Isolated optic nerve T2-hyperintensity is often erroneously presumed to reflect optic neuritis, frequently prompting unnecessary investigations and neuro-ophthalmology consultations. Our goal was to determine the significance of optic nerve/chiasm T2-hyperintensity and/or atrophy on MRI. METHODS: Retrospective study of consecutive patients who underwent brain/orbital MRI with/without contrast at our institution between July 1, 2019, and June 6, 2022. Patients with optic nerve/chiasm T2-hyperintensity and/or atrophy were included. Medical records were reviewed to determine the etiology of the T2-hyperintensity and/or atrophy. RESULTS: Four hundred seventy-seven patients (698 eyes) were included [mean age 52 years (SD ±18 years); 57% women]. Of the 364 of 698 eyes with optic nerve/chiasm T2-hyperintensity without atrophy, the causes were compressive (104), inflammatory (103), multifactorial (49), glaucoma (21), normal (19), and other (68); of the 219 of 698 eyes with optic nerve/chiasm T2-hyperintensity and atrophy, the causes were compressive (57), multifactorial (40), inflammatory (38), glaucoma (33), normal (7), and other (44); of the 115 of 698 eyes with optic nerve/chiasm atrophy without T2-hyperintensity, the causes were glaucoma (34), multifactorial (21), inflammatory (13), compressive (11), normal (10), and other (26). Thirty-six eyes with optic nerve/chiasm T2-hyperintensity or atrophy did not have evidence of optic neuropathy or retinopathy on ophthalmologic examination, and 17 eyes had clinical evidence of severe retinopathy without primary optic neuropathy. CONCLUSIONS: Optic nerve T2-hyperintensity or atrophy can be found with any cause of optic neuropathy and with severe chronic retinopathy. These MRI findings should not automatically prompt optic neuritis diagnosis, workup, and treatment, and caution is advised regarding their use in the diagnostic criteria for multiple sclerosis. Cases of incidentally found MRI optic nerve T2-hyperintensity and/or atrophy without a known underlying optic neuropathy or severe retinopathy are rare. Such patients should receive an ophthalmologic examination before further investigations.

Síndrome de Charles Bonnet en cuatro casos con atrofia óptica / Charles Bonnet syndrome in four cases with optical atrophy

El síndrome de Charles Bonnet constituye un trastorno muy poco reportado. En Cuba, que se conozca, no hay referencia de casos publicados hasta este momento. Se caracteriza por la presencia de alucinaciones visuales complejas en individuos con pérdida visual, mentalmente sanos y con conciencia crítica de la naturaleza irreal de sus alucinaciones. Se presentan cuatro casos con este síndrome relacionados con déficit visual secundario a atrofia óptica...

Charles Bonnet syndrome is an underreported disorder. As far as we know, there are no publicized references in Cuba up to the present time. This syndrome involves complex visual hallucinations in visually impaired individuals, who are mentally healthy and have intact cognition of the unreal nature of their hallucinations...

Síndrome de Wolfram: relato de três casos / Wolfram's Syndrome: report of three cases

Os autores apresentam três casos de uma associaçäo muito rara de Diabetes mellitus juvenil, Diabetes insipidus, surdez, alteraçöes urinárias e atrofia óptica, conhecida como síndrome de Wolfram. Fazem uma abordagem das outras patologias que apresentam alguns elementos comuns e tentam estabelecer ligaçöes entre elas. Os autores discorrem também sobre herança e procuram, com base em outros casos descritos na literatura, considerar essas síndromes mais próximas do que se imagina.

