Neuro-ophthalmological Assessment Research Articles

Late-onset Kjellin syndrome: Diagnosis of SPG11 on fundus examination.

Spastic paraplegia (SPG) is a heterogenous group of neurodegenerative disorders, that may include ocular involvement. Here we report the clinical data of a patient with late-onset Kjellin syndrome, a peculiar form of hereditary SPG with macular dystrophy. Clinical, functional and multimodal retinal imaging data were collected. Genetic testing was performed by Whole Exome Sequencing (WES). A 52-year-old female patient with SPG of unknown origin was referred for a progressive visual acuity loss. Multimodal fundus imaging revealed a peculiar macular dystrophy. Given the specific association of macular dystrophy and SPG, a Kjellin syndrome was suspected and genetic testing performed. WES revealed biallelic pathogenic variants in SPG11, co-segregating with disease in the family. Careful ophthalmological examination prompted the diagnosis and guided molecular testing. This case underlines the importance of a neuro-ophthalmologic assessment in patients with SPG.

Comparison of Eye Tracking and Teller Acuity Cards for Visual Acuity Assessment in Pediatric Cortical/Cerebral Visual Impairment

To compare eye tracking and Teller acuity cards (TAC) for assessment of visual acuity in children with cortical, or cerebral, visual impairment (CVI). Reliability and validity study. We recruited 41 children with CVI from a single academic pediatric neuro-ophthalmology clinic. All children performed eye tracking to measure visual acuity, and 26 children completed TAC assessment by a masked examiner. Additionally, 2 pediatric neuro-ophthalmologists graded visual behavior using the 6-level Visual Behavior Scale (VBS). Eye tracking and TAC were performed at baseline and at 1 month. Test-retest reliability of eye tracking and TAC were assessed using the intraclass correlation coefficient (ICC). Eye tracking and TAC visual acuities were correlated with one another and VBS scores using the Spearman correlation coefficient. Test-retest reliability was excellent for eye tracking measurement of visual acuity (ICC=0.81, P < .0001). For pediatric CVI, TAC test-retest reliability was fair (ICC=0.42, P=.04). There was a moderate correlation between eye tracking and TAC (r=0.43, P=.03) and between TAC and VBS score (r=0.50, P=.009), and a strong correlation between eye tracking grating acuity and VBS score (r=0.72, P < .0001). In our cohort of children with CVI, grating acuity measured by eye tracking demonstrated higher test-retest reliability and stronger correlation with pediatric neuro-ophthalmologic assessment of visual behavior than Teller acuity. Objective determination of gaze direction by an eye tracking camera may be more accurate than human assessment in this population. Future research is needed to determine the optimal methods of longitudinal assessment of visual function and functional vision in children with CVI.

Unilateral Pendular Nystagmus in Multiple Sclerosis: A Case Series.

Acquired pendular nystagmus is most often seen in patients with demyelinating disease. Although it is often bilateral, rare cases may be monocular. There is paucity of data on the spectrum of clinical presentation, underlying mechanism, and response to treatment in patients with monocular pendular nystagmus. Retrospective case series of patients with monocular pendular nystagmus seen in 2 tertiary neuro-ophthalmology clinics between January 2019 and June 2022. All patients underwent a complete neuro-ophthalmological assessment and MRI. We describe 5 patients (3 women) aged 31-49 with monocular pendular nystagmus. All had a diagnosis of multiple sclerosis. Three patients had horizontal and 2 had vertical pendular nystagmus. The Snellen visual acuity in the eye with pendular nystagmus varied from 20/20 to 20/200. Two patients were asymptomatic and 3 suffered visually debilitating oscillopsia. Treatment response was available for 2 patients, both of which responded well to treatment with memantine. The pendular nystagmus was observed in the eye with worse visual acuity in 4 of 5 cases (80%). Three patients had bilateral pontine lesions, and 2 had unilateral pontine lesion ipsilateral to the side of nystagmus. Monocular pendular nystagmus in adults is seen most often in patients with multiple sclerosis. Asymmetry in brainstem lesions and afferent visual input may be the culprit. Treatment with memantine may result in significant improvement in symptomatic patients.

