Natural History of Neuronal Ceroid Lipofuscinosis Type 6, Late Infantile Disease.

BACKGROUND: Mutations in the CLN6 gene cause late infantile neuronal ceroid lipofuscinosis, a neurodegenerative lysosomal storage disease of childhood onset. Clinically, individuals present with progressive motor and cognitive regression, ataxia, and early death. The aim of this study is to establish natural history data of individuals with classic, late-infantile-onset (age less than five years) CLN6 disease. METHODS: We analyzed the natural history of 25 patients with late-infantile-onset CLN6, utilizing the Hamburg motor-language scale to measure disease progression. The key outcomes were CLN6 disease progression, assessed by rate of decline in motor and language clinical domain summary scores (0 to 6 total points); onset and type of first symptom; onset of first seizure; and time from first symptom to complete loss of function. RESULTS: Median age of total motor and language onset of decline was 42 months (interquartile range 36 to 48). The estimated rate of decline in total score was at a slope of -1.20 (S.D. 0.30) per year, after the start of decline. Complete loss of both motor and language function was found to be, on average, 88.1 months (S.D. 13.5). CONCLUSIONS: To our knowledge, this is the largest international study that monitors the longitudinal natural history and progression of CLN6 disease. These data may serve as a template for future interventional trials targeted to slow the progression of this devastating disease.

Persistent Elevation of Opening Pressure Despite Treatment and Symptom Resolution in Pediatric Intracranial Hypertension.

BACKGROUND: Currently no guidelines for repeating a lumbar puncture to guide management in primary intracranial hypertension (PIH) exist. METHODS: An institutional database of patients 18 years and younger followed in the institution's pediatric intracranial hypertension clinic was examined for opening pressure changes in PIH at diagnosis, before medication wean, and following medication wean, as well as to examine whether measurements at the time of diagnosis differed between those with and without disease recurrence. RESULTS: Forty-two patients were included in this study; 36% were male and the mean age at diagnosis was 11.01 years. Treatment duration averaged 9.68 months in those without recurrence and 8.5 months in those with recurrence. Average body mass index percentile of patients with disease recurrence was 83.7 and 72.1 in those without recurrence (P = 0.16). Average opening pressure values of all patients at diagnosis, prewean, and postwean was 36.53 cm H2O, 30.7 cm H2O, and 31.1 cm H2O, respectively. There was no statistically significant difference in opening pressures across these time points (P = 0.14). The change in opening pressure from diagnosis to postwean was statistically significant with a reduction of 5.18 cm H2O (P = 0.04). There was no statistical difference between change in opening pressure at diagnosis versus postwean between those with and without recurrence (P = 0.17). CONCLUSIONS: This clinical observational study suggests that mean opening pressure measurements in patients with PIH remain elevated both before and after medication wean despite papilledema resolution and patient-reported PIH symptoms. Clinically, this suggests that other features such as signs of optic disc edema and symptoms should be used to inform a clinical determination of disease recurrence and treatment course.

Safety and efficacy of cerliponase alfa in children with neuronal ceroid lipofuscinosis type 2 (CLN2 disease): an open-label extension study.

