Trends and Disparities in the Utilization of Thymectomy for Myasthenia Gravis in the United States.

Background and Objectives: In 2016, a randomized controlled trial demonstrated the clinical efficacy of trans-sternal thymectomy for patients with non-thymomatous myasthenia gravis (MG). Whether large-scale changes occurred in clinical practice after this trial is unknown. Methods: We performed a retrospective longitudinal cross-sectional analysis using National Inpatient Sample (NIS) data from 2012 to 2019. Our study included hospitalized adults at least 18 years of age diagnosed with MG without an associated thymoma. We used joinpoint regression to analyze annual trends in thymectomy volume and surgical approach (minimally invasive vs trans-sternal) from 2012 to 2019. Using logistic regression models, we examined patient and hospital-level factors that may have influenced whether thymectomy was performed, such as age, sex, race, insurance payor, hospital size and teaching status, and Elixhauser Comorbidity Index. Sampling weights were applied to account for the complex survey design of NIS. Results: The total number of thymectomy procedures increased by 69.8% per year (95% CI 40.1-105.8) between 2012 and 2019. Trans-sternal thymectomies increased by 62.8% per year (95% CI 35.8-95.2) and minimally invasive thymectomies by 83.7% per year (95% CI 38.1-144.3). Thymectomies were significantly more likely to occur in 2017-2019 compared with 2012-2016 (OR 1.93, 95% CI 1.62-2.31). In a multivariable regression model, several factors decreased the odds of patients with MG having a thymectomy: older age, Black race (OR 0.62, 95% CI 0.49-0.77), female (OR 0.73, 95% CI 0.63-0.86), and higher Elixhauser Comorbidity Index. Patients in medium (OR 1.82, 95% CI 1.30-2.55) or large (OR 2.81, 95% CI 2.07-3.82) size and urban teaching hospitals (OR 6.09, 95% CI 2.65-13.97) were more likely to undergo thymectomy. Discussion: Thymectomy is being performed more frequently for non-thymomatous MG, especially after 2016 after publication of a positive phase III clinical trial. There are several disparities in thymectomy utilization that warrant further attention.

Social Determinants of Health in Idiopathic Intracranial Hypertension.

BACKGROUND: Idiopathic intracranial hypertension (IIH) disproportionately affects women from socioeconomically disadvantaged communities, but specific social determinants of health have not been examined. METHODS: We used data from the National Institutes of Health's All of Us Research Program, an ongoing nationwide study of more than 300,000 diverse individuals in the United States. Height and weight were measured at baseline, and participants completed questionnaires about demographics, health care access, and quality of life. Women aged 18-50 years with IIH were identified through electronic health record data, excluding those with venous thrombosis, meningitis, hydrocephalus, or central nervous system neoplasms. We used logistic regression to compare questionnaire responses for IIH cases and controls, adjusting for age, race, ethnicity, annual income, and body mass index (BMI). RESULTS: We included 416 women with IIH and 107,111 women without IIH. The mean age was 38 years, and 49.3% identified as non-White. After adjusting for age, race/ethnicity, and BMI, women with IIH were more likely to be unemployed (odds ratio [OR] 1.40, 95% confidential interval [CI]: 1.14-1.71) and report delaying care because of difficulty affording copays (OR 1.47, 95% CI: 1.02-2.10) or specialist care (OR 1.52, 95% CI: 1.06-2.18). They also delayed care because of rural residence (OR 2.08, 95% CI: 1.25-3.47) and transportation limitations (OR 2.23, 95% CI: 1.55-3.20). Although women with IIH were more likely to be non-Hispanic Black (OR 1.66, 95% CI: 1.32-2.09), this association lost significance when controlling for BMI and income (OR 1.27, 95% CI: 0.96-1.68). CONCLUSIONS: Women with IIH experience adverse social determinants of health beyond those associated with obesity alone.

Cataract surgery utilization in Parkinson's disease: the english longitudinal survey on ageing.