Emergent cerebral venous stenting: A valid treatment option for fulminant idiopathic intracranial hypertension

BackgroundFulminant idiopathic intracranial hypertension (FIIH) is characterized by rapid, severe, progressive vision loss and often treated surgically. Cerebral transverse venous stenting (CTVS) is efficacious in IIH patients, but emergent CTVS in FIIH is rarely reported. We present our experience with emergent CTVS in patients with FIIH. MethodsSince 01/2019, an institutional protocol allowed emergent CTVS in FIIH patients with bilateral transverse sinus stenosis and gradient pressure > 15 on digital subtraction angiography (DSA). We retrospectively analyzed a prospective registry of all IIH patients with details of neurological and neuro-ophthalmological assessments before and after treatment, and subjective assessments of headache and tinnitus were made pre-and post-procedure. Results259 IIH patients, including 49 who underwent CTVS, were registered. Among them, five female patients met inclusion criteria for FIIH and underwent emergent CTVS. FIIH patients were younger (18.8 ± 1.64 vs 27.7 ± 4.85, p < 0.01), mean BMI was lower (30.8 ± 10.57 vs 34.6 ± 4.3, p < 0.01), and lumbar puncture opening pressure higher (454 ± vs 361 ± 99.4, p < 0.01) than that of IIH patients. They presented with acute visual loss, severe headache, papilledema, significant bilateral transverse sinus stenosis on CT-venography, and mean dominant side gradient pressure of 26.4 ± 6.2 on DSA. CTVS was performed without significant complications, resulting in remarkable improvement in headache, optical coherence tomography, and visual fields within 1 week. At 1-year follow-up (four patients) and 6-month follow-up (1 patient), there was complete resolution of papilledema and headache, and marked improvement in visual acuity. ConclusionsIn these patients, emergent-CTVS was a safe and effective treatment option for FIIH. Further evaluation is warranted.

Adult-onset leukodystrophy with vanishing white matter: a case series of 19 patients.

Leukodystrophy with vanishing white matter (LVWM) is an autosomal recessive disease with typical pediatric-onset caused by mutations in one of the five EIF2B genes. Adult-onset (AO) cases are rare. In this observational study, we reviewed clinical and laboratory information of the patients with AO-LVWM assessed at two referral centers in Italy and Portugal from Jan-2007 to Dec-2019. We identified 18 patients (13 females) with AO-LVWM caused by EIF2B5 or EIF2B3 mutations. Age of neurological onset ranged from 16 to 60years, with follow-ups occurring from 2 to 37years. Crucial symptoms were cognitive and motor decline. In three patients, stroke-like events were the first manifestation; in another, bladder dysfunction remained the main complaint across decades. Brain MRI showed white matter (WM) rarefaction in all cases, except two. Diffusion-weighted imaging documented focal hyperintensity in the acute stage of stroke-like events. 1H-spectroscopy primarily showed N-acetyl-aspartate reduction; 18fluorodeoxyglucose-PET revealed predominant frontoparietal hypometabolism; evoked potential studies demonstrated normal-to-reduced amplitudes; neuro-ophthalmological assessment showed neuroretinal thinning, and b-wave reduction on full-field electroretinogram. Interestingly, we found an additional patient with LVWM-compatible phenotype and monoallelic variants in two distinct eIF2B genes, EIF2B1 and EIF2B2. AO-LVWM presents varying clinical manifestations at onset, including stroke-like events. WM rarefaction is the most consistent diagnostic clue even in the latest onset cases. Spectroscopy and electrophysiological features are compatible with axon, rather than myelin, damage. Cerebral glucose metabolic abnormalities and retinal alterations can be present. LVWM might also be caused by a digenic inheritance affecting the eIF2B complex.

Incidence and Pattern of Neuro-Ophthalmological Disorders Presenting to Vitreoretinal Clinics in Bhutan: A 3-Year National Study.

