The Patient's Clinical Profile From a Neuro-Ophthalmology Center in Brazil: A Retrospective Study.

BACKGROUND: Neuro-ophthalmology (NO) is a subspeciality of Ophthalmology, which represents more than an intersection of Neurology and Ophthalmology. The present report highlights the increasing importance of the subspeciality in Brazil and provides a unique retrospective study of the patient's clinical profile of a NO reference center. METHODS: Our study was retrospectively planned aiming to identify all neuro-ophthalmic cases of Instituto de Olhos of the Medical School Faculdade Ciências Médicas de Minas Gerais from August 2013 to March 2022.The first clinical diagnostic impression was selected from a predetermined list of 18 neuro-ophthalmologic conditions. Some NO conditions were eventually reclassified during the follow-up as the final clinical diagnosis impression. The concordance between the first and final clinical impressions was also investigated, as well as the patient's referral source. RESULTS: The sample comprised 903 patients from which 56.4% were female. The mean age was 51.48 ± 20.93 years. Males were more frequent in lower age groups <1 year (n = 3, 100%) and 1-9 years (n = 19/37, 51.4%). An external referral source represented 23.1%, and patients referred after basic ophthalmic consultation and from glaucoma service were 30.3% and 23.2%, respectively. The most encountered first clinical diagnostic impressions were isolated optic atrophy (13.1%), non-neuro-ophthalmic disease (11.7%), optic disc abnormalities (10.4%), ischemic optic neuropathies/retinal vascular occlusions (10.2%), and other visual field defects (9.0%). The kappa concordance coefficient among the first and final clinical diagnostic impressions was 0.53 (95% CI 0.48-0.59), indicating a moderate concordance level. The concordance among the most frequent diagnoses was lower in isolated optic atrophy (33%), other visual field defects (41%), and idiopathic optic neuritis (40%). CONCLUSIONS: Due to the limited number of epidemiology studies in neuro-ophthalmology, we highlight the importance of a NO service in the public health system in Brazil. It may certainly contribute to better strategy plan assistance among professionals and health care managers. This report should seemingly stimulate other studies regarding the relevant and unique features of this subspeciality, which is undoubtedly increasing its importance among patients, and in the scientific community worldwide.

Coexistence of papilledema and pseudopapilledema after remission of idiopathic intracranial hypertension by bariatric surgery / Coexistência de papiledema e pseudopapiledema após remissão de hipertensão intracraniana idiopática por cirurgia bariátrica

ABSTRACT A 37-year-old woman complained of headaches following bilateral visual loss in the past two years. She was obese and had undergone bariatric surgery three months earlier, followed by a considerable weight loss. Neuro-ophthalmic examination revealed a bilateral swollen optic disk. After a computerized analysis of the visual fields and magnetic resonance imaging of the brain and orbits, a diagnosis of idiopathic intracranial hypertension was made. At six months after the bariatric surgery, the patient reported no further headaches and exhibited better findings on computerized analysis of visual fields. However, fundus examination revealed persistent mild papilledema in both eyes. Ocular B-scan ultrasonography showed bilateral optic disk drusen. This report highlights the coexistence of true papilledema and pseudopapilledema due to optic disk drusen, following remission of idiopathic intracranial hypertension after a bariatric surgery.

RESUMO Uma mulher de 37 anos queixou-se de cefaleia após perda visual bilateral nos últimos dois anos. Apresentava história de obesidade e havia sido submetida à cirurgia bariátrica três meses antes, seguida de considerável perda de peso. O exame neuro-oftálmico revelou um disco óptico inchado bilateral. Após uma análise computadorizada dos campos visuais e ressonância magnética do crânio e órbitas, foi feito um diagnóstico de hiper tensão intracraniana idiopática. Após seis meses da cirurgia bariátrica, a paciente não relatou mais cefaleia e foram descobertas melhoras na análise computadorizada dos campos visuais. No entanto, o exame de fundo de olho revelou papiledema leve persistente em ambos os olhos. A ultrassonografia ocular B-scan mostrou drusas do disco óptico bilateralmente. Este relato destaca a coexistência de papiledema verdadeiro e pseudopapiledema devido à drusa de disco óptico após remissão da hipertensão intracraniana idiopática após uma cirurgia bariátrica.

Coexistence of papilledema and pseudopapilledema after remission of idiopathic intracranial hypertension by bariatric surgery.

