Risk Factors for Fellow Eye Involvement in Nonarteritic Anterior Ischemic Optic Neuropathy Background: Nonarteritic anterior ischemic optic neuropathy (NAION) is the most common acute optic neuropathy in individuals older than 50 years. Demographic, ocular, and systemic risk factors for NAION have been identified, and we sought to determine which, if any, of these factors also increase risk of NAION in the fellow eye. Methods: We performed a retrospective chart review of patients with “ischemic optic neuropathy” (based on International Classification of Disease [ICD] codes) seen at a single eye center between 2007 and 2017. Patients who met diagnostic criteria for unilateral NAION without fellow eye optic neuropathy at diagnosis were included. Demographic information, ocular comorbidities, and systemic diagnoses were recorded, in addition to whether the fellow eye developed NAION during the follow-up period. Univariate and multivariate Cox proportional hazard regression were used to calculate hazard ratios (HRs) for fellow eye involvement. Results: Three hundred eighteen patients were identified by ICD codes, and 119 were included in the study. Twenty-nine (24%) patients developed NAION in the fellow eye over the mean follow-up period of 3.6 years (range: 1 month–11 years). Significant risk factors for fellow eye NAION included the presence of bilateral optic disc drusen (ODD, HR 2.78, 95% confidence interval [CI] 1.12–6.90, P = 0.02) and noncompliance with continuous positive airway pressure (CPAP) in patients with moderate-to-severe obstructive sleep apnea (HR 4.50, 95% CI 1.79–11.3, P = 0.0015). Conclusions: Bilateral ODD and noncompliance with CPAP when indicated are associated with increased risk of NAION in the fellow eye. Patients with these risk factors should be counseled on the potentially devastating visual consequences of bilateral NAION, and compliance with CPAP should be stressed when appropriate. |
Initial Impairment and Recovery of Vision-Related Functioning in Participants With Acute Optic Neuritis From the RENEW Trial of Opicinumab Background: Leucine-rich repeat and immunoglobulin domain-containing Nogo receptor-interacting protein 1 (LINGO-1) is a key suppressor of oligodendrocyte differentiation and axonal remyelination and regeneration. This analysis evaluated the potential benefit of opicinumab, a human monoclonal antibody against LINGO-1, vs placebo on exploratory clinical endpoints of patient-reported vision-related functioning and high-contrast visual acuity (HCVA) in RENEW participants with acute optic neuritis (AON). Methods: Participants were randomized to 100 mg/kg opicinumab intravenous or placebo every 4 weeks (6 infusions). Assessments were conducted in the per-protocol (PP) population and included: 25-item National Eye Institute Visual Functioning Questionnaire (NEI-VFQ-25), 10-item Neuro-Ophthalmic Supplement (NOS-10), and HCVA. Results: The opicinumab group (n = 33) had worse mean (SD) baseline patient-reported vision-related functioning scores vs placebo (n = 36): NEI-VFQ-25 composite, 75.5 (17.6) vs 79.0 (16.6); NOS-10 composite, 63.6 (19.8) vs 69.8 (21.2), respectively. By Week 24, the placebo and opicinumab groups experienced substantial mean improvements from baseline (NEI-VFQ-25 composite, 15.17 vs 13.51 [difference (95% CI): −1.66 (−5.11 to 1.78)]; NOS-10 composite, 17.40 vs 16.04 [difference (95% CI): −1.35 (−7.38 to 4.67)]). Between-treatment differences in mean change from baseline were not significantly different at any time point. Analysis of covariance–adjusted mean recovery from baseline in HCVA at Week 24 for the affected eyes was 11.8 and 8.7 letters for placebo and opicinumab, respectively (P = 0.202). Conclusions: Most participants in the RENEW PP population demonstrated substantial recovery from baseline in patient-reported vision-related functioning and HCVA, regardless of treatment and structural damage. Average scores after recovery remained lower than those of published disease-free control groups. These results provide important information on visual function recovery in patients with AON, as measured by NEI-VFQ-25 and NOS-10. |
