Facilitators and Barriers to Weight Loss Among Patients With Idiopathic Intracranial Hypertension.

BACKGROUND: Little is known about motivation for weight loss and barriers to weight loss among patients with idiopathic intracranial hypertension (IIH). Such information is crucial for developing tailored weight management recommendations and novel interventions. METHODS: We administered a survey to patients with IIH presenting to neuro-ophthalmology clinics at The University of Michigan Kellogg Eye Center (Michigan, USA) and St. Thomas' Hospital (London, England). Participants rated importance and motivation to lose weight (1-10 scale; 10 = extremely important/motivated). Facilitators and barriers to weight loss were assessed using open-ended survey questions informed by motivational interviewing methodology. Open-ended responses were coded by 2 team members independently using a modified grounded theory approach. Demographic data were extracted from medical records. Descriptive statistics were used to analyze quantitative responses. RESULTS: Of the 221 (43 Michigan and 178 London) patients with IIH (Table 1), most were female (n = 40 [93.0%] Michigan and n = 167 [94.9%] London). The majority of patients in the United States were White (n = 35 [81.4%] Michigan), and the plurality were Black in the United Kingdom (n = 67 [37.6%] London]) with a mean (SD) BMI of 38.9 kg/m2 (10.6 kg/m2) Michigan and 37.5 kg/m2 (7.7 kg/m2) London. Participants' mean (SD) level of importance to lose weight was 8.5 (2.2) (8.1 [2.3] Michigan and 8.8 [2.1] London), but their mean (SD) level of motivation to lose weight was 7.2 (2.2) (6.8 [2.4] Michigan and 7.4 [2.1] London). Nine themes emerged from the 992 open-ended coded survey responses grouped into 3 actionable categories: self-efficacy, professional resources (weight loss tools, diet, physical activity level, mental health, and physical health), and external factors (physical/environmental conditions, social influences, and time constraints). Most responses (55.6%; n = 551) were about barriers to weight loss. Lack of self-efficacy was the most discussed single barrier (N = 126; 22.9% total, 28.9% Michigan, and 20.4% London) and facilitator (N = 77; 17.5% total, 15.9% Michigan, and 18.7% London) to weight loss. Other common barriers were related to physical activity level (N = 79; 14.3% total, 13.2% Michigan, and 14.8% London) and diet (N = 79; 14.3% total, 9.4% Michigan, and 16.3% London). Commonly reported facilitators included improvements in physical activity level (N = 73; 16.6% total, 18.5% Michigan, and 15.1% London) and dietary changes (N = 76; 17.2% total, 16.4% Michigan, and 17.9% London). CONCLUSIONS: Patients with IIH believe weight loss is important. Self-efficacy was the single most mentioned important patient-identified barrier or facilitator of weight loss, but professional resource needs and external factors vary widely at the individual level. These factors should be assessed to guide selection of weight loss interventions that are tailored to individual patients with IIH.

Temporally independent association of multiple evanescent white dot syndrome and optic neuritis.

PURPOSE: To describe three patients that developed temporally distinct episodes of optic neuritis and multiple evanescent white dot syndrome (MEWDS). METHODS: We retrospectively reviewed the medical records and imaging studies of three women evaluated at a tertiary referral center for both optic neuritis and MEWDS. RESULTS: Three otherwise healthy women, aged 17, 36, and 41, developed temporally separated episodes of optic neuritis and MEWDS. The time periods between the two events were 3, 48, and 60 months, and in two of the three cases, the optic neuritis event preceded the episode of MEWDS. No patient endorsed prodromal flu-like symptoms prior to developing vision loss. The mean presenting visual acuities were better with the optic neuritis episode (LogMAR 0.360, Snellen 20/46) than with retinal event (LogMAR 0.684, Snellen 20/97). All three patients had improvement in vision, with mean visual acuity of 20/29 (LogMAR 0.165) at last follow-up. One patient later developed idiopathic noninfectious posterior uveitis and another developed multiple sclerosis requiring treatment. CONCLUSION: While a rare association, patients can develop both optic neuritis and MEWDS within the same eye at different time points. It is unknown whether such patients are at even higher risk of developing systemic autoimmune disease than are patients with either MEWDS or optic neuritis alone.