A 37-year-old woman complained of headaches following bilateral visual loss in the past two years. She was obese and had undergone bariatric surgery three months earlier, followed by a considerable weight loss. Neuro-ophthalmic examination revealed a bilateral swollen optic disk. After a computerized analysis of the visual fields and magnetic resonance imaging of the brain and orbits, a diagnosis of idiopathic intracranial hypertension was made. At six months after the bariatric surgery, the patient reported no further headaches and exhibited better findings on computerized analysis of visual fields. However, fundus examination revealed persistent mild papilledema in both eyes. Ocular B-scan ultrasonography showed bilateral optic disk drusen. This report highlights the coexistence of true papilledema and pseudopapilledema due to optic disk drusen, following remission of idiopathic intracranial hypertension after a bariatric surgery.

The contribution of optical coherence tomography in neurodegenerative diseases.

PURPOSE OF REVIEW: This review investigates the contribution of optical coherence tomography (OCT) to monitoring of neurodegeneration in the anterior visual pathway of patients with neuromyelitis optica, Alzheimer's disease, and Parkinson's disease. Despite a 'normal' regular ophthalmologic examination, some patients present visual complaints, and OCT might better explain the mechanism associated to neuronal and axonal losses that contribute to this clinical condition. RECENT FINDINGS: The eye can be considered a 'window' to the central nervous system that can be directly accessed through OCT. Prior studies have suggested that pathologic processes in the brain are very similar to what happens in the eye in neurodegenerative diseases, and OCT has confirmed these abnormalities regarding the anterior visual pathway. It is supposed that transsynaptic neurodegeneration in lesions of the posterior visual pathway may play a role in the neurodegeneration process of the anterior visual pathway. SUMMARY: Retinal nerve fiber layer, retinal ganglion cells, and inner retinal layers are considered surrogate biomarkers in the progression of neurodegenerative diseases. Because OCT is able to accurately measure neuropathological ocular features, its application has increased both in neuroprotection studies and in treatment. In fact, it may prove to be a unique evaluation tool in comparison with conventional visual tests. VIDEO ABSTRACT: (Supplementary Digital content 1, http://links.lww.com/COOP/A10).

Neuromyelitis optica antibody in Leber hereditary optic neuropathy: case report.

Neuromyelitis optica antibody (or aquaporin-4 antibody) is a well established serum marker associated to high-risk neuromyelitis optica syndrome that presents as an inflammatory demyelinating disease characterized by the occurrence of bilateral and simultaneous optic neuritis without complete visual recovery or it occurs as an isolated episode of transverse myelitis accompanied by longitudinally extensive spinal cord lesions. On the other hand, Leber hereditary optic neuropathy is a primarily hereditary disorder that affects all tissues of the body and its clinical presentation is tissue-specific for the optic nerve and, eventually, it might reach the spinal cord. Overlapping clinical features of neuromyelitis optica and Leber hereditary optic neuropathy may suggest common target organ diseases. The case report described herein emphasizes the coexistence of serum markers of both diseases, and suggests that further investigation of this challenging clinical presentation is warranted to confirm or rule out this association.

Neuromyelitis optica antibody in Leber hereditary optic neuropathy: case report / Anticorpo da neuromielite óptica em neuropatia óptica hereditária de Leber: relato de caso

Neuromyelitis optica antibody (or aquaporin-4 antibody) is a well stablished serum marker associated to high-risk neuromyelitis optica syndrome that presents as an inflammatory demyelinating disease characterized by the occurrence of bilateral and simultaneous optic neuritis without complete visual recovery or it occurs as an isolated episode of transverse myelitis accompanied by longitudinally extensive spinal cord lesions. On the other hand, Leber hereditary optic neuropathy is a primarily hereditary disorder that affects all tissues of the body and its clinical presentation is tissue-specific for the optic nerve and, eventually, it might reach the spinal cord. Overlapping clinical features of neuromyelitis optica and Leber hereditary optic neuropathy may suggest common target organ diseases. The case report described herein emphasizes the coexistence of serum markers of both diseases, and suggests that further investigation of this challenging clinical presentation is warranted to confirm or rule out this association.

Anticorpo da neuromielite óptica (ou anticorpo aquaporina-4) é um marcador sorológico bem estabelecido associado à síndrome de alto risco para neuromielite óptica, doença inflamatória desmielinizante, caracterizada por ocorrência bilateral, simultânea de neurite óptica ou por episódio isolado de mielite transversa com achado de lesões espinais longitudinais extensas. Por outro lado, a neuropatia óptica hereditária de Leber é uma doença primariamente hereditária que afeta todos os tecidos do corpo e sua apresentação clínica envolve o nervo óptico e, eventualmente, a medula espinal. Aspectos clínicos comuns sugerem que neuromielite óptica e neuropatia óptica hereditária de Leber possam atingir os mesmos órgãos. O caso descrito enfatiza a coexistência de marcadores sorológicos das duas doenças e sugere a necessidade de investigação futura desta apresentação clínica atípica para confirmar ou não esta associação.