To inform national health policy, we quantified the pattern of neuro-ophthalmological disorders (NODs) presenting to the national vitreoretinal clinics in Bhutan. Retrospective cross-sectional study. We reviewed all new NODs patients over three years. Demographic data, presenting complaints, treatment history, systemic diseases, diagnostic procedures, and diagnoses were quantified. Logistic regression examined the odds of factors linked to more common NODs. Of 226 patients, the majority were males (54.0%), farmers (60.2%), and urbanites (55.8%). Loss of vision was the most common presenting complaint (57.9%), followed by head or orbital trauma (19.5%). The best corrected visual acuity (BCVA) of 216 eyes (47.8%) was ≤6/60. Hypertension was the most common systemic disease (16.4%), followed by diabetes (3.5%), and intracranial space-occupying lesions (3.5%). Neuroimaging (37.6%) was the most common diagnostic test performed, followed by visual field testings (VFTs) (22.9%). With a NOD incidence of 7.8% p.a. (226/2913), optic atrophy (OA) was diagnosed in 134 patients (59.3%). Other common NODs were optic neuritis (15.5%), papilloedema (9.3%), and traumatic optic neuropathy (8.4%). Female gender increased the odds for glaucomatous OA by 2.65× (p = 0.044), and age by 1.09× per year (p < 0.001). Being female increased the odds of optic neuritis by 2.57× (p = 0.03). Over half of the NODs were OA, which could be curable with timely treatment. Improved treatment of glaucoma and non-communicable diseases would reduce the risk of NODs-induced visual loss in Bhutan. The need for improved neuro-ophthalmological assessment and a coordinated multidisciplinary approach to NODs are the highest priorities.

Sellar masses: diagnosis and treatment

Sellar mases can cause a variety of neuro-ophthalmic manifestations, including compressive optic neuropathy, chiasmal syndrome, and ophthalmoplegia due to cranial nerve palsy. Diagnosis involves a thorough history, neuro-ophthalmic examination, and ancillary tests and investigations. Visual field testing is critical in diagnosing and localizing the lesion and determining the extent of visual field loss. Appropriate neuro-imaging is essential in characterizing and localizing the lesion. Neuro-ophthalmologic assessment include meticulous clinical examination and ancillary tests including,visual field testing, which is useful in localizing the lesion, and optical coherence tomography, which is helpful in assessing the degree of axonal and neuronal loss and predicting the visual outcome. Treatment requires a multidisciplinary approach by different specialties, including radiologists, neuro-ophthalmologists, and neurosurgeons. The two primary treatment modalities for these tumors are surgery and radiation therapy. We review the main types of sellar lesions, their neuro-ophthalmologic evaluation, and treatment options.

Small check size pattern reversal visual evoked potentials (VEPs) in patients with chronic idiopathic intracranial hypertension

The condition known as idiopathic intracranial hypertension is characterized by elevated intracranial pressure. The most feared complication of IIH is visual loss due to papilledema and optic nerve atrophy. Visual evoked potentials are sensitive and non-invasive method. They are considered an important objective test in investigating suspected optic nerve disease. This study aims to clarify the effect of chronic increased ICP on the optic nerve. Methods: This case-control study was performed at Beni-Suef University Hospital on 60 female patients divided into two equal groups; Group (I): thirty female patients with chronic idiopathic hypertension and Group (II): thirty age and sex-matched healthy controls. All subjects were subjected to full neuro-ophthalmological assessment by PVEP, a lumbar puncture to measure CSF pressure, and radiological assessment using MRI. SPSS version 20 was used for the statistical analysis. Results: Right Pattern reversed VEP; for the check size 15min, the mean value of P100 latencies of chronic IIH was 109.88±10.46 and for controls was 97.35±4.23. The mean value of P100 amplitudes of chronic IIH was 11.73±5 and for controls was 11.95±3.3. Left pattern reversed VEP; for the check size 15min, the mean value of P100 latencies of chronic IIH was 111.42±11.5 and for controls was 97.13±4.32.

Endoscopic Treatment of Sellar Arachnoid Cysts via a Simple Cyst-Opening Technique: Long-Term Outcomes From a Single Center

Sellar arachnoid cysts (SACs) are rare lesions that require treatment only if symptomatic. The endoscopic endonasal approach has been widely used. Despite their simple cystic appearance and the straight-forward surgical intervention, important associated risks exist, with cerebrospinal fluid (CSF) leak the prevalent risk. A retrospective analysis of patients with pathologically confirmed SAC between January 2006 and December 2019 was conducted. A homogeneous simple cyst-opening technique and skull base reconstruction with nasoseptal flaps was used. A total of 10 patients were identified (7 women and 3 men; median age, 54.5 years; range, 20-77 years). Of the 10 patients, 8 had had newly diagnosed SACs and 2 patients had had recurrence from a previously microsurgically fenestrated SAC. Eight patients had presented with visual symptoms, one with visual symptoms and fatigue, and one with intractable headaches. Neuro-ophthalmological and endocrinological assessments had revealed visual field deficits in 6, visual acuity decline in 5, and hypopituitarism in 2 patients. The median calculated volume was 1.71 mL (range, 0.27-2.54 mL). Postoperatively, no CSF leak and no further surgical complications were noted. The visual field had improved in 4 of 6 patients and visual acuity had improved in 4 of 5 patients. Anterior pituitary function had improved in 1, worsened in 1, and remained stable in 8 patients. One patient had developed diabetes insipidus. One recurrence was recorded at 54 months postoperatively. The results from the present study have shown that SACs can be effectively treated using a simple cyst-opening technique. The routine use of nasoseptal flaps significantly reduced the risk of CSF leakage without compromising nasal quality of life in the long term or mandating additional incisions. Long-term follow-up is important to monitor for late recurrence.

