Central Retinal Artery Occlusion Associated with Takayasu Arteritis.

Takayasu arteritis is a chronic inflammatory vasculitis with granulomatous panarteritis particularly impacting large vessels including the aorta and its branches, especially the subclavian arteries, with clinical manifestation dependent on the involved artery. Sequelae of the active disease vary, including stenosis, occlusions, or aneurysmal dilatations of the large vessels. The prevalence of Takayasu arteritis is higher in the Asian population and in Japan, but quite low in the United States, varying from 0.9-8.4 per million people. Ocular manifestations are rare and lead to a delay in diagnosis and appropriate treatment. Ocular manifestations include Takayasu retinopathy, anterior ischemic optic neuropathy (AION), retinal artery occlusion (RAO) and retinal vein occlusion (RVO). We present two cases in which central retinal artery occlusion (CRAO) was associated with Takayasu arteritis. CRAO is an ophthalmic emergency with an incidence of 1.9 per 100,000 person years in the United States; only 5% of cases are arteritic, which can be observed with inflammatory vasculitides secondary to the formation of immune deposits.

Ocular Neuromyotonia: Clinical Features, Diagnosis, and Outcomes.

PURPOSE: The purpose of this study was to describe the clinical features, management, outcomes, and diagnostic pitfalls in a large series of patients with ocular neuromyotonia. DESIGN: Retrospective cohort. METHODS: Patients diagnosed with ocular neuromyotonia from January 1, 2004, through January 1, 2023, seen at one of the 3 Mayo Clinic sites in Rochester, MN, Scottsdale, AZ, and Jacksonville, FL, comprised the study population. We ascertained patients with ocular neuromyotonia through a search using the medical records database. Only patients with an observed episode of ocular neuromyotonia were included and the medical records were reviewed. The main outcome measures were clinical features and outcomes of patients with ocular neuromyotonia. RESULTS: Forty-two patients who were diagnosed with ocular neuromyotonia were included. The median age was 58 years (range, 16-80 years). A history of cranial radiation therapy was present in 39 patients (93%). The sixth cranial nerve was involved in 31 patients (74%). Bilateral disease was found in 2 patients (5%). The median time from onset of diplopia to diagnosis was 8 months (range, 1 month-25 years), with a high rate of initial misdiagnosis in 52%. Twenty of 42 patients (48%) were treated with oral medication, of whom 95% had significant improvement or resolution of symptoms. CONCLUSION: Prior cranial irradiation is the most common cause for ocular neuromyotonia, affecting the sixth cranial nerve most often. Although delayed and initial misdiagnosis is common, most patients show improved symptoms on medical treatment.

OS reboot.

Cerebrospinal fluid (CSF) diversion or shunting procedures are the most commonly performed surgery for the treatment of hydrocephalus and are often employed in the management of elevated intracranial pressure due to a variety of diseases. Despite their popularity however, approximately 50% of shunts fail within the first two years, and several revisions are required within the first decade after placement. Ophthalmologists may encounter patients with a CSF shunt to evaluate for concerns of vision loss or diplopia and to determine if papilledema is present. We discuss the neuro-ophthalmic manifestations and evaluation of possible CSF shunt malfunction.

Lumbar Puncture for Diagnosis of Idiopathic Intracranial Hypertension in Typical Patients.

BACKGROUND: Patients with typical features of pseudotumor cerebri syndrome (PTCS) must undergo lumbar puncture (LP) to demonstrate elevated opening pressure and cerebrospinal fluid (CSF) analysis to rule out alternative diagnoses. As LP may be associated with significant morbidity, this study aims to determine its necessity in diagnosing typical PTCS. METHODS: Retrospective chart review at 3 university-based neuro-ophthalmology practices included women aged 18-45 years with body mass index >25, papilledema, negative neuroimaging, and who met criteria for PTCS or probable PTCS. RESULTS: One hundred fifty-six patients were enrolled. Seven (4.5%) had clinically insignificant CSF abnormalities. No diagnoses or management changed based on LP/CSF results. CONCLUSION: LP may not be routinely required in the initial evaluation of typical patients with PTCS evaluated by experienced clinicians We caution, however, that further prospective study is required to determine potential risks and benefits of LP as a tool in the diagnosis of IIH before recommending general practice changes.

Neuro-Ophthalmic Features of Autoimmune Encephalitides.

