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Background: 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. Methods: Retrospective case series of patients with monocular pendular nystagmus seen in 2 tertiary neuroophthalmology clinics between January 2019 and June 2022. All patients underwent a complete neuroophthalmological assessment and MRI. Results: 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. Conclusions: 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. [ABSTRACT FROM AUTHOR]

Background: The clinical features of maculopathies and optic neuropathies often overlap: Both present with decreased visual acuity and variable loss of color vision; thus, maculopathy can be misdiagnosed as optic neuropathy, leading to patient harm. We aimed to determine what findings and/or tests were most helpful in differentiating between optic neuropathy and maculopathy. Methods: A retrospective chart review of consecutive patients over 4.5 years who were referred to neuroophthalmology clinics with the diagnosis of optic neuropathy but whose final diagnosis was maculopathy. Patient demographics, mode of presentation, clinical profile, complete ophthalmological examination, results of all ancillary testing, and final diagnosis were recorded. Results: A total of 47 patients (27 women) were included. The median age was 55 years (range, 18--85). Most referrals were by ophthalmologists (72.3%) and optometrists (12.8%). The diagnosis of maculopathy was made in 51.1% of patients at the time of first neuro-ophthalmic consultation. Only 6.4% patients (3) had relative afferent pupillary defect. Benign disc anomalies (tilted, myopic, small, or anomalous discs) were present in 34.0%, and 21.3% had pathologic disc changes unrelated or secondary to maculopathy. Macular ocular coherence tomography (OCT) was abnormal in 84.4% (with outer retinal pathology in 42.2% and inner retina pathology in 17.8%). Retinal nerve fiber layer (RNFL) thickness was normal in 82.6% of patients. Conclusions: Macular OCT is a high-yield test in differentiating between optic neuropathy and maculopathy and should be obtained in patients with suspected optic neuropathies who have normal RNFL thickness. Macular dystrophies, particularly cone dystrophies, unspecified retinal disorders, and macular degeneration were the most common mimics of optic neuropathy. The diagnosis was often present on OCT of the macula. The presence of coexistent benign and pathological disc anomalies may lead to maculopathy being misdiagnosed as optic neuropathy. [ABSTRACT FROM AUTHOR]

Supplemental Digital Content is Available in the Text. Background: Person-first language (PFL) is a linguistic prescription, which places a person before their disease. It is considered an important tool to reduce stigma. However, PFL is not routinely used across the scientific literature, particularly in patients with overweight or obesity. Patients with idiopathic intracranial hypertension (IIH) face various stigmas through high rates of poverty, female gender, and frequent rates of comorbidities. Non-PFL language use intersects and worsen the health inequities faced by these patients. Methods: A systematic review of case reports. MEDLINE and EMBASE were searched for all case reports with "pseudotumor cerebri" [MESH] OR "Idiopathic Intracranial Hypertension" as key word between January 1974 and August 2022. The primary criterion was the article's inclusion of patients with overweight or obesity. The secondary criterion was the article's discussion regarding obesity as risk factor. Articles not meeting primary or secondary criteria were excluded. Results: Approximately 514/716 (71.8%) articles used non-PFL language. The publication year was predictive of non-PFL language: 1976–1991 (82.3%) vs 1992–2007 (72.3%, P = 0.0394) and 2008–2022 (68.3%, P = 0.0056). Non-PFL was significantly higher in obesity compared with other medical conditions (60.3% vs 7.3%, P < 0.001). The patient gender (P = 0.111) and ethnicity (P = 0.697), author's specialty (P = 0.298), and primary English-speaking status (P = 0.231), as well as the journal's impact factor (P = 0.795), were not predictive of non-PFL. Conclusions: Most literature focused on IIH use non-PFL when discussing overweight or obesity, regardless of the patient's gender and ethnicity, journal's impact factor, senior author's specialty, and English-speaking status. Non-PFL use is much more common when discussing obesity compared with other medical conditions. Appropriate use of PFL can decrease stigma and, more importantly, decrease the intersectionality of health stigma faced by patients with IIH. [ABSTRACT FROM AUTHOR]