BACKGROUND: Cerliponase alfa is a recombinant human tripeptidyl peptidase 1 (TPP1) enzyme replacement therapy for the treatment of neuronal ceroid lipofuscinosis type 2 (CLN2 disease), which is caused by mutations in the TPP1 gene. We aimed to determine the long-term safety and efficacy of intracerebroventricular cerliponase alfa in children with CLN2 disease. METHODS: This analysis includes cumulative data from a primary 48-week, single-arm, open-label, multicentre, dose-escalation study (NCT01907087) and the 240-week open-label extension with 6-month safety follow-up, conducted at five hospitals in Germany, Italy, the UK, and the USA. Children aged 3-16 years with CLN2 disease confirmed by genetic analysis and enzyme testing were eligible for inclusion. Treatment was intracerebroventricular infusion of 300 mg cerliponase alfa every 2 weeks. Historical controls with untreated CLN2 disease in the DEM-CHILD database were used as a comparator group. The primary efficacy outcome was time to an unreversed 2-point decline or score of 0 in the combined motor and language domains of the CLN2 Clinical Rating Scale. This extension study is registered with ClinicalTrials.gov, NCT02485899, and is complete. FINDINGS: Between Sept 13, 2013, and Dec 22, 2014, 24 participants were enrolled in the primary study (15 female and 9 male). Of those, 23 participants were enrolled in the extension study, conducted between Feb 2, 2015, and Dec 10, 2020, and received 300 mg cerliponase alfa for a mean of 272·1 (range 162·1-300·1) weeks. 17 participants completed the extension and six discontinued prematurely. Treated patients were significantly less likely than historical untreated controls to have an unreversed 2-point decline or score of 0 in the combined motor and language domains (hazard ratio 0·14, 95% CI 0·06 to 0·33; p<0·0001). All participants experienced at least one adverse event and 21 (88%) experienced a serious adverse event; nine participants experienced intracerebroventricular device-related infections, with nine events in six participants resulting in device replacement. There were no study discontinuations because of an adverse event and no deaths. INTERPRETATION: Cerliponase alfa over a mean treatment period of more than 5 years was seen to confer a clinically meaningful slowing of decline of motor and language function in children with CLN2 disease. Although our study does not have a contemporaneous control group, the results provide crucial insights into the effects of long-term treatment. FUNDING: BioMarin Pharmaceutical.

Effect of Inhaled Sevoflurane on Pediatric Cerebrospinal Fluid Pressure During Lumbar Puncture; Implications for Intracranial Hypertension.

Objective: To determine influence of sevoflurane on changes in cerebrospinal fluid pressure in children presenting for lumbar puncture. Methods: Cerebrospinal fluid pressure, end tidal carbon dioxide, and end tidal sevoflurane concentration measurements were obtained at 2-minute intervals for a total of 10 minutes (T0 to T5). Because of concerns regarding patient safety and comfort, the study measurements were completed at the end of the lumbar procedure, starting with the closing pressure and when sevoflurane was stopped. Results: As end tidal sevoflurane concentration decreased, cerebrospinal fluid pressure initially increased up to T2 before decreasing back to around the initial point. There was no significant correlation between sevoflurane level and cerebrospinal fluid pressure. Both weight status and presence or absence of optic edema did not have a significant impact on pressure over time. However, there was a statistically significant difference in the cerebrospinal fluid pressure over time between those with spontaneous respirations compared to those without. Conclusions: There was no significant correlation between the end tidal sevoflurane concentration and cerebrospinal fluid pressure. Assisted ventilation did produce a statistically significant increase in cerebrospinal fluid pressure and suggests that the most accurate measurements are in those with spontaneous respirations.

Yearly Occurrence and Seasonality of Neuro-ophthalmic Manifestations of Pediatric Lyme Disease.

PURPOSE: To review the neuro-ophthalmic manifestations of Lyme disease at a central Ohio pediatric tertiary care center. METHODS: A retrospective chart review of patients diagnosed as having Lyme disease from September 2015 to September 2020 was completed. Demographic information, diagnosis dates, and manifestations of Lyme disease were recorded. Patients were excluded for age older than 18 years or lack of corroborated Lyme disease diagnosis. Descriptive statistics were performed. RESULTS: Of the 212 cases of pediatric Lyme disease reviewed, 50 patients had neuroborreliosis. The data showed an increase in Lyme disease and neuroborreliosis cases from 2018 to 2020, with a preponderance of diagnoses in the summer months. Twenty-four patients had meningitis, and 6 of these patients (25%) were diagnosed as having bilateral optic disc edema that was clinically consistent with intracranial hypertension. CONCLUSIONS: Papilledema in the setting of Lyme meningitis may be more common than previously reported in central Ohio. If Lyme disease meningitis is suspected, an opening pressure should be recorded at the time of lumbar puncture and, if elevated, an ophthalmologic evaluation for optic nerve edema is indicated. [J Pediatr Ophthalmol Strabismus. 20XX;X(X):XXX-XXX.].

