Neurophthalmology

Diplopia, a very common ophthalmic complaint, is a potential first sign of severe pathology. Here, we present a case of an atypical midbrain infarction targeting the lateral subnucleus of the oculomotor nuclear complex that manifested as diplopia with no additional symptoms of a stroke episode. Axial diffusion-weighted and coronal T2-weighted magnetic resonance imaging showed an infarct in the rostral midbrain affecting the subnucleus of the medial rectus located ventrally. Diffusion-weighted imaging was used to diagnose the medial rectus nucleus infarct...

PURPOSE: To summarize the existing treatment options regarding central retinal artery occlusion (CRAO), branch retinal artery occlusion (BRAO), arteritic anterior ischemic optic neuropathy (AAION), non-arteritic anterior ischemic optic neuropathy (NAION), and ocular ischemic syndrome (OIS), proposing an approach to manage and treat these patients. METHODS: A systematic literature search of articles published since 1st January 2010 until 31st December 2020 was conducted using MEDLINE (PubMed), Scopus, and Web of Science...

INTRODUCTION: Eye oscillations, both nystagmic and non-nystagic, can occur from birth. Most correspond to infantile nystagmus that dubates in the first six months of life, which include idiopathic, sensory, latent, or overt latent motor nystagmus. Those associated with neurological diseases or syndromes correspond to approximately 33%, their identification and correct evaluation being important given the potential visual and vital complications. MATERIAL AND METHODS: We made a systematic review of the literature on supranuclear gaze control mechanisms and the main ocular oscillations with possible neurological implications, both in childhood...

Pseudotumor cerebri, benign intracranial hypertension or idiopathic intracranial hypertension are all terms used for a neurological syndrome consisting of increased intracranial pressure, headache and possible edema of the optic nerve head and decreased visual function. Normal findings in contrast agent computed tomography or magnetic resonance imaging of the head in physiological neurological findings, with the exception of paresis VI, indicate an increase in pressure of laboratory normal cerebrospinal fluid (CSF) of unknown cause...

INTRODUCTION: Eye oscillations, both nystagmic and non-nystagmic, can occur from birth. Most correspond to infantile nystagmus that appears in the first six months of life, which include idiopathic, sensory, latent, or overt latent motor nystagmus. Those associated with neurological diseases or syndromes correspond to approximately 33%, their identification and correct evaluation being important given the potential visual and vital complications. MATERIAL AND METHODS: We made a systematic review of the literature on supranuclear gaze control mechanisms and the main ocular oscillations with possible neurological implications, both in childhood...

Patients with Alzheimer's disease (AD) and Parkinson's disease (PD) develop a progressive decline of visual function. This condition aggravates overall cognitive and motor abilities, is a risk factor for developing hallucinations, and can have a significant influence on general quality of life. Visual problems are common complaints of patients with PD and AD in the early stages of the disease, but they also occur during normal aging, making it difficult to differentiate between normal and pathological conditions...

BACKGROUND:  Pupil examination represents a diagnostic and prognostic test in the management of several neurological diseases. Infrared video pupillometry (IVP) is the gold standard, since it is not routinely available, a noninvasive bedside ultrasound assessment has been proposed as an alternative. The aim of this study was to assess the feasibility and reproducibility of ultrasound pupillometry (UP) in comparison with IVP. MATERIALS AND METHODS:  81 subjects (43 men and 38 women, mean age: 52 ± 20 years and 49 ± 19 years, respectively) with no history of neurophthalmologic disease were enrolled...

Objective: To evaluate the clinical characteristics of myelin oligodendrocyte glycoprotein antibody (MOG-IgG) associated myelitis in a cohort of Chinese Han adults. Methods: From January 2016 to December 2017, 70 patients with MOG-IgG associated disorders (MOGAD) and 120 patients with aquaporin 4 antibody (AQP4-IgG) positive neuromyelitis optica spectrum disorders (NMOSD) visited the NMO/MS clinic or the neurology ward of Huashan Hospital, and the neurophthalmology clinic of Eye and ENT hospital, Shanghai Medical College, Fudan University were enrolled...

Papillorenal syndrome (PAPRS; Mendelian Inheritance in Man [MIM] 120330) is an autosomal dominant disease characterised by the presence of congenital renal and optic nerve abnormalities associated with mutations of the PAX2 gene. In this article, the authors present four patients with PAPRS who are carriers of three new PAX2 mutations, as well as another patient with a possible non-pathogenic variant of the PAX2 gene. All patients were given a full neurophthalmological examination, and all patients underwent a genetic test for PAX2 ...

BACKGROUND: Long-term longitudinal studies with multimodal biomarkers are needed to delve into the knowledge of preclinical AD. Subjective cognitive decline has been proposed as a risk factor for the development of cognitive impairment. Thus, including individuals with SCD in observational studies may be a cost-effective strategy to increase the prevalence of preclinical AD in the sample. OBJECTIVES: To describe the rationale, research protocols and baseline characteristics of participants in the Fundació ACE Healthy Brain Initiative (FACEHBI)...

