Impact of Pregnancy on Papilledema and Vision Loss in Idiopathic Intracranial Hypertension Patients: A Chart Review and Case Series of 13 Patients.

BACKGROUND: Studies suggest that weight gain is a prominent risk factor for recurrence of papilledema in idiopathic intracranial hypertension (IIH). Given this information, the significant weight gain that occurs during pregnancy, and the fact that pharmacologic therapy is many times discontinued, raises concerns for worsening edema and vision loss. To examine the impact of pregnancy weight gain on IIH, a retrospective chart review of patients with IIH and pregnancy was performed. Compared with previous studies, we 1) quantified findings with optical coherence tomography (OCT) and Humphrey visual field (HVF) data, 2) Included baseline data before pregnancy, 3) determined excess pregnancy weight gain using body mass index-adjusted weight gain goals, and 4) correlated worsening in IIH symptoms with changes in papilledema. METHODS: Charts were reviewed for patients with diagnoses of IIH who had at least 2 visits with neuro-ophthalmology during pregnancy. Thirteen patients met inclusion criteria. Data were compared from baseline visits before pregnancy, pregnancy visits, and postpregnancy visits. RESULTS: Comparisons of HVF mean deviation (MD), OCT retinal nerve fiber layer (RNFL), and Max OCT RNFL during pregnancy were not significant compared with baseline ( P = 0.51, 0.41, and 0.25). Three patients were found to have increased papilledema during pregnancy (Max Avg OCT RNFL of 152.5, 129, and 123.5 µm) of which 2 developed new reproducible mild visual field defects (HVF ∆MD -1.78 and -4.49). All patients showed more than the 6% weight gain, typically observed in recurrent IIH. Eleven patients gained more than their weight from initial diagnosis. Eight patients had excess pregnancy weight gain. Six patients discontinued pharmacologic therapy for IIH. CONCLUSIONS: Weight gain seems to carry a lower risk in IIH patients when associated with pregnancy. This is suggested by the high rate of stable or even decreased disc edema in patients despite medication discontinuation and excess pregnancy weight gain. We postulate these findings may be related to changes in weight distribution or endocrine changes during pregnancy.

Misdiagnosis of Third Nerve Palsy.

BACKGROUND: This study identifies the diagnostic errors leading to misdiagnosis of 3rd nerve palsy and to aid clinicians in making this diagnosis. The objective of this article is to determine the incidence of misdiagnosis of 3rd cranial nerve palsy (3rd nerve palsy) among providers referring to a tertiary care neuro-ophthalmology clinic and to characterize diagnostic errors that led to an incorrect diagnosis. METHODS: This was a retrospective clinic-based multicenter cross-sectional study of office encounters at 2 institutions from January 1, 2014, to January 1, 2017. All encounters with scheduling comments containing variations of "3rd nerve palsy" were reviewed. Patients with a documented referral diagnosis of new 3rd nerve palsy were included in the study. Examination findings, including extraocular movement examination, external lid examination, and pupil examination, were collected. The final diagnosis was determined by a neuro-ophthalmologist. The Diagnosis Error Evaluation and Research (DEER) taxonomy tool was used to categorize the causes of misdiagnosis. Seventy-eight patients referred were for a new diagnosis of 3rd nerve palsy. The main outcome measure was the type of diagnostic error that led to incorrect diagnoses using the DEER criteria as determined by 2 independent reviewers. Secondary outcomes were rates of misdiagnosis, misdiagnosis rate by referring specialty, and examination findings associated with incorrect diagnoses. RESULTS: Of 78 patients referred with a suspected diagnosis of 3rd nerve palsy, 21.8% were determined to have an alternate diagnosis. The most common error in misdiagnosed cases was failure to correctly interpret the physical examination. Ophthalmologists were the most common referring provider for 3rd nerve palsy, and optometrists had the highest overdiagnosis rate of 3rd nerve palsy. CONCLUSIONS: Misdiagnosis of 3rd nerve palsy was common. Performance and interpretation of the physical examination were the most common factors leading to misdiagnosis of 3rd nerve palsy.

Peripapillary Pachychoroid in Nonarteritic Anterior Ischemic Optic Neuropathy.

PURPOSE: This study examined the peripapillary choroidal thickness (PCT) in nonarteritic ischemic optic neuropathy (NAION) in comparison to contralateral eyes and normal eyes. METHODS: We used enhanced depth imaging spectral-domain optical coherence tomography to image the optic nerve head of 20 NAION, 10 contralateral eyes, and 102 normal eyes. Following compensation, the scans were manually delineated to identify relevant surfaces including Bruch's membrane opening (BMO), Bruch's membrane, and anterior sclera. The PCT was defined as the measurement between Bruch's membrane and the anterior sclera and was measured at increasing distance from BMO. Models adjusted for age, BMO area, and axial length were used to compare the mean PCT between NAION and normal eyes, and contralateral eyes and normal eyes. Paired t-tests were used to compare the PCT between NAION and contralateral eyes. RESULTS: The mean PCT was thicker in NAION and contralateral eyes when compared with normal eyes at all distances from BMO (P < 0.001). The PCT was not significantly thicker in contralateral eyes when compared with affected NAION eyes. Choroidal thickness was thinnest in the inferior quadrant in all eyes regardless of the group. CONCLUSIONS: Increased peripapillary choroidal thickness was noted in both NAION and contralateral eyes. The thicker choroid may be an associated feature or a result of the disorder. Although further longitudinal study is required to determine causation, these findings may suggest that a thickened peripapillary choroid may be a component of the disk-at-risk clinical phenotype.

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.

Second chances.

An 81-year-old woman presented with light perception vision in her left eye and had pallid swelling of the left optic disk. Temporal artery biopsy was normal. Magnetic resonance imaging revealed enlargement and enhancement of the left orbital optic nerve. There were vitreous cells, but a vitrectomy specimen showed only a benign lymphocytic population. Her vision deteriorated to no light perception in the left eye, and an optic nerve biopsy revealed a diffuse astrocytoma of World Health Organization grade II.

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.

Differentiating retinal from optic nerve syndromes.

PURPOSE OF REVIEW: Differentiate between optic nerve and retinal disease that share common characteristics utilizing clinical history, examination, and ancillary testing. RECENT FINDINGS: Autofluorescence is a quick and noninvasive test that is helpful in detecting macular edema and deposition of lipofuscin. Optical coherence tomography (OCT) is becoming an indispensable tool in detecting subtle macular abnormalities that can be missed on funduscopic examination. Multifocal electroretinogram can also help in early screening of focal macular dystrophy. SUMMARY: Many patients present to the ophthalmology clinic with decreased vision, and occasionally it can be difficult to find a clear cause to the vision loss. Such situations, not infrequently, require the ophthalmologist to differentiate between optic nerve and retinal diseases that can share some common characteristics. The ability to use the clinical history and examination along with additional ancillary testing including OCT, electrophysiology, and fluorescein angiography to differentiate optic nerve from retinal disease can be very helpful in formulating a differential and ultimately establishing a diagnosis.