Secondary Intracranial Hypertension in Pediatric Lyme Meningitis.

Lyme disease is the most common vector-borne disease in the United States and has been associated with secondary intracranial hypertension. We reviewed 11 pediatric patients with Lyme-associated secondary intracranial hypertension. All patients presented with headache, ten had papilledema, 7 with a rash, and 5 with a cranial nerve palsy. All patients were treated with acetazolamide, and 3 received combination therapy with furosemide. Three patients were considered to have fulminant intracranial hypertension because of the severity in their presenting courses. Two of the fulminant intracranial hypertension patients were treated with a temporary lumbar drain in addition to medications, whereas 1 fulminant intracranial hypertension patient was treated exclusively with medical therapy alone. The addition of a lumbar drain decreased the time to resolution of papilledema compared to medical management alone. Final visual acuity was 20/20 in each eye of all patients, suggesting that a titrated approach to therapy depending on the severity of presentation can result in good visual outcomes in these cases. Additionally, symptoms can recur after medication wean, so patients should be monitored closely with any discontinuation of intracranial pressure lowering medications.

Experiences of Pain and Quality of Life in Pediatric Intracranial Hypertension.

OBJECTIVE: To examine psychosocial correlates, specifically pain intensity, pain interference, and quality of life (QOL), in patients with pediatric intracranial hypertension. We hypothesized that parents and children who report higher levels of pain intensity and interference would be associated with lower QOL. METHODS: Eighty-three patients and their families seen in the pediatric intracranial hypertension clinic of a large pediatric hospital completed self-report and parent proxy measures on QOL, mood, and other psychosocial correlates. Bivariate correlations were conducted between parent proxy and self-reports and regression analyses between pain and psychosocial correlate variables were examined. RESULTS: Parent proxy and child reports were highly correlated. Body mass index (BMI) was a significant predictor of parent proxy report of pain intensity with higher BMI associated with higher parent proxy ratings of pain intensity. Parents reported significantly higher levels of pain interference for their children compared to child report. Parent ratings of child depression symptoms, BMI, and age were significant predictors of pain interference. Higher BMI and depression symptoms and younger age were associated with more pain interference. Overall QOL was similar to other headache populations but lower than other chronic illness and healthy populations. CONCLUSIONS: Pediatric intracranial hypertension is associated with decreased QOL. Body mass index was a significant predictor of pain intensity and interference and should be monitored closely in clinic. Clinical care should include psychosocial screening to identify patients who would benefit from interventions to reduce the impact of this condition.

Pediatric Intracranial Hypertension: A Spotlight on Imaging, the Idiopathic Intracranial Hypertension Treatment Trial, and COVID-19 Associated Cases.

Primary intracranial hypertension (PIH) is characterized by clinical signs of increased intracranial pressure, papilledema, elevated opening pressure, and absence of mass lesion, hydrocephalus, or meningeal enhancement on neuroimaging. Visual changes are a common presenting feature and if untreated there is risk of irreversible vision loss. There have been recent proposed changes to the criteria for PIH along with studies looking at the differences in imaging characteristics between adult and pediatric PIH. The presence of transverse sinus stenosis alone was highly sensitive and specific for pediatric PIH. The Idiopathic Intracranial Hypertension Treatment Trial was an adult, multicenter study that examined the use of acetazolamide and weight loss on the course of PIH. The study confirmed many previously held beliefs including the most common presenting symptom in PIH is headache. Most patients present with bilateral papilledema with 58.2% of patients having symmetric Frisen scale grading and within one grade in 92.8%. Although diplopia is a common reported symptom, very few have evidence of cranial nerve palsy. Male gender, high-grade papilledema, and decreased visual acuity at presentation are risk factors for treatment failure. Acetazolamide use is associated with mild metabolic acidosis. During acetazolamide treatment, monitoring for hypokalemia or aplastic anemia is not recommended. Monitoring transaminases in the titration phase of treatment should be considered due to a case of transaminitis and pancreatitis with elevated lipase. Newer case reports have also seen associations of secondary intracranial hypertension with concurrent COVID-19 infection and MIS-C.