Acute optic neuritis: What do complementary tests add to diagnosis?

IntroductionOptic neuritis (ON) is the inflammation of the optic nerve due in many cases, to a pathological immune response. Since its symptoms can be subtle, diagnosis is sometimes challenging. The value of complementary tests for diagnosis and prognosis of ON was demonstrated in retrospective analysis, but their utility in the acute period of ON has been scarcely studied. The aim of this study is to determine the usefulness of clinical assessment, optical coherence tomography (OCT), visual evoked potentials (VEP) and orbit magnetic resonance imaging (MRI) for making diagnosis and prognosis of acute ON (AON). Materials and methodsA cross-sectional study was conducted including patients with ON within 90 days of symptom onset. A complete neuro-ophthalmological evaluation, OCT, VEP and MRI were carried out, determining in each case its sensitivity, specificity and predictive values in the diagnosis of ON and the assessment of its severity. Results75 eyes of 34 patients with ON were included. Regarding diagnosis, low contrast visual acuity (LCVA) displayed the highest sensitivity (100%), being superior than the sensitivity of all complementary tools, always below 80%. Orbit MRI abnormal findings has a Specificity of 100% to confirm diagnosis. Regarding severity assessment and prognosis, Ganglion cell +inner plexiform layer (GCIP) thickness, but not retinal nerve fibre layer (RNFL), correlates significantly with patients’ visual acuity (VA) (p < 0.05). Furthermore, both P100latency and VEP's amplitude showed to be significantly associated with VA (p < 0.05) in the acute period. The combination of two predictors (measurement of RNFL and GCIP) are capable of explaining 60% of the variation of the patient's visual acuity, with statistical significance (p = 0.02) ConclusionsIn depth neuro-ophthalmological assessment during the acute phase of ON, including contrast sensitivity measurement, proved to be superior to complementary tests for diagnosis, surpassing the performance of OCT and VEP. However, these tools can add to prognosis, as GCIP thickness and VEP's amplitude correlate with disease severity and its findings could encourage prompt aggressive treatments in AON.

Re-evaluating diabetic papillopathy using optical coherence tomography and inner retinal sublayer analysis

Background/ObjectivesTo re-evaluate diabetic papillopathy using optical coherence tomography (OCT) for quantitative analysis of the peripapillary retinal nerve fibre layer (pRNFL), macular ganglion cell layer (mGCL) and inner nuclear layer (mINL) thickness.Subjects/MethodsIn this retrospective observational case series between June 2008 and July 2019 at Moorfields Eye hospital, 24 eyes of 22 patients with diabetes and optic disc swelling with confirmed diagnosis of NAION or diabetic papillopathy by neuro-ophthalmological assessment were included for evaluation of the pRNFL, mGCL and mINL thicknesses after resolution of optic disc swelling.ResultsThe mean age of included patients was 56.5 (standard deviation (SD) ± 14.85) years with a mean follow-up duration of 216 days. Thinning of pRNFL (mean: 66.26, SD ± 31.80 µm) and mGCL (mean volume: 0.27 mm3, SD ± 0.09) were observed in either group during follow-up, the mINL volume showed no thinning with 0.39 ± 0.05 mm3. The mean decrease in visual acuity was 4.13 (SD ± 14.27) ETDRS letters with a strong correlation between mGCL thickness and visual acuity (rho 0.74, p < 0.001).ConclusionAfter resolution of acute optic disc swelling, atrophy of pRNFL and mGCL became apparent in all cases of diabetic papillopathy and diabetic NAION, with preservation of mINL volumes. Analysis of OCT did not provide a clear diagnostic distinction between both entities. We suggest a diagnostic overlay with the degree of pRNFL and mGCL atrophy of prognostic relevance for poor visual acuity independent of the semantics of terminology.