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.

Management of Peripapillary Choroidal Neovascular Membrane in Patients With Idiopathic Intracranial Hypertension.

OBJECTIVE: To report the clinical features and treatment outcomes of patients with peripapillary choroidal neovascular membrane (CNVM) secondary to idiopathic intracranial hypertension (IIH). METHODS: Retrospective, multicenter chart review of patients diagnosed with peripapillary CNVM in the course of the treatment and follow-up of IIH. RESULTS: Records were reviewed from 7 different institutions between 2006 and 2016. Ten patients (13 eyes) with a diagnosis of IIH and at least 3 months of follow-up developed CNVM. Three of the total 10 patients developed bilateral CNVM. The mean time from the diagnosis of IIH to CNVM diagnosis was 41 months. Mean follow-up period was 8 months after diagnosis of CNVM. All patients were treated with acetazolamide for IIH. Seven eyes were observed, and 6 eyes were given anti-vascular endothelial growth factor (anti-VEGF) injections, including bevacizumab, ranibizumab, and aflibercept. All CNVMs regressed with subretinal fibrosis, and visual acuity improved in most patients. Papilledema resolved in only 1 eye, while the other 12 eyes had persistent papilledema at last follow-up. CONCLUSIONS: Peripapillary CNVM, a rare complication of IIH, often resolves spontaneously with treatment of IIH. In vision-threatening and/or persistent cases, intravitreal anti-VEGF treatment may be a safe and effective therapeutic option.

Accuracy and Reliability of a Handheld, Nonmydriatic Fundus Camera for the Remote Detection of Optic Disc Edema.

BACKGROUND: Optic disc edema can be an important indicator of serious neurological disease, but is poorly detected using the direct ophthalmoscope. Portable fundus photography may overcome this difficulty. INTRODUCTION: The purpose of this study was to determine the sensitivity and specificity of a handheld, nonmydriatic fundus camera for the detection of optic disc edema. MATERIALS AND METHODS: Retrospective review of nonmydriatic optic disc photographs taken with a portable fundus camera (Pictor Plus; Volk Optical, Mentor, OH) from the University of Michigan Neuro-Ophthalmology Clinics. We included 103 consecutive eyes with optic disc edema and 103 consecutive eyes without optic disc edema of 109 patients. Four masked neuro-ophthalmologists graded a single photograph of each optic disc presented in randomized order and documented the presence of optic disc edema. Sensitivity and specificity of graders' photographic interpretation was compared with clinical examinations. Reliability of assessments within and between graders was determined using kappa statistics. RESULTS: The sensitivity and specificity for detection of optic disc edema were 71.8-92.2% and 81.6-95.2%, respectively. Photos were found to be ungradable in 0-8.3% of cases. The intergrader reliabilities ranged from 0.60 [95% confidence interval (CI): 0.52-0.67] to 0.72 (95% CI: 0.66-0.77). Intragrader reliability ranged from 0.76 (95% CI: 0.63-0.92) to 0.82 (95% CI: 0.69-0.95). DISCUSSION: Photographs taken with portable, nonmydriatic technology met threshold sensitivity and specificity for remote screening for optic disc edema when performed by most, but not all graders. Reliability between graders was moderate-strong and strong within individual providers. CONCLUSIONS: Portable photography holds promise for use in remote screening of optic disc edema.

Transient Monocular Vision Loss on Awakening: A Benign Amaurotic Phenomenon.