Case Series of Rapid Surgical Interventions in Fulminant Intracranial Hypertension.

AIMS: Examine ophthalmologic outcomes and complications of lumbar drain and cerebrospinal fluid shunts in pediatric fulminant intracranial hypertension. METHODS: Patients under 21 years of age with a diagnosis of fulminant intracranial hypertension with temporary lumbar drain only, shunt after lumbar drain, and shunt only were included. Parameters investigated include lumbar drain data, medication freedom, time to resolution of papilledema, improvement in cranial nerve palsy, afferent pupillary defects, visual fields, visual acuity, and complications of each intervention. RESULTS: Four patients had temporary lumbar drain, 2 temporary lumbar drain and cerebrospinal fluid shunt, and 3 shunt only. All achieved medication freedom and resolution of papilledema and cranial nerve palsies (if present). Most had resolution of preprocedure afferent pupillary defects. Minor residual visual field deficits occurred in 67%, and all had visual acuity improvement. One patient's lumbar drain dislodged, and one patient had 2 cerebrospinal fluid shunt revisions. CONCLUSION: Temporary lumbar drain with medical therapy may be a viable first approach to fulminant intracranial hypertension.

Intracranial Hypertension Recurrence Risk After Wean of Intracranial Pressure-Lowering Medication.

BACKGROUND: The proportion of children with recurrent signs and symptoms of intracranial hypertension after medication wean has been reported to be between 18% and 50%. Few studies have reported intracranial hypertension recurrence risk in children while adjusting for each individual's observed follow-up time after medication wean. In addition, the role of intracranial hypertension etiology on the risk of disease recurrence has not been widely studied. METHODS: The medical charts of patients with intracranial hypertension treated with intracranial pressure-lowering medication were analyzed retrospectively for disease recurrence. Baseline characteristics from diagnosis were recorded in addition to information regarding duration of therapy, medication wean, and recurrence. Survival analyses as well as Poisson regression models with time under observation as an offset were performed. RESULTS: One hundred and thirty-three patients were included in the study. The cumulative risk of intracranial hypertension recurrence increased rapidly within the first six months after medication wean and was 1.5% at one month, 9.5% at three months, and 20% at six months. This risk leveled off near 12 to 18 months. CONCLUSIONS: While the cumulative risk of intracranial hypertension recurrence increases most dramatically within the first six months after medication wean, it does not appear to taper until 12 to 18 months. Given the possibility of delayed or asymptomatic recurrences, long-term follow-up is ideal, although patients can likely be seen less frequently after the first 12 to 18 months after medication wean.

Cerebrospinal Fluid Analysis for Viruses by Metagenomic Next-Generation Sequencing in Pediatric Encephalitis: Not Yet Ready for Prime Time?