Cerebral visual impairment in CDKL5 deficiency disorder: vision as an outcome measure.

To characterize the neuro-ophthalmological phenotype of cyclin-dependent kinase-like 5 (CDKL5) deficiency disorder (CDD) and assess visual acuity as a reproducible, quantitative outcome measure. We retrospectively analyzed clinical data from patients with CDD. Complete neuro-ophthalmological assessments, including visual acuity, were evaluated. Of 26 patients (22 females, four males; median age 4y, interquartile range 2y 1mo-7y 10mo), cerebral visual impairment (CVI), defined as visual dysfunction in the absence of ocular or anterior visual pathway abnormalities, was diagnosed in all those over 2years of age. Ophthalmological examinations revealed nystagmus in 10 patients and strabismus in 24 patients. Visual acuity was measured in 24 patients, by preferential looking in all and by sweep visual evoked potential in 13. Visual acuities were lower than age expectations and demonstrated improvement in the first 3years. Adjusting for age and sex, average preferential looking visual acuity after 2years of age was higher in patients with intact mobility than in those who were non-mobile. CVI was observed in patients with CDD. Visual acuity improved over time and correlated with mobility. Visual acuity, as a quantifiable measure of visual function, should be considered as an outcome measure in pre-clinical and clinical studies for CDD. What this paper adds Cerebral visual impairment is highly prevalent in cyclin-dependent kinase-like 5 deficiency disorder (CDD). Visual acuity is a measurable quantitative outcome measure in CDD. Visual acuity in CDD correlates with gross motor ability.

Subtemporal, Transapical, and Transcavernous Approach to Clip Low-Lying Basilar Tip Aneurysm: 2-Dimensional Operative Video.

Basilar tip aneurysm clipping is technically challenging because of the depth of operative corridor, rarity in presentation, and important perforators supplying deep, critical structures. Two major approaches to basilar tip aneurysms include (1) a frontotemporal (transorbital) trans-sylvian approach for most aneurysms and (2) a modified subtemporal approach for aneurysms with low-lying necks. A 53-yr-old woman presented to our institution with a large unruptured basilar tip aneurysm notable for a low, broad neck (6.4 mm). After discussion of risks and benefits of endovascular vs surgical options, the patient consented to operative intervention. She underwent a right frontotemporal craniotomy with zygomatic osteotomy, intradural petrous apicectomy, elective sectioning of the fourth cranial nerve (CN IV), and intracavernous removal of the dorsum sellae and posterior clinoid process to provide more space for aneurysm dissection. After temporary clipping of the basilar artery, the perforating arteries were dissected free from the aneurysm and the aneurysm occluded with 2 fenestrated clips. Important technical nuances of the approach include (1) achieving ample working room for temporary occlusion aneurysm dissection, (2) careful dissection of the perforators and contralateral P1, and (3) utilization of 2 fenestrated clips to accommodate and preserve the ipsilateral P1 segment. Postoperative angiogram showed complete aneur-ysmal occlusion. Postoperatively, the patient demonstrated mild cognitive impairment and a right CN IV palsy. At 6-wk follow-up, cognition recovered to normalcy. More recently, at 12-mo follow-up, the patient noted intermittent diplopia. Formal neuro-ophthalmologic assessment confirmed persistence of a CN IV palsy treated with prism lenses but no other neurological deficits.

Meningiomas of the Planum Sphenoidale and Tuberculum Sella.

Patients with meningiomas of the planum sphenoidale and tuberculum sella often present with insidious vision loss in one or both eyes as the only sign or symptom of their disease, although other sensory, oculomotor, and even endocrine abnormalities may be seen in a minority of cases. Incidentally discovered tumors also are common, as patients may undergo neuroimaging for unrelated symptoms or events. Depending on the size and orientation of the tumor, central vision loss from optic nerve compression may be a later sign, and loss of peripheral vision in one or both eyes may not be recognized until it has progressed to areas closer to fixation. A thorough neuroophthalmologic assessment including visual field testing will help to define the extent of optic pathway involvement. Both fundus examination and optical coherence tomography of the retinal nerve fiber layer and macular ganglion cell complex will aid in determining prognosis after treatment of the tumor. Orbital surgery rarely is indicated as primary therapy for meningiomas in this location, and surgical resection or debulking is usually pursued before consideration is given to radiation therapy. Because of the long-term risk of residual tumor growth or recurrence, neuroophthalmic surveillance along with serial neuroimaging is required for years after tumor resection and/or radiation therapy.