BACKGROUND: Transient monocular vision loss (TMVL) is an alarming symptom owing to potentially serious etiologies such as thromboembolism or giant cell arteritis. Our objective is to describe the phenomenon of TMVL present on awakening, which may represent a distinct and benign entity. METHODS: We performed a retrospective observational case series of 29 patients who experienced TMVL on awakening. Patients who described monocular dimming or blackout of vision were included, and those with blurred vision, concurrent eye pain, and binocular vision loss were excluded. Descriptive statistics were used to summarize the study population. RESULTS: Of the 29 patients we studied, 90% (n = 26) were female and 48% had crowded discs (cup-to-disc ratio ≤0.2). The mean age was 45.4 years, although women were significantly younger than men (mean ages 43.4 and 62.7 years, respectively, P = 0.017). Brain magnetic resonance imaging and vascular imaging (magnetic resonance angiography, computed tomographic angiography, or carotid Doppler) were performed in 69% and 55% of cases, respectively, and were uniformly negative. In 14 patients for whom clear follow-up data could be obtained, no medically or visually significant sequelae of this syndrome were found, and 50% experienced resolution of symptoms. CONCLUSIONS: Evaluation was uniformly negative when patients described waking with isolated vision loss in 1 eye with subsequent resolution, usually in less than 15 minutes. The natural history seems benign with symptoms frequently remitting spontaneously. This visual phenomenon may represent an autoregulatory failure resulting in a supply/demand mismatch during low-light conditions.

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.

Levonorgestrel intrauterine device use and incident idiopathic intracranial hypertension among commercially insured women.

OBJECTIVES: To estimate the hazard of incident idiopathic intracranial hypertension, a potentially blinding condition, among women using levonorgestrel intrauterine devices (LNG-IUD) compared to copper IUD, as conflicting associations have been reported. STUDY DESIGN: This retrospective, longitudinal cohort study identified women ages 18-45 years in a large care network (January 1, 2001, to December 31, 2015) using LNG-IUD, subcutaneous etonogestrel implant, copper IUD, tubal device/surgery, or hysterectomy. Incident idiopathic intracranial hypertension was defined as the first diagnosis code for after 1 year without any codes and following brain imaging or lumbar puncture. Kaplan-Meier analysis estimated time-dependent probabilities of idiopathic intracranial hypertension at 1 and 5 years after incident contraception use, stratified by type. Cox regression estimated the hazard of idiopathic intracranial hypertension associated with LNG-IUD use compared to copper IUD (primary comparison) after adjusting for sociodemographics and factors associated with idiopathic intracranial hypertension (e.g., obesity) or contraception selection. A sensitivity analysis with propensity score-adjusted models was performed. RESULTS: Of 268,280 women, 78,175 (29%) used LNG-IUD, 8715 (3%) etonogestrel implant, 20,275 (8%) copper IUD, 108,216 (40%) hysterectomy, 52,899 (20%) tubal device/surgery, and 208 (0.08%) developed idiopathic intracranial hypertension over a mean follow-up of 2.4 ± 2.4 years. Also, 1-/5-year Kaplan-Meier idiopathic intracranial hypertension probabilities were 0.0004/0.0021 for LNG-IUD and 0.0005/0.0006 for copper IUD users. LNG-IUD use did not show significantly different hazard of idiopathic intracranial hypertension compared to copper IUD (adjusted hazard ratio 1.84 [95% CI 0.88, 3.85]). Sensitivity analyses were similar. CONCLUSIONS: We did not observe a significantly increased hazard of idiopathic intracranial hypertension among women using LNG-IUD compared to copper IUDs. IMPLICATIONS: The lack of an association between LNG-IUD use and idiopathic intracranial hypertension in this large observational study provides reassurance to women considering initiation or continued use of this highly effective contraceptive method.

Multiple sclerosis presenting as acute acquired comitant esotropia in a pediatric patient.

Acute acquired comitant esotropia (AACE) is a rare form of esotropia in the older pediatric population. Although the workup for pediatric AACE varies, patients often do not undergo lab testing and imaging, because the overwhelming majority of cases are idiopathic. We describe AACE as the presenting manifestation of multiple sclerosis in a pediatric patient. His only other finding was a horizontal jerk nystagmus isolated to end gaze. Magnetic resonance imaging revealed extensive demyelinating lesions, with a small thalamic lesion possibly accounting for his esotropia. Our case underscores the need for extensive diagnostic workup for any ophthalmic or neurologic findings or symptoms accompanying AACE.

