A retrospective observational cohort study of the anesthetic management and outcomes of pediatric patients with Alexander disease undergoing lumbar puncture or magnetic resonance imaging.

BACKGROUND: Alexander disease is a rare, progressive leukodystrophy, which predisposes patients to complications under general anesthesia due to clinical manifestations including developmental delay, seizures, dysphagia, vomiting, and sleep apnea. However, study of anesthetic outcomes is limited. AIMS: Our aim was to describe patient characteristics, anesthetic techniques, and anesthesia-related complications for Alexander disease patients undergoing magnetic resonance imaging and/or lumbar puncture at a quaternary-care children's hospital. METHODS: We performed a retrospective review of anesthetic outcomes in patients with Alexander disease enrolled in a prospective observational study. Included patients had diagnosed Alexander disease and underwent magnetic resonance imaging and/or lumbar puncture at our institution. We excluded anesthetics for other procedures or at outside institutions. Collected data included patient characteristics, anesthetic techniques, medications, and complications under anesthesia and in the subsequent 24 h. We performed descriptive statistics as appropriate. RESULTS: Forty patients undergoing 64 procedures met inclusion criteria. Fifty-six procedures (87.5%) required general anesthesia or monitored anesthesia care (MAC) and eight (12.5%) did not. The general anesthesia/MAC group tended to be younger than nonanesthetized patients (median age 6 years [IQR 3.8; 9] vs. 14.5 years [IQR 12.8; 17.5]). In both groups, dysphagia (78.6% vs. 87.5%, respectively), seizures (62.5% vs. 25%), and recurrent vomiting (17.9% vs. 25%) were frequently reported preprocedure symptoms. Inhalational induction was common (N = 48; 85.7%), and two (3.6%) underwent rapid sequence induction. Serious complications were rare, with no aspiration or seizures. Hypotension resolving with ephedrine occurred in eight cases (14.3%). One patient each (1.8%) experienced postprocedure emergence agitation or vomiting. Fifty-three (94.6%) were ambulatory procedures. No inpatients required escalation in acuity of care. CONCLUSIONS: In this single-center study, patients with Alexander disease did not experience frequent or irreversible complications while undergoing general anesthesia/MAC. Co-morbid symptoms were not increased postanesthesia. Some patients may not require anesthesia to complete short procedures.

Acquisition and Loss of Developmental Milestones and Time to Disease-Related Outcomes in Cerebral Alexander Disease.

Objective: To determine the ages at acquisition of developmental milestones, loss of motor function, and clinical symptoms in Alexander disease. Methods: Patients with confirmed cerebral Alexander disease were included. Data abstraction of developmental and disease-specific milestones was performed from medical records, physical exams, and questionnaires. Mixed effects logistic regression was used to determine if key clinical features were associated with milestone achievement, controlling for patient age. Results: 51 patients with cerebral/infantile Alexander disease were evaluated at a mean age of 10.96 years (range 2.29-31.08 years). Developmental milestones in Alexander disease were often achieved but delayed. Ambulation was achieved in 44 subjects (86%); 34 (67%) subjects walked independently (mean age 1.9 years, range 0.91-3.25 years) and an additional 10 (20%) subjects walked with assistance (mean age 3.9 years, range 1.8-8 years) but did not progress to independent ambulation. Developmental delay was the earliest and most prevalent symptom (N = 48 [94%], mean age 0.58 years), compared to an initial seizure (N = 41 [80%], mean age 2.80 years), and macrocephaly (N = 28 [55%], mean age 4.04 years), P < .0001 between these ages of onset. Loss of independent ambulation occurred in 11 of the 34 (32%) children who had acquired ambulation (range 3.41-15.10 years). Presence of seizures or macrocephaly did not predict the achievement or loss of ambulation. Conclusions: The clinical triad of developmental delay, seizures, and macrocephaly are not universally present in cerebral Alexander disease. Clinicians should have a high index of suspicion for Alexander disease in patients with mild delays and a first seizure.

Reliability of the Telemedicine Application of the Gross Motor Function Measure-88 in Patients With Leukodystrophy.