PURPOSE: Older adults with Alzheimer's disease are less likely to be offered cataract surgery than the general population, but these disparities have not been examined in the context of other neurodegenerative disorders such as Parkinson's disease (PD). METHODS: Using data from the English Longitudinal Study on Ageing (ELSA), an ongoing, longitudinal population-based survey of older adults in England, we examined the relationship between PD and cataract surgery among adults 50 and older. We used logistic regression to measure the association between PD and history of cataract surgery at baseline. In longitudinal analyses of subjects with no history of cataract surgery at time of enrollment, we used semiparametric, discrete-time proportional hazards models to model the incidence of cataract surgery as a function of PD and other time-dependent covariates. Models were adjusted for demographic variables, self-reported comorbidities, and measures of daily activity limitation. RESULTS: We included data from 19,241 eligible ELSA respondents, of whom 231 (1.2%) reported a diagnosis of PD. PD was positively associated with a history of self-reported cataract surgery at baseline (OR 3.66, 95% CI: 2.55-5.26), but this did not remain significant after adjusting for confounders (OR 1.22, 95% CI: 0.75-1.98). Among subjects with no history of cataract surgery at baseline, PD was also not associated with incident cataract surgery (adjusted HR 1.32, 95% CI: 0.86-2.02). CONCLUSION: Unlike Alzheimer's disease, people with PD were no less likely to receive cataract surgery compared to those without PD.

Attitudes and Beliefs Toward Thymectomy in the Myasthenia Gravis Patient Registry.

OBJECTIVES: To evaluate patient attitudes and beliefs toward thymectomy for myasthenia gravis (MG). METHODS: The Myasthenia Gravis Foundation of America administered a questionnaire to the MG Patient Registry, an ongoing longitudinal survey of adult MG patients. Questions assessed reasons for or against thymectomy and how hypothetical scenarios would have affected their decision. RESULTS: Of 621 respondents, 190 (31%) reported a history of thymectomy. Of those who underwent thymectomy for nonthymomatous MG, 97 (51.6%) ranked symptom improvement as most important and 100 (53.2%) ranked reducing medication as least important. Among 431 nonthymectomy patients, the most frequent reason for not undergoing thymectomy was that their doctor did not discuss it (152 of 431 = 35.2%) and 235 (56.8%) said that they would have considered it more strongly if their doctor spent more time discussing it. CONCLUSIONS: Thymectomies are motivated more by symptoms than by medication, and a lack of neurologist discussion is the most common barrier to thymectomy.

Frequency and Etiologies of Visual Disturbance After Cataract Surgery Identified in Neuro-Ophthalmology Clinics.

BACKGROUND: To identify the frequency and etiologies of visual disturbances after cataract surgery in patients referred to Neuro-ophthalmology. METHODS: This study is a retrospective chart review. Records of patients 18 years and older referred to neuro-ophthalmology clinics for new-onset visual disturbances within 6 months of cataract surgery were reviewed. Those with pre-existing neuro-ophthalmic disorders, combined intraocular procedures with cataract surgery, or inadequate follow-up were excluded. The main outcome measures were frequency and etiologies of visual disturbances after cataract surgery. Secondary analyses of a cohort of patients who had cataract surgery at our institution were performed to determine the frequency and etiology of visual disturbances after uneventful cataract surgery. RESULTS: One hundred seventy-three patients met the inclusion criteria (internal referral: 36/173, from outside surgeons: 137/173). Sixty-one percent (106/173) were newly diagnosed with neuro-ophthalmic etiologies, including 21% (36/173) with afferent and 40% (70/173) with efferent disorders. Thirty-six percent (62/173) of patients had non neuro-ophthalmic causes and 3% (5/173) had systemic conditions responsible for visual disturbances postoperatively. Decompensated strabismus causing diplopia was the most common neuro-ophthalmic diagnosis after cataract surgery (50%, 53/106). Of the 13,715 patients who had cataract surgery performed at our institution over a 9-year period, 20 of 36 patients referred for visual disturbances were identified with neuro-ophthalmic etiologies of which 85% (17/20) had postoperative diplopia. CONCLUSIONS: In our study, decompensated strabismus causing diplopia was the most common neuro-ophthalmic visual disturbance after cataract surgery. Detailed history and ocular alignment should be assessed before cataract surgery to identify patients with the risk.

A Validated Method to Identify Neuro-Ophthalmologists in a Large Administrative Claims Database.