Background: Papilledema must be managed distinctly from other causes of optic disc edema (ODE) due to its basis in raised intracranial pressure (ICP). However, evidence indicates that the term "papilledema" is widely misused across specialties to describe ODE without raised ICP. Sources of this misconception remain undiscerned. Because all physicians consult medical databases, our objective was to evaluate whether nonspecific "papilledema" subject heading definitions misleadingly associate articles on other conditions with papilledema proper. Methods: Systematic review of case reports, prospectively registered on PROSPERO (CRD42022363651). MEDLINE and Embase were searched to July 2022 for any full-length case report indexed to the "papilledema" subject heading. Studies were graded for incorrect indexing, defined as cases lacking evidence for raised ICP. Nonpapilledema diagnoses were assigned to a predefined set of diseases and pathophysiological mechanisms for subsequent comparison. Results: Incorrect indexing occurred in 40.67% of 949 included reports. Embase-derived studies were misindexed significantly less than MEDLINE-derived studies (P, 0.01). There was also significant heterogeneity in incorrect indexing among specific diseases (P = 0.0015) and mechanisms (P = 0.0003). The most commonly misindexed diseases were uveitis (21.24% of errors), optic neuritis (13.47%), and instances with no mention of ODE (13.99%). The most commonly misindexed mechanisms were inflammation (34.97%), other mechanism (e.g., genetic; 25.91%), and ischemia (20.47%). Conclusions: Database subject headings, especially from MEDLINE, do not adequately distinguish between true papilledema and other causes of ODE. Inflammatory diseases were most often incorrectly indexed among other diseases and mechanisms. Current "papilledema" subject headings should be revised to reduce the probability of misinformation. [ABSTRACT FROM AUTHOR]

Background: Lumbar puncture (LP) is considered an essential component of the diagnosis of idiopathic intracranial hypertension (IIH) and ruling out IIH mimics, such as meningeal inflammation and neoplastic disease. Such mimics are unlikely in patients who are systemically well and fit the clinical demographic of IIH. It is important to take into account the risks of performing a LP as patients commonly experience mild adverse effects and infrequently more serious ones including psychological distress. LP can also be difficult to obtain in some health care settings, requiring inpatient admission. We examined the clinical course of a subset of presumed patients with IIH with mild vision loss and papilledema to determine whether LP can be safely deferred in this group. Methods: This was a retrospective study looking at the clinical characteristics, final visual outcome, and diagnosis of patients with presumed IIH and papilledema determined by clinical examination who did not undergo LP. The inclusion criteria included i) no symptoms suspicious for systemic infectious/neoplastic/inflammatory processes, ii) no secondary causes of raised intracranial pressure seen on magnetic resonance imaging/magnetic resonance venography, iii) optical coherence tomography (OCT)-RNFL thickness ≤300 µm, and iv) automated mean deviation (MD) ≤ −5.00 dB v) at least one follow-up visit. Results: A total of 132 eyes of 68 patients (66 female and 2 male) were included in the study. The mean ± SD age was 31.4 ± 10.2 years, and body mass index was 35.1 ± 6.8 kg/m2. Systemic symptoms included headache (n = 47), pulsatile tinnitus (n = 28), transient visual obscurations (n = 10), and diplopia (n = 2). Presenting logarithm of the minimum angle of resolution visual acuity was 0.020 ± 0.090, automated MD was −2.23 ± 1.38 dB, and OCT RNFL thickness was 150.8 ± 48.4 µm. Patients were followed for a mean number of 63.3 ± 78.3 weeks. No additional cause of intracranial hypertension was discovered, and all patients remained systemically well. Two patients were started on acetazolamide, and 31 patients lost at least some weight. There was a significant improvement in the automated MD (−1.73 ± 1.74 dB; P < 0.001) and OCT RNFL thickness (128.1 ± 38.6 µm; P < 0.001) at final follow-up. Seventy-six eyes of 38 patients were considered to have resolved papilledema at the final follow-up. Conclusions: Some patients with presumed IIH may not be able to undergo LP because of patient factors such as refusal, failed attempts, or the environment in which neuro-ophthalmologists practice. This study suggests that it may be acceptable to defer LP for patients with suspected IIH who are under the care of a neuro-ophthalmologist with experience in diagnosing and managing IIH. These patients should be systemically well, in a typical demographic for IIH patients, have mild optic disc edema, and preserved visual function. Patients should be informed about the controversial nature of this decision. [ABSTRACT FROM AUTHOR]

Supplemental Digital Content is Available in the Text. [ABSTRACT FROM AUTHOR]