BACKGROUND: Leukodystrophies are a rare class of disorders characterized by severe neuromotor disability. There is a strong need for research regarding the functional status of people with leukodystrophy which is limited by the need for in-person assessments of mobility. The purpose of this study is to assess the reliability of the Gross Motor Function Measure-88 (GMFM-88) using telemedicine compared with standard in-person assessments in patients with leukodystrophy. METHODS: A total of 21 subjects with a diagnosis of leukodystrophy (age range = 1.79-52.82 years) were evaluated by in-person and by telemedicine evaluations with the GMFM-88 by physical therapists. Inter-rater reliability was assessed through evaluation of the same subject by two independent raters within a three-week period (n = 10 encounters), and intrarater reliability was assessed through blinded rescoring of video-recorded assessments after a one-week time interval (n = 6 encounters). RESULTS: Remote assessments were performed by caregivers in all 21 subjects using resources found in the home with remote guidance. There was agreement between all paired in-person and remote measurements (Lin's concordance correlation ≥0.995). The Bland-Altman analysis indicated that the paired differences were within ±5%. Intrarater and inter-rater reliability demonstrated an intraclass correlation coefficient of >0.90. CONCLUSIONS: These results support that remote application of the GMFM-88 is a feasible and reliable approach to assess individuals with leukodystrophy. Telemedicine application of outcome measures may be of particular value in rare diseases and those with severe neurologic disability that impacts the ability to travel.

Autism Spectrum Disorder in Pediatric Idiopathic Intracranial Hypertension.

In recent years, the substantial burden of medical comorbidities in autism spectrum disorder (ASD) populations has been described. We report a retrospective observational case series of pediatric patients with suspected idiopathic intracranial hypertension (IIH) and concurrent ASD. Pediatric subjects with suspected IIH aged 2-18 years were identified by review of a pediatric neuro-ophthalmologist's database spanning from July 1993 to April 2013. ASD diagnoses were identified within this cohort by an ICD-9 diagnosis code search and database review. Three subjects had concurrent ASD diagnoses; all were non-obese males. Since the retrospective observational case series was performed in April 2013, we identified three additional IIH cases in boys with ASD. Our experience suggests that IIH may be a comorbidity of ASD, particularly in non-obese boys.

Interocular Difference in Retinal Nerve Fiber Layer Thickness Predicts Optic Neuritis in Pediatric-Onset Multiple Sclerosis.

BACKGROUND: Optical coherence tomography (OCT) is capable of quantifying retinal damage. Defining the extent of anterior visual pathway injury is important in multiple sclerosis (MS) as a way to document evidence of prior disease, including subclinical injury, and setting a baseline for patients early in the course of disease. Retinal nerve fiber layer (RNFL) thickness is typically classified as low if values fall outside of a predefined range for a healthy population. In adults, an interocular difference (IOD) in RNFL thickness greater than 5 µm identified a history of unilateral optic neuritis (ON). Through our PERCEPTION (PEdiatric Research Collaboration ExPloring Tests in Ocular Neuroimmunology) study, we explored whether RNFL IOD informs on remote ON in a multicenter pediatric-onset MS (POMS) cohort. METHODS: POMS (defined using consensus criteria and first attack <18 years) patients were recruited from 4 academic centers. A clinical history of ON (>6 months prior to an OCT scan) was confirmed by medical record review. RNFL thickness was measured on Spectralis machines (Heidelberg, Germany). Using a cohort of healthy controls from our centers tested on the same machines, RNFL thickness <86 µm (<2 SDs below the mean) was defined as abnormal. Based on previously published findings in adults, an RNFL IOD >5 µm was defined as abnormal. The proportions of POMS participants with RNFL thinning (<86 µm) and abnormal IOD (>5 µm) were calculated. Logistic regression was used to determine whether IOD was associated with remote ON. RESULTS: A total of 157 participants with POMS (mean age 15.2 years, SD 3.2; 67 [43%] with remote ON) were enrolled. RNFL thinning occurred in 45 of 90 (50%) ON eyes and 24 of 224 (11%) non-ON eyes. An IOD >5 µm was associated with a history of remote ON (P < 0.001). An IOD >5 µm occurred in 62 participants, 40 (65%) with remote ON. Among 33 participants with remote ON but normal RNFL values (≥86 µm in both eyes), 14 (42%) were confirmed to have ON by IOD criteria (>5 µm). CONCLUSIONS: In POMS, the diagnostic yield of OCT in confirming remote ON is enhanced by considering RNFL IOD, especially for those patients with RNFL thickness for each eye in the normal range. An IOD >5 µm in patients with previous visual symptoms suggests a history of remote ON.

Structural correlates of atypical visual and motor cortical oscillations in pediatric-onset multiple sclerosis.