BACKGROUND: Over the past decade, there has been a remarkable advancement in the understanding of autoimmune etiologies of encephalitis. The first identified generation of paraneoplastic encephalitis tends to occur in older populations, responds poorly to immunotherapy, and is mediated by T-cell damage with antibodies directed toward intracellular antigens. A new generation of autoimmune encephalitides has been described, which are mediated by antibodies to cell-surface proteins, tend to occur in younger individuals, are less frequently associated with malignancy, and often respond better to treatment compared to their intracellular antigen-related paraneoplastic counterparts. This review will focus on several specific antibody-mediated autoimmune encephalitides with neuro-ophthalmic pertinence. EVIDENCE ACQUISITION: Literature review and personal clinical experience. RESULTS: Several of the antibody-mediated encephalitides, specifically N-methyl-D-aspartate receptor, dipeptidyl-peptidase-like protein 6, glial fibrillary acidic protein, metabotropic glutamate receptor 1 (mGluR1), gamma-aminobutyric acid receptor, glutamic acid decarboxylase 65 (GAD65), collapsing response mediator protein 5 (CRMP5), and kelch-like protein 11 (KLHL11), contain features of neuro-ophthalmic interest. CONCLUSIONS: The novel cell-surface protein-directed autoimmune encephalitis group can present with a wide range of afferent and efferent neuro-ophthalmic manifestations. Neuro-ophthalmologists should be familiar with these antibody-associated syndromes, which are treatable and often require a high index of suspicion for diagnosis.

Endocrine and Visual Outcomes Following Gross Total Resection and Subtotal Resection of Adult Craniopharyngioma: Systematic Review and Meta-Analysis.

OBJECTIVE: The optimal surgical strategy for management of adult patients with craniopharyngioma remains controversial. To analyze the functional outcomes ofadult patients with gross total resection (GTR) and subtotal resection (STR) of craniopharyngioma. METHODS: MEDLINE, EMBASE, Scopus, and Cochrane databases were searched from inception to July 19, 2018, for articles comparing postoperative endocrine function, vision, complications, and recurrence rates for adult patients with GTR and STR of craniopharyngioma. The articles were analyzed by meta-analysis of proportions using a random-effects model to calculate summary odds ratios (ORs). RESULTS: The initial search resulted in 2468 studies and 540 studies selected for full text review. Seventeen studies were included in the final analyses with 748 patients in the GTR cohort and 559 patients in the STR cohort. GTR resulted in a significantly lower likelihood of recurrence when compared with STR (OR, 0.106; 95% confidence interval [CI], 0.067-0.168; P < 0.001), but a significantly greater likelihood of panhypopituitarism (OR, 2.063; 95% CI, 1.058-4.024; P = 0.034) and permanent diabetes insipidus (OR, 2.776; 95% CI, 1.321-5.832; P = 0.007). There was no significant difference between the groups for postoperative worsened vision (P = 0.868), improved vision (P = 0.876), pathologic weight gain (P = 0.724), cerebrospinal fluid leak (P = 0.788), complications (P = 0.656), or death (P = 0.261). CONCLUSIONS: This is the first systematic review of functional outcomes of adult patients with craniopharyngioma. GTR results in decreased likelihood of recurrence, but increased likelihood of postoperative panhypopituitarsm and permanent diabetes insipidus. Surgeons should be aware of these associations when determining the optimal operative strategy for adult patients with craniopharyngioma.

Disorders of Vergence Eye Movements.

PURPOSE OF REVIEW: The purpose of this review is to summarize current understanding regarding disorders of gaze with comitant ocular misalignment at distance or near and a full range of extraocular movement. Emphasis is placed on clinical features that may be used to differentiate underlying neurologic disease from the more common benign causes. The approach to the diagnostic evaluation and treatment is discussed. RECENT FINDINGS: Randomized controlled trials and Cochrane review suggest the superiority of formal office-based vision therapy in treating convergence insufficiency in children. Divergence insufficiency in older adults is a common disorder caused by involution of connective tissues in the orbit. In contrast, divergence insufficiency in children may be a harbinger of central nervous system disease, particularly intracranial tumors. Disorders of vergence are common in pediatric and aging adult populations. Benign causes are common but appropriate history and exam emphasizing ocular motility is essential to rule out more concerning diagnoses. Atypical presentations should prompt comprehensive evaluation including neuroimaging. Treatment of benign causes of vergence abnormalities should have a stepwise approach, beginning with the least invasive available intervention, though some patients may require surgery.

Treatment of Blepharospasm/Hemifacial Spasm.