AIM: To study the usefulness of orbital ultrasonography in the diagnosis of papilledema. METHODS: Fifty patients who were referred to the neurophthalmology clinic and clinically suspected to have papilledema were selected. Thorough, clinical examination with slitlamp biomicroscopy and visual acuity assessment was done. These patients underwent ultrasonography to demonstrate the crescent sign. The patients were further evaluated with the neurologist and magnetic resonance imaging (MRI) thus confirming the diagnosis of papilledema...

OBJECTIVE: In February 2009, the authors' center formed a team of neurosurgeons, otolaryngologists, endocrinologists, and radiologists to perform pituitary surgery using the endoscopic endonasal transsphenoidal approach (EETSA). This paper reviews the authors' experience with the technique, pathological outcomes, hormone profiles, and postoperative complications. METHODS: Between February 2009 and December 2015, 535 patients underwent the EETSA with 2-nostrils/4-hands surgery...

OBJECTIVE: In February 2009, the authors' center formed a team of neurosurgeons, otolaryngologists, endocrinologists, and radiologists to perform pituitary surgery using the endoscopic endonasal transsphenoidal approach (EETSA). This paper reviews the authors' experience with the technique, pathological outcomes, hormone profiles, and postoperative complications. METHODS: Between February 2009 and December 2015, 535 patients underwent the EETSA with 2-nostrils/4-hands surgery...

BACKGROUND: To analyze the most common neurophthalmological conditions that may mimic glaucomatous optic neuropathy and to determine which most often lead to misdiagnosis when evaluated by a glaucoma specialist. METHODS: We reviewed the charts of consecutive patients with optic neuropathies caused by neurophthalmological conditions screened in a single Eye Clinic within a period of 24 months. Within these enrolled patients, we selected the eyes whose fundoscopic appearance could resemble glaucoma based in pre-defined criteria (vertical cup-to-disc ratio ≥0...

Giant cell arteritis with ocular involvement is an ocular emergency. Arteritic anterior ischaemic optic neuropathy (AAION) is the most common ophthalmological manifestation associated with this disease. Visual loss is usually permanent with rare cases showing visual recovery. Visual improvement, if it occurs, is generally limited, and the visual field defects are persistent and severe. The main goal of AAION treatment is the preservation of vision in the fellow eye. In patients with neurophthalmological manifestations, high-dose corticosteroids should be initiated immediately and aggressively, and maintained thereafter...

Cerebral visual impairment is a visual function deficit caused by damage to the retrogeniculate visual pathways in the absence of any major ocular disease. It is the main visual deficit in children in the developed world. Preperinatal hypoxic-ischemic damage is the most frequent cause of cerebral visual impairment, but the etiology is variable. The authors set out to evaluate the presence of visual disorders not attributable to any major ocular pathology in a sample of children with central nervous system disease and to describe the clinical picture of cerebral visual impairment in this cohort...

OBJECTIVE: Paraneoplastic neurologic syndrome with otoneurophthalmologic manifestations is much less common than direct, metastatic, and treatment-related complications of cancer. Few studies have focused on patients presenting with paraneoplastic syndrome before a cancer is identified. PATIENT: We describe a case of combined paraneoplastic brainstem/limbic encephalitis and Lambert-Eaton myasthenic syndrome in a patient with small cell lung cancer and positive antiamphiphysin antibodies who initially presented with otoneurophthalmologic signs and symptoms to the ears, nose, and throat clinic...

OBJECTIVE: To determine the causative factors and epidemiology of bilateral vestibulopathy (BV). METHODS: This is a retrospective review of 255 patients (mean age, 62 +/- 16 years) with BV diagnosed in our dizziness unit between 1988 and 2005. All patients had undergone a standardized neurophthalmological and neurootological examination, electronystagmography with caloric irrigation, cranial magnetic resonance imaging or computed tomography (n = 214), and laboratory tests...

Sensory deficits have been documented in Parkinson's disease, in particular within the visual domain. However, ageing factors related to the brain and to neural and non-neural ocular structures could explain some of the previously reported results, in particular the claimed impairment within the koniocellular pathway. This study addressed visual impairment attributable to the magno- (luminance), parvo- (red-green) and koniocellular (blue-yellow) pathways in a population of Parkinson's disease patients. To avoid potentially confounding factors, all subjects underwent a full neurophthalmological assessment which led to exclusion of subjects with increased intraocular pressure, diabetes even in the absence of retinopathy, and ocular abnormalities (from a total of 72 patients' eyes, 12 were excluded)...

Leber congenital amaurosis (LCA) is the most severe form of inherited retinal dystrophy that presents in infancy. LCA is both clinically and genetically heterogeneous. The aim of our study was to clarify the clinical aspects of LCA and to contribute to improved characterization of the disorder. We studied 40 children affected by LCA (mean age at first observation: 19 months, range: 8-50 months), who underwent a comprehensive evaluation that included: neurophthalmological evaluation, electroretinogram (ERG), and visual evoked potentials (VEPs), general and neurological examinations, developmental assessment using scales for visually impaired children, neuroradiological examinations, hepatic and renal function and metabolic investigations, brainstem auditory evoked potentials (BAEPs), EEG, and hand radiographs...