Pearls: visual loss.

The clinician who is evaluating a patient with visual loss faces a large initial differential diagnosis. By taking a systematic approach to the history of the present illness, including reviewing systems, examination, and subsequent testing, several potential pitfalls will be avoided, and a brief differential diagnosis list can be generated. This article uses illustrative cases to present a variety of history, examination, and testing pearls for diagnosing the source of visual loss.

Association of Individual-Level Factors With Visual Outcomes in Optic Neuritis: Secondary Analysis of a Randomized Clinical Trial.

Importance: Using corticosteroids to treat acute demyelinating optic neuritis has been identified as an area for shared decision-making. However, no analysis exists to support personalized shared decision-making that considers long- and short-term treatment benefits. Objective: To develop models of individual-level visual outcomes for patients with optic neuritis. Design, Setting, and Participants: This secondary analysis of the Optic Neuritis Treatment Trial (ONTT), a randomized clinical trial, was performed at 14 academic eye centers and 1 large community eye center. Adults aged 18 to 46 years with incident acute unilateral optic neuritis within 8 days of vision loss onset were included. Data were collected from July 1988 to June 1991, downloaded on October 15, 2018, and analyzed from January 24, 2019, to February 20, 2020, using multivariable linear regression modeling. Exposures: Intravenous corticosteroids vs placebo. Main Outcomes and Measures: Visual acuity (VA) at 1 year. Secondary outcomes were 1-year contrast sensitivity (CS) and VA and CS at 15 and 30 days. Independent variables included age, sex, race, multiple sclerosis status, optic neuritis episodes in the fellow eye, vision symptoms (days), pain, optic disc swelling, viral illness, treatment group, and baseline VA or CS. Results: Of the 455 participants, median age was 31.8 (interquartile range [IQR], 26.3-37.0) years; 350 (76.9%) were women; and 388 (85.3%) were white. For 410 participants (90.1%) with 1-year outcomes, median VA improved from 20/66 (IQR, 20/28-20/630) at enrollment to 20/17 (IQR, 20/14-20/21) at 1 year. Baseline VA was the primary variable associated with 1-year VA (regression coefficient, 0.056 [95% CI, 0.008-0.103]; P = .02) if baseline VA was better than count fingers (CF). At 15 days, baseline VA and treatment status were associated with VA in those participants with baseline VA better than CF (regression coefficient, 0.305 [95% CI, 0.231-0.380]; F = 9.42; P < .001). However, the difference of medians (20/18 [95% CI, 20/17-20/19] with intravenous corticosteroids vs 20/23 [95% CI, 20/21-20/26] with placebo) was small for the median VA (20/66) in the trial. Treatment was not associated with 15-day or 1-year VA in participants with baseline VA of CF or worse. Conclusions and Relevance: In this study, long-term VA was associated with severity of baseline vision loss. Early benefits with intravenous corticosteroid treatment were limited to participants with baseline VA better than CF. However, the early, temporary benefit of intravenous corticosteroids is of questionable clinical significance and should be weighed against potential harms.

Posterior Cortical Atrophy: Characteristics From a Clinical Data Registry.

Background: Posterior cortical atrophy (PCA) is a neurodegenerative syndrome that presents with higher-order visual dysfunction with relative sparing of memory and other cognitive domains, and it is most commonly associated with Alzheimer's disease pathology. There is a lack of data regarding the presentation of PCA to non-cognitive specialists. Therefore, we collected clinical data from neuro-ophthalmologists regarding the presentation of PCA to their practices and compared data to published cohorts and a published survey of cognitive specialists. Methods: Members of the North American Neuro-Ophthalmology Society Listserv (NANOSnet) were invited to complete an online, retrospective, chart-review data-entry survey regarding their patients with PCA, and REDCap was used for data collection. Results: Data for 38 patients were entered by 12 neuro-ophthalmologists. Patient mean age at presentation was 67.8 years, and 74% of patients were women. Difficulty reading was reported at presentation by 91% of patients, and poor performance on color vision, stereopsis, and visual field testing (performed reliably by 36/38 patients) were common findings. Most patients who were treated were treated with donepezil and/or memantine. Conclusions: Compared to published data from cognitive specialists, patients presenting to neuro-ophthalmology with PCA were more likely to be older and female and have a reading complaint. Reliable visual field testing was the norm with homonymous defects in the majority of patients. The neuro-ophthalmologist plays an important role in diagnosing PCA in older adults with unexplained visual signs and symptoms, and future studies of PCA should involve multiple specialists in order to advance our understanding of PCA and develop effective treatments.