OPINION STATEMENT: The treatment of both hemifacial spasm (HFS) and blepharospasm (BEB) requires making the appropriate clinical diagnosis. Advance imaging and electrophysiologic studies are useful; however, one's clinical suspicion is paramount. The purpose of this review is to summarize current and emerging therapies for both entities. Botulinum toxin (BTX) remains the first-line therapy to treat both conditions. If chemodenervation has failed, surgery may be considered. Due to the risks associated with surgery, the benefits of this option must be carefully weighed. Better surgical outcomes are possible when procedures are performed at tertiary centers with experienced surgeons and advanced imaging techniques. Microvascular decompression is an efficacious method to treat HFS, and myectomy is an option for medication-refractory BEB; the risks of the latter may outweigh any meaningful clinical benefits. Oral agents only provide short-term relief and can cause several unwanted effects; they are reserved for patients who cannot receive BTX and/or surgery. Transcranial magnetic stimulation has gained some traction in the treatment of BEB and may provide safer non-invasive options for refractory patients in the future.

Preserved Visual Acuity in Anterior Ischemic Optic Neuropathy Secondary to Giant Cell (temporal) Arteritis.

OBJECTIVE: To evaluate the prevalence and clinical profile of patients with biopsy-proven arteritic anterior ischemic optic neuropathy presenting with preserved visual acuity of 20/40 or better and those with an initial poor visual acuity of 20/50 or worse through a retrospective chart review. RESULTS: Nine of 37 patients with arteritic anterior ischemic optic neuropathy presented with a preserved visual acuity of 20/40 or better in the affected eye. All patients with preserved visual acuity had initial visual field defects that spared the central field. All 37 patients immediately received high-dose corticosteroid therapy. Visual acuity worsened by > 2 lines in one of nine patients (11%) with preserved visual acuity, with a corresponding progression of visual field constriction. CONCLUSION: Although preserved visual acuity of 20/40 or better has traditionally been associated with the nonarteritic form of anterior ischemic optic neuropathy, giant cell arteritis should still be strongly considered, especially if they have giant cell arteritis systemic symptoms.

Clinical Utility of Acetylcholine Receptor Antibody Testing in Ocular Myasthenia Gravis.

IMPORTANCE: The sensitivity of acetylcholine receptor (AChR) antibody testing is thought to be lower in ocular myasthenia gravis (OMG) compared with generalized disease, although estimates in small-scale studies vary. There is little information in the literature about the implications of AChR antibody levels and progression from OMG to generalized myasthenia gravis. OBJECTIVES: To test the hypothesis that serum AChR antibody testing is more sensitive in OMG than previously reported and to examine the association between AChR antibody levels and progression from OMG to generalized myasthenia gravis. DESIGN, SETTING, AND PARTICIPANTS: A retrospective, observational cohort study was conducted of 223 patients (mean [SD] age, 59.2 [16.4] years; 139 [62.3%] male) diagnosed with OMG between July 1, 1986, and May 31, 2013, at 2 large, academic medical centers. MAIN OUTCOMES AND MEASURES: Baseline characteristics, OMG symptoms, results of AChR antibody testing, and progression time to generalized myasthenia gravis (if this occurred) were recorded for each patient. Multiple logistic regression was used to measure the association between all clinical variables and antibody result. Kaplan-Meier survival analysis was performed to examine time to generalization. RESULTS: Among the 223 participants, AChR antibody testing results were positive in 158 participants (70.9%). In an adjusted model, increased age at diagnosis (odds ratio [OR], 1.03; 95% CI, 1.01-1.04; P = .007) and progression to generalized myasthenia gravis (OR, 2.92; 95% CI, 1.18-7.26; P = .02) were significantly associated with positive antibody test results. Women were less likely to have a positive antibody test result (OR, 0.36; 95% CI, 0.19-0.68; P = .002). Patients who developed symptoms of generalized myasthenia gravis had a significantly higher mean (SD) antibody level than those who did not develop symptoms of generalized myasthenia gravis (12.7 [16.5] nmol/L vs 4.2 [7.9] nmol/L; P = .002). CONCLUSIONS AND RELEVANCE: We demonstrate a higher sensitivity of AChR antibody testing than previously reported in the largest cohort of patients with OMG available to date. Older age, male sex, and progression to generalized myasthenia gravis were significantly associated with a positive antibody test result. In addition, to our knowledge, this is the first report of an association between high AChR antibody levels and progression from OMG to generalized disease.

Prognosis of Ocular Myasthenia Gravis: Retrospective Multicenter Analysis.