Manifestaciones oftalmológicas en el SIDA / Ocular manifestations in AIDS

Durante un año fueron estudiados 61 pacientes consecutivos VIH positivos, con el fin de establecer la prevalencia de las manifestaciones oftalmológicas. 29 pacientes estaban en el estadio de SIDA (IV de la clasificación CDC) y 32 eran portadores asintomaticos. En la primera evaluación, se encontro compromiso oftalmológico en 15 de 29 pacientes con SIDA (51.72 por ciento); siendo los hallazgos:microangiopatia retinal no infecciosa 27.6 por ciento, infecciones oportunisticas 20.68 por ciento; Sarcoma de Kaposi 3.44 por ciento y Atrofia optica en 3.44 por ciento. De las infecciones oportunisticas encontradas en la primera visita, la mas importante fue la retinitis por citomegalovirus (CMV) en 10.34 por ciento. En portadores asintomaticos, uno presento microangiopatia retinal no infecciosa con presencia de manchas algodonosas retinales (3.1 por ciento). En el seguimiento de los pacientes con SIDA cuyo examen inicial era normal, 2 desarrollaron microangiopatia y 3 retinitis por CMV; mientras que de los pacientes que al inicio tenian microangiopatia retinal, 5 desarrollaron retinitis por CMV en la evolución. Tambien se observo un nuevo caso de Sarcoma de Kaposi conjuntival y uno de paralisis del VI par craneal y papilitis. Al termino de este estudio habiamos acumulado 9 casos de retinitis por CMV (31 por ciento) y compromiso oftalmológico en 19 de 29 pacientes con SIDA (65.51 por ciento). De los 32 portadores asintomaticos, uno desarrollo retinitis por CMV, debutando como SIDA con una manifestación ocular. Se concluye que elcompromiso oftalmológico en el SIDA es importante, por lo que es necesario un adecuado seguimiento oftalmológico en esta población

Prefuraçäo ocular em crianças / Penetrating ocular injuries in children

Os autores estudaram, prospectivamente, 174 crianças e adolescentes de um total de 473 casos com ferimento perfurante ocular, atendidos no Pronto Socorro de Oftalmologia do Hospital Säo Paulo - Escola Paulista de Medicina, com seguimento de 2 a 36 meses (x=9,5 meses). Observou-se um predomínio do sexo masculino (74,7 por cento), obedecendo uma proporçäo de 3:1. A córnea foi o local-escleral (30,3 por cento). A maioria das perfuraçöes se deveu a acidentes em ambiente doméstico (71,3 por cento), seeguida dos acidentes causados por violência (14,4 por cento), envolvendo objetos pérfuro-contundentes (28,7 por cento), contundentes (228,2 por cento) e pontiagudos (22,5 por cento). É discutida a relaçäo do prognóstico visual com o agente causal e achados pré-operatórios, bem como a alta incidência de atrofia ocular (25,8 por cento)

Atrofia óptica pós-neurite induzida pelo etambutol, isoniazida e drogas correlatas / Optic atrophy induced for ethambutol, isoniazid and others drugs

O autor apresenta um caso de uso de drogas anti-microbianas usada na quimioterapia da tuberculose que levou a uma trofia óptica pós-neurite em ambos os olhos em uma paciente de 58 anos, do sexo feminino, branca, natural de Alegrete, RS

Esclerosis lateral amiotrófica juvenil asociada a compromiso cócleo-vestibular, atrofia óptica, ptosis palpebral y fatigabilidad muscular / Juvenile amyotrophic lateral sclerosis associated with cocleo-vestibular disturbances, optic atrophy, palpebral ptosis and muscle weakness

Se comunica el caso de un paciente joven portador de un síndrome progresivo, caracterizado por compromiso de la motoneurona, de predominio bulbar, asociado a hipoacusia neurosensorial, disfunción vestibular central, atrofia óptica, ptosis palpebral y elementos cerebelosos. Se discute la nosología de la entidad, estableciéndose semejanzas con la parálisis bulbar en asociación con sordera y atrofia óptica, cuya primera descripción, corresponde a Brown a fines del siglo pasado. Este caso tratado con Nicergolina, y lográndose una mejoría aceptable, sugiere para este grupo de enfermos una definición mórbida distinta a la de otras atrofias espinales, postulándose un origen mitocondrial del daño.