BACKGROUND: Validated methods to identify neuro-ophthalmologists in administrative data do not exist. The development of such method will facilitate research on the quality of neuro-ophthalmic care and health care utilization for patients with neuro-ophthalmic conditions in the United States. METHODS: Using nationally representative, 20% sample from Medicare carrier files from 2018, we identified all neurologists and ophthalmologists billing at least 1 office-based evaluation and management (E/M) outpatient visit claim in 2018. To isolate neuro-ophthalmologists, the National Provider Identifier numbers of neuro-ophthalmologists in the North American Neuro-Ophthalmology Society (NANOS) directory were collected and linked to Medicare files. The proportion of E/M visits with International Classification of Diseases-10 diagnosis codes that best distinguished neuro-ophthalmic care ("neuro-ophthalmology-specific codes" or NSC) was calculated for each physician. Multiple logistic regression models assessed predictors of neuro-ophthalmology specialty designation after accounting for proportion of ophthalmology, neurology, and NSC claims and primary specialty designation. Sensitivity, specificity, and positive predictive value (PPV) for varying proportions of E/M visits with NSC were calculated. RESULTS: We identified 32,293 neurologists and ophthalmologists who billed at least 1 outpatient E/M visit claim in 2018 in Medicare. Of the 472 NANOS members with a valid individual National Provider Identifier, 399 (84.5%) had a Medicare outpatient E/M visit in 2018. The model containing only the proportion of E/M visits with NSC best predicted neuro-ophthalmology specialty designation (odds ratio 1.05 [95% confidence interval 1.04, 1.05]; P < 0.001; area under the receiver operating characteristic [AUROC] = 0.91). Model predictiveness for neuro-ophthalmology designation was maximized when 6% of all billed claims were for NSC (AUROC = 0.89; sensitivity: 84.0%; specificity: 93.9%), but PPV was low (14.9%). The threshold was unchanged when limited only to neurologists billing ≥1% ophthalmology claims or ophthalmologists billing ≥1% neurology claims, but PPV increased (33.3%). CONCLUSIONS: Our study provides a validated method to identify neuro-ophthalmologists who can be further adapted for use in other administrative databases to facilitate future research of neuro-ophthalmic care delivery in the United States.

TRIANGULAR SIGN OF AMALRIC IN INTRAVASCULAR LYMPHOMA.

PURPOSE AND METHODS: To report a case of bilateral ocular ischemia caused by intravascular lymphoma with the presence of bilateral triangular sign of Amalric. RESULTS: A 67-year-old woman was admitted to our hospital for progressive paraplegia and a 6-month history of acute painless vision loss in the right eye. Initial examination showed vision of counting fingers in the right eye and 20/20 in the left eye with normal anterior examination. Fundus examination revealed a likely previous central retinal artery occlusion in the right eye with pale nerve and attenuated vessels. Both fundi had triangular regions of pigmentary change known as the triangular sign of Amalric, indicative of choroidal ischemia. However, the left eye was asymptomatic. Neuroimaging revealed multifocal enhancing lesions throughout the central nervous system of unclear etiology. An extensive neurologic and systemic workup was unrevealing, including a brain biopsy, and empiric treatment for an unspecified inflammatory condition with intravenous corticosteroids was initiated. During her hospitalization, she developed acute painless vision loss in the left eye, and examination showed no light perception vision in both eyes with signs of acute retinal and choroidal ischemia in the left eye. A subsequent brain biopsy revealed intravascular lymphoma. CONCLUSION: Triangular pigmentary changes indicate choroidal ischemia and can be seen in many conditions. This patient presented with the triangular sign of Amalric in both eyes, including her asymptomatic left eye. Intravascular lymphoma should be considered in cases of concomitant inflammatory brain lesions and chorioretinal ischemia.

Socioeconomic and Geographic Disparities in Idiopathic Intracranial Hypertension.

OBJECTIVE: To identify relationships between idiopathic intracranial hypertension (IIH) and socioeconomic determinants of health, such as low-income status and proximity to healthy food. METHODS: This retrospective case-control study of adult female neuro-ophthalmology patients from one institution identified 223 women with and 4,783 women without IIH. Street addresses were geocoded and merged with US census data to obtain census tract-level information on income and food access. Choropleth maps visualized IIH clusters within certain neighborhoods. Logistic regression compared the proportion of patients with IIH from racial and ethnic minority backgrounds, low-income census tracts, and food deserts and swamps to controls without IIH. RESULTS: In our cohort, when adjusted for age, women with IIH were more likely to be Black (odds ratio [OR] 3.96, 95% confidence interval [CI] 2.98-5.25), Hispanic (OR 2.23, 95% CI 1.14-4.36), and live in low-income tracts (OR 2.24, 95% CI 1.71-2.95) or food swamps (OR 1.54, 95% CI 1.15-2.07). Patients with IIH were less likely to live in food deserts than controls (OR 0.61, 95% CI 0.45-0.83). The association between Black race and IIH remained significant even after adjusting for other variables. CONCLUSION: IIH is more common among Black and Hispanic women than expected even when accounting for the demographics of a metropolitan city. Some of this relationship is driven by the association of obesity and IIH incidence with low income and proximity to unhealthy foods.