PURPOSE: To calculate the rate and timing of conversion from ocular myasthenia gravis to generalized myasthenia gravis. DESIGN: Retrospective multicenter analysis. SUBJECTS: Patients included in the study were diagnosed with ocular myasthenia gravis without the presence of generalized disease at onset. METHODS: We conducted a retrospective multicenter analysis. We reviewed charts of 158 patients who met diagnostic criteria for ocular myasthenia gravis. Patients were divided into 2 subgroups: an immunosuppressant treatment group and a nonimmunosuppressant treatment group. Timing of conversion to generalized disease and duration of follow-up also was evaluated. Additional data such as clinical symptoms at presentation, laboratory test results, and chest imaging results also were recorded. MAIN OUTCOME MEASURES: Conversion rates to generalized myasthenia at 2 years, effect of immunosuppression on conversion, and timing of conversion. RESULTS: The 158-patient cohort included 76 patients who received immunosuppressant therapy; the remaining 82 patients did not. The overall conversion rate to generalized disease was 20.9%. At 2 years, generalized myasthenia developed in 8 of 76 patients in the treated group and in 15 of 82 patients in the nonimmunotherapy group (odds ratio, 0.52; 95% confidence interval, 0.20-1.32). Median time for conversion to generalized disease was 20 months in the nonimmunosuppressant group and 24 months in the immunosuppressant group. Conversion occurred after 2 years of symptom onset in 30% of patients. CONCLUSIONS: Conversion rates from ocular to generalized myasthenia gravis may be lower than previously reported both in immunosuppressed and nonimmunosuppressed patients. A subset of patients may continue to convert to generalized disease beyond 2 years from onset of symptoms, and close monitoring should be continued.

Skew deviation: clinical updates for ophthalmologists.

PURPOSE OF REVIEW: This article discusses the current approach in diagnosing skew deviation, as well as recent findings in the lesion localization. RECENT FINDINGS: Skew deviation can be defined as vertical misalignment of the eyes that does not map to any of cyclovertical muscles, in association with neurologic symptoms and signs and with posterior fossa lesion. It can be differentiated from trochlear nerve palsy by the direction of ocular torsion and the change in the degree of vertical deviation with upright and supine head position. It is commonly caused by ischemia of the posterior paramedian pons, medial thalamus, or cerebellum. Other less common mechanism being demyelinating lesion, mass effect, infection, hemorrhage, or intracranial hypertension. When the vestibular nuclei are involved, skew deviation may occur with acute vestibular syndrome. Ground-in or Fresnel prism may alleviate diplopia in relatively small vertical deviation; however, patient with larger deviation or with the presence of ocular torsion may benefit from surgery of the cyclovertical muscles. SUMMARY: Skew deviation can be appropriately diagnosed from the nature of the ocular torsion and the vertical deviation, along with the presence of lesion involving posterior paramedian pons and/or medial thalamus.

Clinical utility and assessment of cyclodeviation.

PURPOSE OF REVIEW: This article reviews and updates basic concepts, diagnosis and treatment of cyclotorsion. RECENT FINDINGS: Cyclodeviation in congenital superior oblique palsy (SOP) seems to correlate with the extent of superior oblique muscle hypoplasia. Genetic polymorphisms such as PHOX2B polymorphism, considered to be risk factors for congenital fibrosis of the extraocular muscles, may play a role in SOP and consequently in cyclotorsion. Two components of the ocular tilt reaction, ocular torsion and tilt of subjective visual vertical, seem to share similar sites of impairment in the brainstem. Harada-Ito surgery continues to be the procedure of choice in patients with isolated cyclodeviation, evidencing better outcome if less than 10° of preoperative excyclotorsion and preoperative fusion exist. Ocular torsion is not infrequent in patients with intermittent exotropia, especially in the most exo-deviated eye, emphasizing a possible role in pathogenesis. A new device for the assessment of dynamic torsion during ocular counter roll response using after-image has been described. Similarly, a new method to measure objective ocular torsion using retinal arcade tilt as a reference has been proposed. Finally, torsional data transformation such as the sum of angles of excyclodeviation, rather than using the angle of excyclodeviation of the paretic eye, is becoming increasingly popular among studies on torsion. SUMMARY: Exciting developments on ocular torsion have been described recently, and new ways to access and interpret ocular torsion have been devised as well.

Persistent diplopia and superior oblique muscle dysfunction following dissection of the orbital periosteum in cranial base surgery.

BACKGROUND/AIMS: Persistent diplopia secondary to a fourth cranial nerve palsy is poorly documented after open cranial base surgery. METHODS: Six cases of fourth cranial nerve palsy after cranial base surgery were drawn from the Neuro-Ophthalmology and Head and Neck Surgery Clinics at the University of Michigan from 2004 to 2012. RESULTS: Six patients developed diplopia and ocular misalignment in a pattern suggestive of superior oblique palsy following dissection of the medial orbital periosteum as part of a surgical approach to the anterior cranial base. Among the four patients in whom follow-up examination was available, the misalignment improved spontaneously in three patients and was stable in the fourth patient, but did not completely resolve in any patient. CONCLUSIONS: This sparsely documented phenomenon is likely caused by dysfunction of the superior oblique muscle, possibly the result of malposition of the trochlea after spontaneous reattachment of the periosteum. Special factors such as invasive tumours, repeated surgeries of this nature, prior radiation, or chemical cementing material that adversely affects wound healing may be contributory.