Successful transarterial embolization of a Barrow type D dural carotid-cavernous fistula with ethylene vinyl alcohol copolymer (Onyx).

Endovascular occlusion via the transvenous route is the favored treatment method for dural carotid-cavernous fistulas (CCFs). Ethylene vinyl alcohol copolymer (Onyx), recently approved for treatment of arteriovenous malformations, has advantages over conventional liquid embolic agents in its nonadhesive nature, which allows for longer injections with decreased risk of catheter retention. We report the use of Onyx in the successful transarterial embolization of a dural CCF fed by arterial branches of the internal and external carotid arteries (Barrow type D) after multiple failed attempts to access the cavernous sinus transvenously. Transarterial Onyx embolization could be a valuable option in transarterial treatment of CCFs when venous access is difficult.

Sensitivity of orbital magnetic resonance imaging in acute demyelinating optic neuritis.

OBJECTIVE: To determine the sensitivity of orbital magnetic resonance imaging (MRI) in acute demyelinating optic neuritis (ON) in routine clinical practice, and the added value of a dedicated neuroradiology interpretation. DESIGN: Retrospective chart review. PARTICIPANTS: Patients with clinically proven ON evaluated between 2004 and 2014 in the University of Michigan neuro-ophthalmology clinics. Inclusion criteria involved visual recovery and orbital MRI completed within 30days of symptom onset and before corticosteroid treatment. METHODS: Demographics, clinical examination, and MRI report data (high T2 signal, gadolinium contrast enhancement) were abstracted for each eligible eye. Every MRI was reinterpreted by a neuroradiologist masked to the affected side. Descriptive statistics summarized patient and eye characteristics. Interrater agreement between the neuroradiologist and the radiology report for the radiographic diagnosis of ON was assessed with Cohen's kappa statistic. RESULTS: Of 92 patients who met all inclusion criteria, 70 (76.1%) were reported to have at least 1 MRI feature consistent with ON. After dedicated review by a neuroradiologist, 77 (83.7%) were determined to have a positive MRI for ON. Agreement between the neuroradiologist and MRI report was moderate (κ = 0.63). Gadolinium enhancement was the most common feature in MRI positive ON (72 [78.3%] of neuroradiology reviewed MRIs; 66 [71.7%] of clinical MRI reports). CONCLUSIONS: The sensitivity of MRI in ON was lower than previously reported and confirms the importance of making a clinical diagnosis of ON without relying on neuroimaging for confirmation. MRI interpretation by a skilled neuroradiologist increased sensitivity, underscoring the complexity of orbital MRI interpretation.

Treatment of Ocular Myasthenia Gravis.

Myasthenia gravis is a relatively common neuromuscular disorder, with ocular myasthenia gravis being a subset defined as myasthenia gravis limited to the orbicularis, levator, and extraocular muscles. Patients with ocular myasthenia gravis can have disabling diplopia or functional blindness from ptosis and in most cases treatment is required. Like generalized myasthenia gravis, there are a variety of treatments available that include pyridostigmine, immunosuppression, intravenous immunoglobulin, plasmapheresis, thymectomy, lid crutches, ptosis surgery, and extraocular muscle surgery. Unfortunately, there is limited data on the use of individual treatments in ocular myasthenia gravis and no data comparing treatments. Using a combination of available data on treatment of generalized myasthenia gravis, data on treatment of ocular myasthenia gravis, best practices, and clinical experience we will provide a rational framework for treatment of ocular myasthenia gravis.

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.