Pharmacosafety of fluoroquinolone and macrolide antibiotics in the clinical care of patients with myasthenia gravis.

INTRODUCTION/AIMS: Anecdotal case reports have suggested a potential association of fluoroquinolones and macrolides with myasthenia gravis (MG) exacerbation, prompting warnings against the use of these drugs in this population. However, large-scale and reliable population-based data that demonstrate this association are lacking. This study aims to examine the association between outpatient treatment with fluoroquinolones or macrolides and MG-related hospitalization. METHODS: A retrospective cohort study consisting of adult MG patients was conducted using a large de-identified healthcare claims database. Antibiotic prescription claims were identified, and MG-related hospitalizations were assessed at 15, 30, and 90 days after the date of prescription. We used mixed effects survival regression with log-logistic distribution and independent covariance matrix to estimate odds ratios (ORs) of hospitalization for each potentially exacerbating antibiotic using beta-lactam as the reference and adjusting for covariates. RESULTS: Among 1556 MG patients receiving 894 fluoroquinolone prescriptions, 729 macrolide prescriptions, and 1608 beta-lactam prescriptions during the study period, there was no difference in 15, 30, or 90-day odds of MG-related hospitalization between fluoroquinolone or macrolide users compared to prescribed beta-lactams. However, estimates were higher for fluoroquinolones than macrolides, even after covariate adjustment (adjusted OR [aOR] 4.60, 95% confidence interval [CI] 0.55-38.57 for fluoroquinolones and OR 0.56, 95% CI 0.32-0.97 for macrolides, respectively, at 15 days). DISCUSSION: Fluoroquinolone and macrolide antibiotics are prescribed frequently to patients with MG. While statistical imprecision precludes a definitive conclusion, elevated ORs for fluoroquinolones raise the possibility of an underpowered association that merits further investigation.

Vision loss and hallucinations: perspectives from neurology and ophthalmology.

PURPOSE OF REVIEW: The aim of this article is to summarize the evidence for visual impairment as a risk factor for visual hallucinations in neurologic disease and recent advances in our understanding of the central visual pathways that mediate this association. RECENT FINDINGS: Recent studies have described the prevalence Charles Bonnet syndrome and questioned its lack of association with cognitive impairment, used advanced neuroimaging to show that disinhibition of the occipital lobe is involved in the pathogenesis of visual hallucinations in Parkinson's disease, and demonstrated that visual impairment because of eye disease is a consistent risk factor for visual hallucinations across a number of different neurodegenerative disease populations. SUMMARY: Through connections between the primary visual cortex and other brain structures, visual function is closely tied to visual hallucinations. Given that the vast majority of vision loss is caused by ophthalmic disease, much of which is preventable or treatable, the detection and treatment of vision loss in at-risk populations may reduce the burden and consequences of visual hallucinations in older adults.

Trends in the Surgical Treatment of Pseudotumor Cerebri Syndrome in the United States.