Isolated third, fourth, and sixth cranial nerve palsies from presumed microvascular versus other causes: a prospective study.

PURPOSE: To estimate the proportion of patients presenting with isolated third, fourth, or sixth cranial nerve palsy of presumed microvascular origin versus other causes. DESIGN: Prospective, multicenter, observational case series. PARTICIPANTS: A total of 109 patients aged 50 years or older with acute isolated ocular motor nerve palsy. TESTING: Magnetic resonance imaging (MRI) of the brain. MAIN OUTCOME MEASURES: Causes of acute isolated ocular motor nerve palsy (presumed microvascular or other) as determined with early MRI and clinical assessment. RESULTS: Among 109 patients enrolled in the study, 22 had cranial nerve III palsy, 25 had cranial nerve IV palsy, and 62 had cranial nerve VI palsy. A cause other than presumed microvascular ischemia was identified in 18 patients (16.5%; 95% confidence interval, 10.7-24.6). The presence of 1 or more vasculopathic risk factors (diabetes, hypertension, hypercholesterolemia, coronary artery disease, myocardial infarction, stroke, and smoking) was significantly associated with a presumed microvascular cause (P = 0.003, Fisher exact test). Vasculopathic risk factors were also present in 61% of patients (11/18) with other causes. In the group of patients who had vasculopathic risk factors only, with no other significant medical condition, 10% of patients (8/80) were found to have other causes, including midbrain infarction, neoplasms, inflammation, pituitary apoplexy, and giant cell arteritis (GCA). By excluding patients with third cranial nerve palsies and those with GCA, the incidence of other causes for isolated fourth and sixth cranial nerve palsies was 4.7% (3/64). CONCLUSIONS: In our series of patients with acute isolated ocular motor nerve palsies, a substantial proportion of patients had other causes, including neoplasm, GCA, and brain stem infarction. Brain MRI and laboratory workup have a role in the initial evaluation of older patients with isolated acute ocular motor nerve palsies regardless of whether vascular risk factors are present.

Saccadic intrusions: review and update.

PURPOSE OF REVIEW: This work reviews saccadic intrusions focusing on recent developments in pathophysiology and treatment. RECENT FINDINGS: Saccadic intrusions have been recognized as features of oculomotor apraxia type 2 and neuromyelitis optica. Novel fixation instabilities have been identified such as 'staircase' square wave jerks, or the pervasive ocular microtremor seen in Parkinson's disease. Although evidence supports a network underlying the pathophysiology of square wave jerks involving cerebral hemispheres, subcortex, brainstem and cerebellum, the debate regarding the pathogenesis of ocular flutter and opsoclonus centres on a cerebellar and brainstem hypotheses. The cerebellar hypothesis explains functional imaging findings, whereas the brainstem hypothesis provides possible explanations for some therapeutic responses as well as accompanying myoclonus, startle and tremor. A study of immunotherapies in children with opsoclonus-myoclonus syndrome found that treatment combinations were more effective than corticotropin alone. SUMMARY: Recognition of saccadic intrusions can assist in the diagnosis of neurological disease. We are gaining new insights about pathogenesis through models, functional imaging and genetic approaches.

Current electrophysiology in ophthalmology: a review.

PURPOSE OF REVIEW: To summarize current technique, indications, and pitfalls of electrophysiologic testing used in ophthalmology. RECENT FINDINGS: Visual evoked potentials (VEPs) may be useful as an objective measurement of refractive error in complicated patients. VEP P100 latency was found superior to color vision and visual field in early stages of hydroxychloroquine maculopathy. VEP results can be predictive of visual recovery in traumatic optic neuropathy. Multifocal electroretinogram (ERG) or VEP can provide an objective assessment of visual field defects not yet present on automated perimetry in patients with glaucomatous and nonglaucomatous optic neuropathies. In patients with intraocular lymphoma, reduced amplitudes of all ERG components can be recorded, with the b-wave amplitude being most significantly affected. SUMMARY: Various visual electrophysiologic tests are useful to the ophthalmologist, each with different indications. The flash ERG is most useful in diffuse retinal disorders, whereas the multifocal ERG is superior in localized retinal disease. VEPs can be valuable in diagnosing optic neuropathies, nonorganic visual loss, and assessing visual function in infants or children.