Knee deep in the nerve.

A 62-year-old man presented with a 3-week history of a progressive right vision loss. His right optic disk showed some mild elevation. Automated perimetry revealed a junctional scotoma. Magnetic resonance imaging of the brain showed enlargement and enhancement of the right optic nerve, chiasm, and proximal optic tract. A chiasmal biopsy revealed a lesion consistent with malignant optic glioma of adulthood.

Ophthalmologic manifestations commonly misdiagnosed as demyelinating events in multiple sclerosis patients.

PURPOSE OF REVIEW: Multiple sclerosis may affect both afferent and efferent visual pathways, and sometimes physicians err on ordinary ophthalmologic diagnosis due to overlapping symptoms between demyelinating and nondemyelinating visual diseases. The present article highlights nondemyelinating ocular occurrences due to physiologic or other pathologic processes that may appear in some patients. RECENT FINDINGS: Optic neuritis is representative of the most common and best-studied demyelinating visual occurrence in multiple sclerosis. However, other nondemyelinating visual disturbances also seen in the general population may be erroneously interpreted as being part of the underlying disease. This comparison has not been documented and may be helpful to overcome such difficulties. SUMMARY: Based on clinical history and some strategies of ophthalmologic examination, physicians can achieve the correct diagnosis. Some clinical situations, however, may be challenging and a multidisciplinary approach in the care of multiple sclerosis is warranted.

The Bell's toll.

A 70-year-old man presented complaining of lid and facial drooping and facial numbness, which progressed over several months. Magnetic resonance imaging of the head showed an enlarged right cavernous sinus. His past medical history was remarkable to squamous cell carcinoma of the face. The patient underwent a craniotomy with biopsy of the cavernous sinus that confirmed malignancy. High index of suspicion in a patient that presents facial drooping and/or numbness should alert ophthalmologists about the occurrence of perineural spread of a previous malignant lesion of the skin.

The Brazilian version of the 25-Item National Eye Institute Visual Function Questionnaire: translation, reliability and validity.

PURPOSE: To develop and investigate the reliability and validity of a Brazilian version of the 25-Item National Eye Institute Visual Function Questionnaire. METHODS: A Brazilian version of the questionnaire was developed and applied to patients with chronic eye diseases and healthy controls. Reliability was assessed by Cronbach's alpha coefficient, intraclass correlation coefficient and interrater reliability coefficient. Participants were submitted to the Medical Outcomes Study Short Form-36 Questionnaire. Validity was estimated by the correlation between the questionnaires and among the 12 subscales of the questionnaire, subscales scores and visual acuity, patients' and controls' subscales scores, and the factor analysis. Responsiveness of the questionnaire was assessed in a group of patients before and following cataract surgery. RESULTS: Cronbach's alpha, intraclass correlation and interrater reliability coefficients were highly significant. Validity was well established in all tests. In patients who underwent cataract surgery, a better vision-related quality of life was observed following surgery. CONCLUSION: The Brazilian version of the 25-Item National Eye Institute Visual Function Questionnaire has valid and reliable psychometric properties and can be applied to vision-related quality of life research.

The Brazilian version of the 25-Item National Eye Institute Visual Function Questionnaire: translation, reliability and validity / Versão brasileira do questionário de função visual de 25 itens do questionário do National Eye Institute: tradução, confiabilidade e validação

PURPOSE: To develop and investigate the reliability and validity of a Brazilian version of the 25-Item National Eye Institute Visual Function Questionnaire. METHODS: A Brazilian version of the questionnaire was developed and applied to patients with chronic eye diseases and healthy controls. Reliability was assessed by Cronbach's alpha coefficient, intraclass correlation coefficient and interrater reliability coefficient. Participants were submitted to the Medical Outcomes Study Short Form-36 Questionnaire. Validity was estimated by the correlation between the questionnaires and among the 12 subscales of the questionnaire, subscales scores and visual acuity, patients' and controls' subscales scores, and the factor analysis. Responsiveness of the questionnaire was assessed in a group of patients before and following cataract surgery. RESULTS: Cronbach's alpha, intraclass correlation and interrater reliability coefficients were highly significant. Validity was well established in all tests. In patients who underwent cataract surgery, a better vision-related quality of life was observed following surgery. CONCLUSION: The Brazilian version of the 25-Item National Eye Institute Visual Function Questionnaire has valid and reliable psychometric properties and can be applied to vision-related quality of life research.