We have previously demonstrated that pediatric-onset multiple sclerosis (POMS) negatively impacts the visual pathway as well as motor processing speed. Relationships between MS-related diffuse structural damage of gray and white matter (WM) tissue and cortical responses to visual and motor stimuli remain poorly understood. We used magnetoencephalography in 14 POMS patients and 15 age- and sex-matched healthy controls to assess visual gamma (30-80 Hz), motor gamma (60-90 Hz), and motor beta (15-30 Hz) cortical oscillatory responses to a visual-motor task. Then, 3T MRI was used to: (a) calculate fractional anisotropy (FA) of the posterior visual and corticospinal motor WM pathways and (b) quantify volume and thickness of the cuneus and primary motor cortex. Visual gamma band power was reduced in POMS and was associated with reduced FA of the optic radiations but not with loss of cuneus volume or thickness. Activity in the primary motor cortex, as measured by postmovement beta rebound amplitude associated with peak latency, was decreased in POMS, although this reduction was not predicted by structural metrics. Our findings implicate loss of WM integrity as a contributor to reduced electrical responses in the visual cortex in POMS. Future work in larger cohorts will inform on the cognitive implications of this finding in terms of visual processing function and will determine whether the progressive loss of brain volume known to occur in POMS ultimately contributes to both progressive dysfunction in such tasks as well as progressive reduction in cortical electrical responses in the visual cortex.

High- and Low-Contrast Letter Acuity Perception Matures With Age in Normally Sighted Children.

BACKGROUND: High-contrast visual acuity (HCVA) changes with age, yet little is known about pediatric-specific age- and sex-normative values for low-contrast letter acuity (LCLA). We define maturational changes in monocular and binocular HCVA and LCLA in childhood and adolescence. METHODS: Normally sighted youth (ages 5-20 years, without neurologic or ophthalmologic disease and best-corrected HCVA of 20/25 or better in each eye) were recruited. Mean monocular and binocular scores using Early Treatment Diabetic Retinopathy Study (for HCVA) and 2.5% and 1.25% Sloan (for LCLA) charts and the magnitude of binocular summation were calculated using 2-year bins. Relationships between scores and age were explored using scatterplots with Locally Weighted Scatterplot Smoothing (LOWESS) and analysis of variance that accounts for intereye correlation, followed by test of linear trend for age effect. RESULTS: Among 101 (202 eyes) healthy participants (mean age 13 years, 42% males), monocular and binocular scores varied by age, with highest mean scores achieved in the 13 to 14-year age group for both HCVA and LCLA. Between the ages of 5 and 14.9 years, monocular scores increased linearly with age (0.76 letter/year for HCVA, 1.11 letters/year for 2.5% LCLA, and 0.97 letter/year for 1.25% LCLA; all P < 0.0001). Binocular HCVA scores also increased with age between 5 and 14.9 years (0.71 letters/year, P < 0.0001). The magnitude of binocular summation for HCVA or LCLA did not change with age. CONCLUSIONS: HCVA and LCLA abilities mature into adolescence, peak between 13 and 14.9 years of age, and then plateau into adulthood. Evaluation of patients with visual deficits should consider age-expected normal visual acuity.

Effects of Optic Neuritis, T2 Lesions, and Microstructural Diffusion Integrity in the Visual Pathway on Cortical Thickness in Pediatric-Onset Multiple Sclerosis.

BACKGROUND AND PURPOSE: Pediatric-onset multiple sclerosis (POMS) is associated with focal inflammatory lesions and the loss of cortical and deep gray matter. Optic neuritis (ON) and white matter (WM) lesions in the visual pathway can directly contribute to visual cortical mantle thinning. We determine the relative contributions of MS insult on anterior and posterior visual pathway integrity. METHODS: High- and low-contrast visual acuity, optical coherence tomography (OCT), and 3T MRI scans were obtained from 20 POMS patients (10 with remote ON) and 22 age- and sex-matched healthy controls. Cortical mantle thickness was measured using FreeSurfer. Fractional anisotropy (FA) and mean diffusivity were calculated for postchiasmal optic radiations (with and without WM lesions). Groups were compared using Student's t-test (adjusted for multiple comparisons), and simple linear regression was used to investigate interrelationships between measures. RESULTS: Mean cortical thickness of the whole brain was reduced in patients (2.49 mm) versus controls (2.58 mm, P = .0432) and in the visual cortex (2.07 mm vs. 2.17 mm, P = .0059), although the foveal confluence was spared. Mean FA of the optic radiations was reduced in POMS (.40) versus controls (.43, P = .0042) and correlated with visual cortical mantle thickness in POMS (P = .017). Visual acuity, OCT measures, and lesion volumes in the optic radiations were not associated with cortical mantle thickness. CONCLUSIONS: POMS negatively impacts the integrity of the anterior visual pathway, but it is the loss of WM integrity that drives anterograde loss of the cortical mantle. Preserved visual acuity and foveal sparing imply some degree of functional and structural resilience.