Importance: Optic nerve sheath fenestration (ONSF) and cerebrospinal fluid shunting are sometimes used to treat pseudotumor cerebri syndrome (PTCS), but their use patterns are unknown. Objectives: To investigate the frequency of surgical PTCS treatment in the United States and to compare patients undergoing ONSF with those treated with shunting. Design, Setting, and Participants: This was a retrospective longitudinal cross-sectional study. Inpatient data were obtained from the National Inpatient Sample (NIS), and outpatient surgical center data were obtained from the National Survey of Ambulatory Surgery (NSAS) and National Hospital Ambulatory Medical Care Survey (NHAMCS). Included in the analysis were 10 720 patients aged 18 to 65 years with a diagnosis code for PTCS, excluding venous thrombosis and other causes of intracranial hypertension. Time trends were explored and logistic regression was used to measure differences according to age, race/ethnicity, sex, Elixhauser comorbidity index, and other patient and hospital characteristics. Data analysis was performed from March 31 to October 7, 2020. Exposure: Treatment for PTCS, excluding venous thrombosis and other causes of intracranial hypertension. Main Outcomes and Measures: Annual number of PTCS-related admissions, ONSFs, and shunt procedures from 2002-2016. Patient and hospital-level characteristics of patients with PTCS undergoing ONSF or shunting were compared. Results: Between 2010 and 2016, 297 ONSFs were performed and 10 423 shunts were placed as treatment for PTCS. The procedures were most commonly performed in individuals aged 26 to 35 years (39.4%), and 9920 (92.4%) of the surgically treated patients were women. ONSF was more common among younger patients (eg, adjusted odds ratio [AOR] for patients ≥46 years vs those 18-25 years, 0.22; 95% CI, 0.08-0.61) and in Black, Hispanic, or other minority populations (AOR, 2.37; 95% CI, 1.31-4.30) and less common in the South (AOR, 0.34; 95% CI, 0.13-0.88) and West (AOR, 0.15; 95% CI, 0.04-0.58) compared with the Northeast. Total PTCS-related hospitalizations increased from 6081 (95% CI, 5137-7025) in 2002 to 18 020 (95% CI, 16 607-19 433) in 2016. Shunting increased from 2002 to 2011 and subsequently plateaued and declined. ONSF was used much less frequently, and use has not increased. No instances of outpatient ONSF or shunting for PTCS were recorded in the NSAS or NHAMCS databases. Conclusions and Relevance: This study's findings suggest that shunting is more common than ONSF and that the use gap has widened as shunting has increased. However, because overall PTCS-related hospitalizations have increased even more rapidly, the percentage of inpatients with PTCS undergoing surgery has decreased. These trends may reflect changes in medical treatment practices and outcomes or growing limitations in access to ophthalmic surgical expertise.

Validity of International Classification of Diseases Codes for Identifying Neuro-Ophthalmic Disease in Large Data Sets: A Systematic Review.

BACKGROUND: Administrative health claims data have been used for research in neuro-ophthalmology, but the validity of International Classification of Diseases (ICD) codes for identifying neuro-ophthalmic conditions is unclear. EVIDENCE ACQUISITION: We performed a systematic literature review to assess the validity of administrative claims data for identifying patients with neuro-ophthalmic disorders. Two reviewers independently reviewed all eligible full-length articles and used a standardized abstraction form to identify ICD code-based definitions for 9 neuro-ophthalmic conditions and their sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). A quality assessment of eligible studies was also performed. RESULTS: Eleven articles that met criteria for inclusion are as follows: 3 studies of idiopathic intracranial hypertension (PPV 54%-91% and NPV 74%-85%), 2 studies of giant cell arteritis (sensitivity 30%-96% and PPV 94%), 3 studies of optic neuritis (sensitivity 76%-99%, specificity 83%-100%, PPV 25%-100%, and NPV 98%-100%), 1 study of neuromyelitis optica (sensitivity 60%, specificity 100%, PPV 43%-100%, and NPV 98%-100%), 1 study of ocular motor cranial neuropathies (PPV 98%-99%), and 2 studies of myasthenia gravis (sensitivity 53%-97%, specificity 99%-100%, PPV 5%-90%, and NPV 100%). No studies met eligibility criteria for nonarteritic ischemic optic neuropathy, thyroid eye disease, and blepharospasm. Approximately 45.5% provided only one measure of diagnostic accuracy. Complete information about the validation cohorts, inclusion/exclusion criteria, data collection methods, and expertise of those reviewing charts for diagnostic accuracy was missing in 90.9%, 72.7%, 81.8%, and 36.4% of studies, respectively. CONCLUSIONS: Few studies have reported the validity of ICD codes for neuro-ophthalmic conditions. The range of diagnostic accuracy for some disorders and study quality varied widely. This should be taken into consideration when interpreting studies of neuro-ophthalmic conditions using administrative claims data.

Self-reported vision and hallucinations in older adults: results from two longitudinal US health surveys.

BACKGROUND: Vision loss may be a risk factor for hallucinations, but this has not been studied at the population level. METHODS: To determine the association between self-reported vision loss and hallucinations in a large community-based sample of older adults, we performed a cross-sectional and longitudinal analysis of two large, nationally representative US health surveys: the National Health and Aging Trends Study (NHATS) and the Health and Retirement Study (HRS). Visual impairment and hallucinations were self- or proxy-reported. Multivariate single and mixed effects logistic regression models were built to examine whether visual impairment and history of cataract surgery were associated with hallucinations. RESULTS: In NHATS (n = 1520), hallucinations were more prevalent in those who reported difficulty reading newspaper print (OR 1.77, 95% confidence interval (CI): 1.32-2.39) or recognising someone across the street (OR 2.48, 95% CI: 1.86-3.31) after adjusting for confounders. In HRS (n = 3682), a similar association was observed for overall (OR 1.32, 95% CI: 1.08-1.60), distance (OR 1.61, 95% CI: 1.32-1.96) and near eyesight difficulties (OR 1.52, 95% CI: 1.25-1.85). In neither sample was there a significant association between cataract surgery and hallucinations after adjusting for covariates. CONCLUSIONS: Visual dysfunction is associated with increased odds of hallucinations in the older US adult population. This suggests that the prevention and treatment of vision loss may potentially reduce the prevalence of hallucinations in older adults.