OBJETIVO: Desenvolver e investigar a confiabilidade e a validade da versão brasileira do Questionário de Função Visual de 25 Itens do National Eye Institute. MÉTODOS: A versão brasileira do questionário foi desenvolvida e aplicada em pacientes com doenças oculares crônicas e controles sadios. A confiabilidade foi calculada pelo coeficiente alfa de Cronbach de correlação intraclasse e de confiabilidade entre examinadores. Foi usado o questionário "The Medical Outcomes Study Short Form-36". A validade foi calculada pela correlação entre as escalas dos questionários e entre as 12 subescalas do questionário, a pontuação das subescalas e a acuidade visual, a pontuação das subescalas de pacientes e controles e a análise fatorial. A validação do questionário foi calculada em grupo de pacientes antes e após cirurgia de catarata. RESULTADOS: O coeficiente de alfa de Cronbach, de correlação intraclasse e de variação entre entrevistadores, e os testes de validade da versão brasileira foram significantes. A responsividade foi confirmada pela melhora na qualidade de vida visual no grupo submetido à cirurgia de catarata. CONCLUSÃO: A versão brasileira do Questionário de Função Visual de 25 Itens apresenta propriedades psicométricas válidas e confiáveis e pode ser aplicado em pesquisa de qualidade de vida visual.

Validation of the Brazilian version of Guy's neurological disability scale.

The Guy's neurological disability scale (GNDS) has recently been introduced as a new measure of disability in multiple sclerosis. It is patient-oriented, multidimensional, and not biased towards any particular disability. The purpose of the present study was to validate the Brazilian version of the GNDS. The adaptation of the scale was based on the translation/back-translation methodology. Sixty-two patients with clinically definite multiple sclerosis (CDMS) according to Poser's criteria were recruited for this study. GNDS was administered individually to each subject. The EDSS and the ambulation index (AI) scores were assigned by a neurologist. The intraclass correlation coefficient and the Cronbach's alpha values of the Brazilian version of GNDS (0.94 and 0.83, respectively) were comparable to the original one (0.98 and 0.79, respectively). Furthermore, the factor analysis of the Brazilian version of GNDS suggested, as the original article, a four-factor solution which accounted for 68.8% of the total variance. The Brazilian version of GNDS was found to be clinically relevant as it correlated significantly with the EDSS and AI. In conclusion, the Brazilian version of GNDS can be considered an important tool to evaluate the disability in MS patients, with clinical usefulness and psychometrics soundness.

Validation of the brazilian version of Guy's neurological disability scale

The Guy's neurological disability scale (GNDS) has recently been introduced as a new measure of disability in multiple sclerosis. It is patient-oriented, multidimensional, and not biased towards any particular disability. The purpose of the present study was to validate the Brazilian version of the GNDS. The adaptation of the scale was based on the translation/back-translation methodology. Sixty-two patients with clinically definite multiple sclerosis (CDMS) according to Poser's criteria were recruited for this study. GNDS was administered individually to each subject. The EDSS and the ambulation index (AI) scores were assigned by a neurologist. The intraclass correlation coefficient and the Cronbach's alpha values of the Brazilian version of GNDS (0.94 and 0.83, respectively) were comparable to the original one (0.98 and 0.79, respectively). Furthermore, the factor analysis of the Brazilian version of GNDS suggested, as the original article, a four-factor solution which accounted for 68.8 percent of the total variance. The Brazilian version of GNDS was found to be clinically relevant as it correlated significantly with the EDSS and AI. In conclusion, the Brazilian version of GNDS can be considered an important tool to evaluate the disability in MS patients, with clinical usefulness and psychometrics soundness.

A Guy's neurological disability scale (GNDS) é uma escala de incapacidade criada recentemente, orientada para o paciente, multidimensional, e que não privilegia nenhum tipo específico de incapacidade. O objetivo deste estudo é validar a GNDS para a língua portuguesa. A adaptação da escala foi feita através do método de tradução e re-tradução. Sessenta e dois pacientes com esclerose múltipla (EM) clinicamente definida de acordo com os critérios de Poser participaram deste estudo. A GNDS foi aplicada individualmente em cada paciente. O EDSS e o índice ambulatorial (IA) foram determinados por neurologista. A correlação intra-classe e o valor de Cronbach's alfa da versão brasileira da GNDS (0.94 e 0.83, respectivamente) foram comparáveis aos do artigo original (0.98 e 0.79, respectivamente). Como no artigo original, a análise fatorial da versão brasileira da GNDS sugeriu uma solução de quatro fatores que explicaria 68.8 por cento da variação total. A versão brasileira da GNDS mostrou-se clinicamente relevante uma vez que se correlacionou com o EDSS e o IA. Em conclusão, a versão brasileira da GNDS pode ser considerada como um importante instrumento de avaliação incapacidade na EM com significado clínico e relevância psicométrica.