BACKGROUND: Metagenomic next-generation sequencing offers an unbiased approach to identifying viral pathogens in cerebrospinal fluid of patients with meningoencephalitis of unknown etiology. METHODS: In an 11-month case series, we investigated the use of cerebrospinal fluid metagenomic next-generation sequencing to diagnose viral infections among pediatric hospitalized patients presenting with encephalitis or meningoencephalitis of unknown etiology. Cerebrospinal fluid from patients with known enterovirus meningitis were included as positive controls. Cerebrospinal fluid from patients with primary intracranial hypertension were included to serve as controls without known infections. RESULTS: Cerebrospinal fluid metagenomic next-generation sequencing was performed for 37 patients. Among 27 patients with encephalitis or meningoencephalitis, 4 were later diagnosed with viral encephalitis, 6 had non-central nervous system infections with central nervous system manifestations, 6 had no positive diagnostic tests, and 11 were found to have a noninfectious diagnosis. Metagenomic next-generation sequencing identified West Nile virus (WNV) in the cerebrospinal fluid of 1 immunocompromised patient. Among the 4 patients with known enterovirus meningitis, metagenomic next-generation sequencing correctly identified enteroviruses and characterized the viral genotype. No viral sequences were detected in the cerebrospinal fluid of patients with primary intracranial hypertension. Metagenomic next-generation sequencing also identified sequences of nonpathogenic torque Teno virus in cerebrospinal fluid specimens from 13 patients. CONCLUSIONS: Our results showed viral detection by cerebrospinal fluid metagenomic next-generation sequencing only in 1 immunocompromised patient and did not offer a diagnostic advantage over conventional testing. Viral phylogenetic characterization by metagenomic next-generation sequencing could be used in epidemiologic investigations of some viral pathogens, such as enteroviruses. The finding of torque Teno viruses in cerebrospinal fluid by metagenomic next-generation sequencing is of unknown significance but may merit further exploration for a possible association with noninfectious central nervous system disorders.

Secondary Intracranial Hypertension in Pediatric Patients With Cryopyrin-Associated Periodic Syndrome.

BACKGROUND: Cryopyrin-associated periodic syndrome is characterized by periodic fever, rash, and joint pain. Papilledema rarely occurs. We present our series of patients with cryopyrin-associated periodic syndrome who clinically met the diagnostic criteria for Muckle-Wells syndrome and our experience with secondary intracranial hypertension. METHODS: Retrospective review of all patients with cryopyrin-associated periodic syndrome at Nationwide Children's Hospital from October 2015 to September 2017. RESULTS: Eighteen children met inclusion criteria: 15 females and three males, aged 1.5 to 16.2 years. Fifteen had periodic fever genetic testing; three had a known genetic defect identified, eight had a defect identified not currently known to be associated with cryopyrin-associated periodic syndrome, and four had no defect identified. Six patients (30%) developed headaches and were diagnosed with secondary intracranial hypertension. Lumbar puncture opening pressures ranged from 28 to 45 cm H2O. Only one patient had papilledema. Initial treatment was medical in all cases, by increasing interleukin-1 inhibitor dose and/or acetazolamide. One patient required a ventriculoperitoneal shunt for headache management. No visual acuity loss was detected. All six patients with secondary intracranial hypertension had a known genetic mutation or genetic variant of unknown significance; five involved the NLRP3 gene. CONCLUSIONS: In our series of 18 patients with cryopyrin-associated periodic syndrome, secondary intracranial hypertension occurred at a higher than expected rate. We suspect that genetic defects involving the NLRP3 gene may be a risk factor. Papilledema was present in only one patient. Physicians treating cryopyrin-associated periodic syndrome should be aware of this vision-threatening association and potential therapeutic approach.

Sleep disturbance in pediatric intracranial hypertension.