Outcomes After Transcervical Thymectomy for Ocular Myasthenia Gravis: A Retrospective Cohort Study With Inverse Probability Weighting.

BACKGROUND: The benefit of thymectomy in reducing requirement for corticosteroids, symptom severity, need for immunosuppression, and hospitalization rates in patients with seropositive generalized myasthenia has recently been established. It is unclear whether this benefit applies to patients with myasthenia and purely ocular manifestations (ocular myasthenia gravis [OMG]). METHODS: We conducted a retrospective single-center cohort study of patients with OMG. Patients were included if their diagnosis was confirmed by acetylcholine receptor or muscle-specific kinase antibodies, abnormal electrophysiology, or a positive edrophonium test and at least 1 year of clinical follow-up. At each visit, the presence and severity of ocular and generalized symptoms was ascertained using a 4-point scale. Prednisone dose, steroid-sparing agent use, and need for intravenous immunoglobulin or plasmapheresis were recorded. The effect of thymectomy on time-weighted prednisone dose and symptom severity score was assessed using linear regression models. To adjust for nonrandomization of thymectomy, we used inverse probability weighting using a propensity score model derived from the prethymectomy observation period for thymectomy patients and a 6-month lead-in period for nonthymectomy patients that incorporated age, sex, acetylcholine receptor antibody seropositivity, disease severity (as defined by both symptom severity and treatment requirement), and treating physician preferences. RESULTS: Eighty-two patients (30 with thymectomy and 52 nonthymectomy) were included. In unadjusted analyses, time-weighted daily prednisone dose was 2.9 mg higher with thymectomy compared with nonthymectomy (95% CI: 0.2-5.7), but after inverse probability weighting, this was no longer statistically significant (difference = 1.7 mg, 95% CI: -0.8 to 4.2). There was no statistically significant difference in symptom severity score (adjusted difference = 0.35, 95% CI: -0.02 to 0.72) or risk of generalization (P = 0.22). CONCLUSIONS: In this retrospective study that used statistical techniques to account for nonrandomization, no significant differences in prednisone dose or symptom severity after thymectomy in ocular myasthenia were demonstrated.

Headache Characteristics in Children With Pseudotumor Cerebri Syndrome, Elevated Opening Pressure Without Papilledema, and Normal Opening Pressure: A Retrospective Cohort Study.

BACKGROUND: Certain headache characteristics and associated symptoms are commonly attributed to increased intracranial pressure, but they have not been systematically studied among children in the context of revised diagnostic criteria for pseudotumor cerebri syndrome (PTCS). METHODS: We performed a retrospective cohort study of patients treated for suspected or confirmed PTCS. Charts were reviewed for PTCS and headache diagnostic criteria and associated characteristics. Chi-squared or Fisher's exact tests were used to compare the frequency of headache characteristics between groups. RESULTS: One hundred and twenty-seven individuals were identified: 61 had definite PTCS, 10 had probable PTCS, 31 had elevated opening pressure (OP) without papilledema, and 25 had normal OP without papilledema. Eleven children had no headache (6 with definite PTCS, 5 with probable PTCS). Headache pattern was episodic in 49% (95% CI: 34-64%) of those with definite PTCS, 18% (95% CI 6-37%) of those with elevated OP without papilledema, and 16% (5-36%) of those with normal OP without papilledema. Headache location was more likely to involve the head along with neck or shoulders in those with definite PTCS compared with elevated OP without papilledema (OR = 7.2, 95% CI: 1.9-27.6) and normal OP (OR = 4.5, 95% CI: 1.3-15.6) groups. DISCUSSION: While missing data and small cohort size are limitations, this study suggests that headache in PTCS is more likely to involve the head along with neck/shoulders, and that headache in PTCS may be episodic or constant. Headache is occasionally absent in PTCS.