Identifying Incidence of and Risk Factors for Fluoroscopy-Guided Lumbar Puncture and Subsequent Persistent Low-Pressure Syndrome in Patients With Idiopathic Intracranial Hypertension Background: To explore the incidence of and potential risk factors for developing persistent low-pressure syndrome after lumbar puncture (LP) in patients with idiopathic intracranial hypertension (IIH), as measured by use of blood patches. Methods: A retrospective chart review was conducted of patients with definitively diagnosed IIH by clinical examination and LP, comparing them to patients with multiple sclerosis (MS) as controls who also received diagnostic LPs. Demographic, clinical, and radiological data were collected for each patient. The main outcome measure was the rate of post-LP blood patches in IIH patients compared with MS patients. Secondary outcome measures were the likelihood of undergoing an epidural blood patch related to age, body mass index, volume removed, opening pressure, the difference between opening and closing pressure, and the level of puncture within the IIH cohort. Results: One hundred four IIH patients and 149 MS patients were included in the study. Among IIH patients, 12/104 (11.5%) underwent an epidural blood patch after LP as compared to 8/149 (5.4%) of the MS control patients (P = 0.086). Within the IIH population, none of the clinical or LP parameters were significantly correlated with increased risk of needing a blood patch. Conclusions: The incidence of low-pressure syndrome, as measured by blood patches, is similar in IIH patients and MS controls. This suggests that having elevated intracranial pressure before an LP is not protective against developing postpuncture low-pressure syndrome, contrary to common assumptions. |
Aquaporin-4 Serostatus and Visual Outcomes in Clinically Isolated Acute Optic Neuritis Background: Aquaporin-4 antibodies (AQP4-Ab) are associated with neuromyelitis optica spectrum disorder (NMOSD) and typically this disorder has a poor visual prognosis as a result of optic neuritis (ON). Our aim was to report the clinical features at onset and final visual outcomes at 6 months of patients with ON who were positive for AQP4-Ab vs. those who were negative for AQP4-Ab. Methods: Retrospective cohort study. AQP4-Ab were tested by indirect immunofluorescence in 57 patients with a first episode of ON. All patients initially were referred for consideration of multiple sclerosis ON (MSON), NMOSD, or any other inflammatory central nervous system disorder during follow-up (41.31 ± 24.32 months). Our patients were diagnosed as having NMOSD, MSON, chronic relapsing inflammatory ON, and single isolated ON. Risk factors associated with visual outcomes of ON patients were assessed through an ordinal regression model. Results: Positive AQP4-Ab were associated with male sex (P = 0.02), earlier age of onset (P = 0.01), and myelitis relapses (P = 0.04). Seronegative group had fewer recurrences of ON than the seropositive group (35% vs 58%, P = 0.14). Patients that were positive for AQP4-Ab did not have worse visual acuity at baseline and after 6 months. However, poor visual acuity during first attack was associated with a worse visual acuity at 6 months (odds ratio = 2.28, 95% CI [1.58–3.28], P = 0.03). Conclusions: At 6 months, positive AQP4-Ab vs negative AQP4-Ab patients no evidence of poorer visual acuity. Lower visual acuity at baseline was associated with poor visual recovery at 6 months. |
Radiation-Induced Optic Neuropathy: Clinical and Imaging Profile of Twelve Patients Background: Radiation-induced optic neuropathy (RON) is a form of delayed radionecrosis of the anterior visual pathways, which develops within months to years after external cranial irradiation and causes severe and irreversible vision loss. Small series reports have adequately documented its clinical features, but imaging characteristics have been less completely described. Methods: We accrued cases from the University of Michigan Neuro-Ophthalmology Clinic files and from cases coded as “radiation optic neuropathy” at the University of Michigan Medical Center between 1994 and 2017. All patients had undergone 3D-conformal linear accelerator (photon) external beam radiation. We collected clinical details of vision loss, including the temporal relationship to radiation. A single neuroradiologist (E.A.L.) evaluated all available magnetic resonance imaging (MRI) studies, noting the presence of enhancement, expansion, or volume loss of the optic nerves or chiasm, corresponding T2 signal abnormalities, and the absence of demyelination or confounding compressive lesions. Results: Twelve patients (15 eyes) met inclusion criteria. Vision loss was usually monocular at outset, but both optic nerves were eventually involved in 3 (25%) patients. Although usually sudden in onset, vision loss often declined slowly over many months, frequently to finger counting, or worse without recovery. An afferent pupillary defect was always present at the time of presentation. Most affected optic discs were pale at the time of first visual symptoms, indicating that subclinical optic nerve damage had been present for several weeks. The latency from completion of radiation to onset of vision loss ranged from 7 to 48 months (average: 18 months). In 2 patients, radiation was delivered to the whole brain, rather than being limited to the anterior visual pathway. MRI typically displayed