STUDY OBJECTIVES: There is a well-established association between headache disorders and sleep disturbances in children, but it is unknown whether sleep disturbance plays a role in pediatric intracranial hypertension. The objective of this study was to examine sleep issues related to pediatric intracranial hypertension. METHODS: Patients with intracranial hypertension in the Pediatric Intracranial Hypertension Clinic were recruited between July 2017 and September 2018. Demographic data were collected from the electronic medical record in addition to patient and parent completed questionnaires. Information on sleep behaviors was gathered using the Children's Sleep Habits Questionnaire, and control data were obtained from patient siblings. Statistical analyses were performed using paired t tests or two-sample t tests, as appropriate. RESULTS: Sixty-two pairs of patients and matched sibling controls were compared. We found a statistically significant difference in total sleep disturbance score (control mean, 44.3; patient mean, 48.1; n = 33 pairs, t = -2.2, P = .035), as well as subscale scores of sleep onset delay (control mean, 1.4; patient mean, 1.7; n = 52 pairs, t = -2.53, P = .014), parasomnias (control mean, 8.5; patient mean, 9.5; n = 42 pairs, t = -2.59, P = .013), and sleep-disordered breathing (control mean, 3.1; patient mean, 3.4; n = 44 pairs, t = -2.61, P = .013). No difference was found in bedtime resistance, sleep duration, sleep anxiety, night awakenings, and daytime sleepiness subscales. Furthermore, no difference was found in total sleep disturbance score between patient subsets, including primary vs secondary intracranial hypertension, body mass index, pubertal status, presence of headaches, or intracranial hypertension treatment. CONCLUSIONS: This observational study suggests that pediatric intracranial hypertension is associated with a modest increase in sleep disturbances.

Sleep Disturbance in Pediatric Intracranial Hypertension.

STUDY OBJECTIVES: There is a well-established association between headache disorders and sleep disturbances in children, but it is unknown if sleep disturbance plays a role in pediatric intracranial hypertension. The objective of this study was to examine sleep issues related to pediatric intracranial hypertension. METHODS: Patients with intracranial hypertension who were followed in the Pediatric Intracranial Hypertension Clinic were recruited between July 2017 and September 2018. Demographic data was collected from the electronic medical record in addition to patient and parent completed questionnaires. Information on sleep behaviors was gathered using the Children's Sleep Habits Questionnaire, and control data was obtained from patient siblings. Statistical analyses were performed using paired t-tests or two-sample t-tests, as appropriate. RESULTS: Sixty-two pairs of patients and matched sibling controls were compared. There was a statistically significant difference in total sleep disturbance score (control mean 44.3; patient mean 48.1; n=33 pairs, t=-2.2, p=0.035) as well as subscale scores of sleep onset delay (control mean 1.4; patient mean 1.7; n=52 pairs, t=-2.53, p=0.014), parasomnias (control mean 8.5; patient mean 9.5; n=42 pairs, t=-2.59, p=0.013), and sleep disordered breathing (control mean 3.1; patient mean 3.4; n=44 pairs, t=-2.61, p=0.013). There was no difference found in bedtime resistance, sleep duration, sleep anxiety, night wakings, and daytime sleepiness subscales. Furthermore, there was no difference in total sleep disturbance score between patient subsets including: primary versus secondary intracranial hypertension, body mass index, pubertal status, presence of headaches, or intracranial hypertension treatment. CONCLUSIONS: This observational study suggests that pediatric intracranial hypertension is associated with a modest increase in sleep disturbances.

Presenting Symptomatology and Risk Factors in Pediatric Secondary Intracranial Hypertension due to Venous Sinus Thrombosis.

BACKGROUND: There remains debate regarding the need for venous imaging in pediatric intracranial hypertension. METHODS: Records of patients aged 18 years or younger who were evaluated in the intracranial hypertension clinic at Nationwide Children's Hospital in Columbus, Ohio, were reviewed. Past medical history, diagnostic evaluation, and presenting symptoms were examined to evaluate differences in symptomatology presentation and risk factors in patients with pediatric intracranial hypertension with and without thrombosis. RESULTS: A total of 226 patients met inclusion criteria, 145 were diagnosed with primary intracranial hypertension, 81 with secondary intracranial hypertension, with 17 noted to have venous sinus thrombosis as the cause of their secondary intracranial hypertension. Of those with thrombosis, 41.2% did not have any thrombosis risk factors. Headache was the most prominent symptom, present in 73.8% (n = 107) of patients with primary intracranial hypertension, 87.5% (n = 56) of patients with secondary intracranial hypertension without thrombosis, and 82.4% (n = 14) with thrombosis. CONCLUSIONS: The only clinically significant difference in presenting symptomatology between the thrombosis and the other groups was nausea or vomiting. Predisposing factors to develop thrombosis were absent in 41.2% of patients. Hence, the need for venous imaging in pediatric intracranial hypertension cannot be clearly determined by clinical presentation or risk factors alone. Patients with indwelling catheters should receive imaging in the region of their catheter to rule out catheter-associated thrombosis.