Recent advances and future directions on the use of optical coherence tomography in neuro-ophthalmology.

Optical coherence tomography (OCT) is a noninvasive imaging technique used to qualitatively and quantitatively analyze various layers of the retina. OCT of the retinal nerve fiber layer (RNFL) and ganglion cell–inner plexiform layer (GCIPL) is particularly useful in neuro-ophthalmology for the evaluation of patients with optic neuropathies and retrochiasmal visual pathway disorders. OCT allows for an objective quantification of edema and atrophy of the RNFL and GCIPL, which may be evident before obvious clinical signs and visual dysfunction develop. Enhanced depth imaging OCT allows for visualization of deep structures of the optic nerve and has emerged as the gold standard for the detection of optic disc drusen. In the evaluation of compressive optic neuropathies, OCT RNFL and GCIPL thicknesses have been established as the most important visual prognostic factor. There is increasing evidence that inclusion of OCT as part of the diagnostic criteria for multiple sclerosis (MS) increases its sensitivity. Moreover, OCT of the RNFL and GCIPL may be helpful in the early detection and monitoring the treatment of conditions such as MS and Alzheimer's disease. OCT is an important aspect of the neuro-ophthalmologic assessment and its use is likely to increase moving forward.

Protocolo diagnóstico del incidentaloma hipofisario

Pituitary incidentalomas are a previously unsuspected pituitary lesions that are discovered on an imaging study performed for unrelated pituitary symptoms. They are quite prevalent, 15% in autopsy series and 22.5% in imaging tests data. Proper diagnosis requires adequate imaging study (gadolinium-enhanced magnetic resonance imaging is the imaging test of choice) as well as complete hormonal assessment. Occasionally, neuro-ophthalmological assessment is necessary. Hormonal study in all patients should be aimed at ruling out pituitary hyper- and hypofunction. Since prolactinomas are the only pituitary adenomas whose first-line treatment is medical with dopaminergic agonists, prolactin is probably the most important determination. Cushing syndrome screening is not necessary in patients without clinical suspicion of hypercortisolism.

Ultrasound Examination of the Pupil - A New Tool for the Neuro-Ophthalmological Assessment.

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. 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. UP was performed with a 12-MHz linear probe according to current guidelines for orbital insonation. Light and painful stimuli were applied to test pupillary light reflex (PLR) and ciliospinal reflex (CR). In 30 of these subjects IVP examination was performed additionally to obtain intra-observer and inter-observer agreement. Increasing age was associated with a decreased pupillary diameter (PD) at rest, after PLR and CR (R -0.728, p < 0.01, R -0.643, p < 0.01, R 0.674, p < 0.001 respectively), while no association was noticed with time to constriction/dilation. UP measurements were reproducible (rate of inter- and intra-observer agreement: R 0.979, p < 0.01, R 0.946, p < 0.01 respectively) and concordant with IVP (PLR R 0.831, p < 0.01; CR R 0.879, p < 0.01). According to our study, ultrasound pupillometry is a feasible and reliable technique for bedside pupillary function assessment, and is a good alternative to infrared video pupillometry. Moreover, it represents the only way for functional pupillary assessment in patients with periorbital hematoma.

Optic Nerve Angle in Idiopathic Intracranial Hypertension.