a discrete region of enhancement of the affected prechiasmatic optic nerve, often with expansion and high T2 signal in the enhancing segment. In 3 affected eyes, enhancement was apparent on imaging completed 3–6 weeks before the onset of vision loss. In one patient, segmental prechiasmatic enhancement became evident only on repeat MRI completed 7 months after vision loss. The duration of enhancement among 9 eyes with follow-up MRIs was at least 2 months, but in one case, enhancement was still present on a study performed 17 months after treatment. Conclusions: This study further delineates the profile of RON. Visual loss is often acute, profound, and monocular but may decline slowly after acute onset and later affect both optic nerves. High-resolution MRI of the optic nerves usually will display enhancement of a discrete segment of the intracranial prechiasmatic optic nerve, often with accompanying expansion and T2 hyperintensity. In some cases, these imaging features may precede vision loss. They may be subtle or appear after vision loss. Enhancement lingers for a wide interval, ranging in this study from 2 to at least 17 months. Recognition of these imaging characteristics assists in confirmation of the diagnosis of RON. |
Visual and Positional Modulation of Pendular Seesaw Nystagmus: Implications for the Mechanism Background: The mechanisms of pendular seesaw nystagmus (SSN) remain unknown. Methods: We evaluated modulation of pendular SSN by removal of visual fixation, convergence, and positional changes in 2 patients, one with bitemporal hemianopia due to a traumatic damage of the optic chiasm and the other with platybasia compressing the medulla and lower cerebellum. Results: In both patients, the pendular SSN markedly decreased or disappeared with convergence, without visual fixation in darkness, during static head tilt toward each shoulder while sitting and while supine. Conclusions: The similar patterns of nystagmus modulation observed in our patients with a different etiology indicate a common role of both visual and otolithic inputs in generating pendular SSN. |
Visual Field Mean Deviation at Diagnosis of Idiopathic Intracranial Hypertension Predicts Visual Outcome Background: A robust predictor of visual outcome in idiopathic intracranial hypertension (IIH) would be useful in management, but there is limited information on this point. The purpose of this study was to ascertain whether visual field mean deviation on standard static perimetry performed at diagnosis in a large patient cohort is a reliable predictor of visual outcome. Methods: We retrospectively reviewed the automated visual field mean deviations at diagnosis and at final encounter in 79 patients with IIH examined in the neuro-ophthalmology clinics at a single academic medical center from 1999 to 2015. Results: Of the 79 study patients, 66 (84%) entered with visual field mean deviations of −7 dB or better. Of those 66 patients, 59 (89%) had final mean deviations of −4 dB or better and 33 (56%) had final mean deviations of −2 dB or better. The single patient who had an initial mean deviation of −7 dB or better and a poor final mean deviation (−32 dB) was nonadherent to prescribed medication. Of the 13 (21%) patients who entered with mean deviations worse than −7 dB, 11 (85%) ended up with poor visual outcomes, their final mean deviations ranging from −5 dB to −32 dB. Over half of those 13 patients had required surgery for IIH, often within 3 weeks of diagnosis, owing to severe papilledema and visual dysfunction at the time of diagnosis. Conclusions: Based on this retrospective study, patients with IIH who have relatively mild visual dysfunction at diagnosis are likely to have a favorable visual outcome, provided they are adherent to recommended treatment. Many of those with poor visual function at diagnosis will have unfavorable visual outcomes despite aggressive treatment. |
Effects of Varying Intranasal Treatment Regimens in ST266-Mediated Retinal Ganglion Cell Neuroprotection Introduction: Previous studies have shown that intranasally administered ST266, a novel biological secretome of amnion‐derived multipotent progenitor cells containing multiple growth factors and anti-inflammatory cytokines, attenuated visual dysfunction and prevented retinal ganglion cell (RGC) loss in experimental optic neuritis. Long-term effects and dose escalation studies examined here have not been reported previously. Methods: Optic neuritis was induced in the multiple sclerosis model experimental autoimmune encephalomyelitis (EAE). EAE and control mice were treated once or twice daily with intranasal placebo/vehicle or ST266 beginning after onset of optic neuritis for either 15 days or continuously until sacrifice. Visual function was assessed by optokinetic