Surgical intervention in pediatric intracranial hypertension: incidence, risk factors, and visual outcomes.

PURPOSE: To evaluate the incidence of surgical intervention in pediatric intracranial hypertension (IH), evaluate the visual outcomes of surgically managed patients, and identify potential predictors for surgical intervention. METHODS: The medical records of patients with primary and secondary IH at Nationwide Children's Hospital from 2010 to 2017 were reviewed retrospectively. Presenting characteristics of medically and surgically managed patients were compared, and the clinical courses of surgically managed patients were reviewed. RESULTS: A total of 129 medically and 14 surgically managed patients were included. The surgical incidence was 9.8%. Final visual acuity in 27 of 28 surgically managed eyes was 20/25 or better. In combined primary and secondary IH patients, elevations in body mass index (BMI; OR = 1.06; 95% CI, 1.01-1.11; P = 0.022) and lumbar puncture opening pressures ≥52 cm H2O (OR = 6.17; 95% CI, 1.93-19.67; P = 0.002) were significantly associated with the likelihood of surgical intervention when assessed by univariate logistic regression; grade of papilledema >2 was of marginal significance. After controlling for BMI, a lumbar puncture opening pressure of ≥52 cm H2O was more likely to result in surgery (adjusted OR = 4.69; 95% CI = 1.39-15.98; P = 0.013). CONCLUSIONS: Most pediatric IH can be treated medically. Patients with lumbar puncture opening pressures ≥52 cm H2O at the time of diagnosis are at a higher risk of surgical intervention and should be monitored closely. Elevations in presenting BMI and grade of papilledema may also increase the odds of surgery.

Intracranial hypertension: a current review.

PURPOSE OF REVIEW: To provide a current review of recent publications with regards to intracranial hypertension. RECENT FINDINGS: Attempts were made to provide pediatric data; however, the recent completion of the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) has provided a wealth of data with regards to adult intracranial hypertension.The pediatric incidence of intracranial hypertension ranges between 0.63 and 0.71 per 100 000 children. A majority of pediatric cases responded to acetazolamide, with resolution of headache averaging 3.8 weeks. Most patients require less than 1 year of treatment with male sex, older age at diagnosis, primary intracranial hypertension, and lack of headache being predictors of good response. Fluorescein angiography has the highest accuracy in distinguishing true papilledema from pseudopapilledema. The IIHTT found Frisen grade of papilledema was within 1 grade in 92.8% of patients. Monitoring of potassium levels is not required and aplastic anemia was not seen in patients taking acetazolamide. SUMMARY: Although the newer pediatric studies report incidence rates in pediatric intracranial hypertension are lower than seen in adults, intracranial hypertension is still a concern in pediatrics. There has been a wealth of information with regards to symptomatology, treatment, and outcomes from the IIHTT that will hopefully assist with management in the pediatric population.

Pediatric Intracranial Hypertension: a Current Literature Review.

PURPOSE OF REVIEW: The purpose of this review is to provide an update on pediatric intracranial hypertension. RECENT FINDINGS: The annual pediatric incidence is estimated at 0.63 per 100,000 in the USA and 0.71 per 100,000 in Britain. The Idiopathic Intracranial Hypertension Treatment Trial found improvement in visual fields, optical coherence tomography, Frisen grade, and quality of life with acetazolamide compared to placebo in adult patients, and these findings have been translated to the pediatric population. Pediatric intracranial hypertension is a disorder that if left untreated can lead to poor quality of life and morbidity. There are no current treatment studies in pediatrics, but adult data suggests acetazolamide remains an acceptable first-line medication.