The tortuosity of the optic nerve can be quantified radiologically by measuring the angle of optic nerve deformation (the "optic nerve angle" [ONA]). In patients with idiopathic intracranial hypertension (IIH), lowering the intracranial pressure (ICP) to a normal range by lumbar puncture leads to straightening of the optic nerve and an increase in the measured sagittal ONA on MRI. It is uncertain whether there is any correlation between ONA and cerebrospinal fluid (CSF) opening pressure or visual function. Retrospective study of patients with and without IIH who had MRI of the brain followed by lumbar puncture with CSF opening pressure within 24 hours of MRI. Before LP and within 24 hours of MRI of the brain, all patients with IIH had neuro-ophthalmologic assessment including visual acuity, Humphrey Visual Field (HVF), and fundus photography. Sagittal ONA was measured on multiplanar T2-SPACE images on a DICOM viewer. Papilledema on the fundus photographs was graded using the Frisén scale. Fifty-four patients with IIH and 30 unmatched controls were included. The IIH group was 6.3 years younger (95% CI 2.4-10.3, P = 0.002), had 8.7 kg/m2 higher body mass index (4.9-12.5, P < 0.001), and 26.3% more women (P = 0.011) compared with controls. In both eyes, the ONA was significantly smaller in patients with IIH by 12° compared with controls (7°-17°, P < 0.00001). In the IIH group, no correlation between ONA and the CSF opening pressure was present in either eye (right eye r = 0.19, P = 0.15; left eye r = 0.18, P = 0.19) The ONA did not correlate with logarithm of the minimum angle of resolution visual acuity (right eye r = 0.26, P = 0.063; left eye r = 0.15, P = 0.27), HVF mean deviation (right eye r = 0.0059, P = 0.97; left eye r = -0.069, P = 0.63), or Frisén grade (Spearman's rho right eye 0.058, P = 0.67; left eye 0.14, P = 0.30). The ONA is significantly smaller in patients with IIH compared to controls, but does not correlate with CSF opening pressure, severity of papilledema, or visual function. The ONA may be useful in identifying patients with raised ICP, but not necessarily those with a poor visual prognosis.

Visual impairment in aging and cognitive decline: experience in a Memory Clinic

Visual impairment is common in people living with dementia and regular ophthalmological exams may improve their quality of life. We evaluated visual function in a cohort of elderly individuals and analyzed its association with their degree of cognitive impairment. Participants underwent neurological and neuropsychological exams, neuro-ophthalmological assessment (visual acuity, intraocular pressure, rates of past ophthalmological pathologies, use of ocular correction, treatments and surgeries) and optical coherence tomography (OCT) scan. We analyzed differences in ophthalmological characteristics among diagnostic groups. The final sample of 1746 study participants aged ≥ 50 comprised 229 individuals with Subjective Cognitive Decline (SCD), 695 with mild cognitive impairment (MCI) and 833 with Dementia (Alzheimer disease: n = 660; vascular dementia: n = 92, Lewy body dementia: n = 34; frontotemporal dementia: n = 19 and other: n = 28). Age, gender and education were used as covariates. Patients with Dementia, compared to those with SCD and MCI, presented worse visual acuity (p < 0.001), used less visual correction (p = 0.02 and p < 0.001, respectively) and fewer ophthalmological treatments (p = 0.004 and p < 0.001, respectively) and underwent fewer ocular surgeries (p = 0.009 and p < 0.001, respectively). OCT image quality worsened in parallel to cognitive decline (Dementia vs SCD: p = 0.008; Dementia vs MCI: p < 0.001). No group differences in past ophthalmological disorders or abnormal OCT findings were detected. Efforts should be made to ensure dementia patients undergo regular ophthalmological assessments to correct their visual function in order to improve their quality of life.

Neuro-ophthalmological biomarkers of visual outcome in newly diagnosed idiopathic intracranial hypertension

BackgroundIdiopathic intracranial hypertension (IIH) is a disorder of unidentified etiology characterized by raised intracranial pressure (ICP) without clinical, laboratory, or radiological evidence of intracranial pathology. The aim of this work was to determine the visual outcome in newly diagnosed IIH patients.MethodsThe study included 68 IIH patients; 59 responded to medical treatment and nine needed lumboperitoneal shunting (LPS). Patients were submitted to papilledema grading using Frisén Scale, water CSF manometry, brain MRI/MRV, mean deviation of visual field examination (MD-VFE), optic nerve sheath diameter (ONSD), average optic disc optical coherence tomography–retinal nerve fiber layer (OCT–RNFL) thickness, and pattern–reversal visual evoked potential (VEP).ResultsPatients needed LPS showed statistically significant increase in baseline papilledema grade, MD-VFE, ONSD, average OCT–RNFL thickness, and P100 VEP latency. On the other hand, both studied groups showed statistically non-significant differences regarding the patients’ ages and opening CSF pressure.ConclusionNewly diagnosed IIH patients’ evaluation must be based on multimodality neuro-ophthalmological assessment where papilledema grade, MD-VFE, and OCT–RNFL are valuable biomarkers of PVD while P100 VEP latency delay is a predictor of poor visual outcome and ONSD is an early indicator of elevated ICP regression after LPS surgery.