responses (OKRs). RGC survival and optic nerve inflammation and demyelination were measured. Results: Both once and twice daily continuous intranasal ST266 treatment from disease onset to 56 days after EAE induction significantly increased OKR scores, decreased RGC loss, and reduced optic nerve inflammation and demyelination compared with placebo (saline, nonspecific protein solution, or cell culture media)-treated EAE mice. ST266 treatment given for just 15 days after disease onset, then discontinued, only delayed OKR decreases, and had limited effects on RGC survival and optic nerve inflammation 56 days after disease induction. Conclusions: ST266 is a potential neuroprotective therapy to prevent RGC damage, and intranasal delivery warrants further study as a novel mechanism to deliver protein therapies for optic neuropathies. Results suggest that once daily ST266 treatment is sufficient to sustain maximal benefits and demonstrate that neuroprotective effects promoted by ST266 are specific to the combination of factors present in this complex biologic therapy. |
Neuro-Ophthalmic Symptoms of Primary Headache Disorders: Why the Patient With Headache May Present to Neuro-Ophthalmology Background: Primary headache disorders can cause many ophthalmic symptoms that lead many patients to present for neuro-ophthalmic evaluation. Neuro-ophthalmologists frequently encounter these patients in clinical practice. Evidence Acquisition: A literature review was completed in PubMed using the following terms paired with “migraine” and “headache:” dry eye, eye pain, monocular diplopia, binocular diplopia, photophobia, visual field defect, tunnel vision, floaters, amaurosis fugax, transient visual obscuration, autonomic symptoms, anisocoria, visual snow, Alice in Wonderland syndrome, and palinopsia. Results: Patients with migraine experience a wide range of visual disturbances including aura and more complex perceptual abnormalities such as Alice in Wonderland syndrome and visual snow. Visual disturbances may consist of positive and/or negative phenomena and may be binocular or monocular. Migraine and other primary headache disorders can be associated with photophobia, eye pain, dry eye, autonomic features, and anisocoria. Conclusions: Patients with primary headache disorders may experience a wide range of visual and ophthalmic symptoms. An understanding of the typical features of these disorders allows providers to help patients find appropriate treatment without unnecessary testing and to recognize when atypical presentations require additional evaluation. |
Vestibular Migraine: How to Sort it Out and What to Do About it Background: Vestibular migraine (VM) is the most common neurologic cause of vertigo in adults and results in significant utilization of health care resources, but remains under-recognized and underdiagnosed. Evidence Acquisition: Review of literature in PubMed using the following terms: vestibular migraine, migraine-associated vertigo, vertiginous migraine, benign recurrent vertigo, migraine-associated dizziness, migraine, migraine treatment, Meniere disease (MD), vertebrobasilar ischemia (VBI), posterior circulation stroke, benign paroxysmal positional vertigo, and episodic-ataxia Type 2 (EA2). Results: VM can manifest with a variety of vestibular symptoms, including spontaneous vertigo, triggered vertigo, positional vertigo, and head-motion dizziness. Patients may report more than 1 vestibular symptom. Episodes of vertigo are often, but not always, accompanied by headache. Auditory symptoms are frequently associated with VM attacks and may mimic the manifestations of MD. Other migrainous features that accompany VM attacks include photophobia, phonophobia, osmophobia, and visual aura. Interictally, patients may suffer from persistent dizziness or isolated paroxysmal vestibular symptoms. Mood disorders (particularly anxiety) are often found in VM. Abnormal neuro-otologic findings are not uncommon in patients with VM. Differential diagnoses for VM include MD, VBI, EA2, and migraine with brainstem aura. For rescue treatment, triptans, vestibular suppressants, and/or antiemetic agents may be considered. Pharmacologic migraine preventives (antiepileptics, beta-blockers, and antidepressants) are often useful. Conclusions: The keys to correctly diagnosing VM is identifying a relationship between vestibular symptoms and migrainous features and being aware of the heterogeneity of manifestations of this enigmatic, but treatable, condition. The principles of treatment of VM include rescue therapy, lifestyle modification, nonpharmacologic migraine preventives, pharmacologic migraine prophylaxis, and treatment of comorbidities. |
Πέμπτη 29 Αυγούστου 2019
Εγγραφή σε:
Σχόλια ανάρτησης (Atom)
Δεν υπάρχουν σχόλια:
Δημοσίευση σχολίου