Validation of a symptom-based questionnaire for pediatric CNS demyelinating diseases.

PURPOSE: Optic neuritis is a manifestation of numerous neuroinflammatory disorders. Recognition of current and prior symptoms may facilitate identification of an underlying multifocal neurologic disease. The purpose of this study was to determine whether a symptom-based questionnaire could inform clinical decision making by identifying children with visual complaints who may have a systemic demyelinating disorder. METHODS: Children with visual changes from non-demyelinating disease were compared with patients with confirmed pediatric-onset multiple sclerosis (MS) or neuromyelitis optica spectrum disorder (NMOSD). Participants completed a 21-item questionnaire to capture their recent (<30 days) and remote (>30 days) symptoms of neurologic dysfunction. The questionnaire scores were compared using t tests, and the 95% confidence interval for each group was used to determine a threshold score suggesting demyelinating disease. RESULTS: We enrolled 51 participants (30 females [59%]) with a mean age of 14.6 years (range, 4-21): 25 in the non-demyelinating disease group and 26 with MS/NMOSD. The mean questionnaire score for the non-demyelinating group was 5.0 points (95% CI, 3.3-6.9); for the MS/NMOSD group, 9.4 points (95% CI, 7.4-11.4) for the MS/NMOSD group (P < 0.002). Questionnaire results were dichotomized using a score of ≥7 as indicative of demyelinating disease, with 69% sensitivity and 72% specificity. An abbreviated questionnaire, using 8 questions that differed between groups, had a sensitivity of 65% and specificity of 92%. CONCLUSIONS: A symptom-based questionnaire is sensitive and specific for identifying children with CNS demyelinating disease and may be useful as a screening tool for children with vision complaints and possible demyelination.

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.

Clinical and Prognostic Significance of Cerebrospinal Fluid Opening and Closing Pressures in Pediatric Pseudotumor Cerebri Syndrome.

BACKGROUND: The purpose of this study was to determine the prognostic utility of closing pressure and volume of cerebrospinal fluid removed with respect to papilledema resolution and headache improvement in pediatric pseudotumor cerebri syndrome. METHODS: This is a retrospective observational study of 93 children with definite pseudotumor cerebri syndrome. The primary outcome measure was time to resolution of papilledema, and the secondary outcome measure was time to resolution of headache. RESULTS: There were no significant differences in gender, age, or body mass index z score observed between subjects with (N = 35) and without (N = 58) documented closing pressure. The median time to resolution of papilledema was not statistically different between children above or equal to and those below the median closing pressure (170 mm of cerebrospinal fluid, n = 31, P = 0.391) or the volume of median cerebrospinal fluid removed (16 mL, n = 19, P = 0.155). There was no statistically significant difference detected in days of headache between the children with opening pressure above and equal to the median (400 mm of cerebrospinal fluid) and the children with opening pressure below the median (n = 44, P = 0.634). CONCLUSIONS: No significant association between closing pressure, amount of cerebrospinal fluid removed, and time to resolution of papilledema due to pseudotumor cerebri syndrome was detected. The diagnostic and therapeutic purposes of either measuring the closing pressure or maximizing the volume of cerebrospinal fluid removed were not evident in these analyses.

Optical coherence tomography and visual evoked potentials in pediatric MS.

OBJECTIVE: To determine the relative ability of optical coherence tomography (OCT) and pattern-reversal visual evoked potentials (pVEPs) to detect visual pathway involvement in pediatric-onset MS. METHODS: Pediatric-onset MS participants (onset <18 years) and healthy controls (HCs) underwent OCT (Cirrus HD-OCT) and pVEPs. Retinal nerve fiber layer (RNFL), ganglion cell layer to inner plexiform layer (GCL-IPL), and P100 pVEP latency were measured. Generalized estimating equation models were used to compare the groups, adjusting for age and intereye correlations. RESULTS: Twenty-four pediatric MS participants, 14 with a history of remote (>6 months) optic neuritis (ON) in one eye (8 participants) or both the eyes (6 participants), and 24 HCs were enrolled. RNFL thinning (<83 µm, 2 SDs below HC eyes) occurred in 50% of ON eyes vs 5% of non-ON eyes. Prolonged VEP latency (>109 msec) occurred in 58% of ON eyes and 55% of non-ON eyes. A clinical history of ON predicted RNFL (p < 0.001) and GCL-IPL thinning (p = 0.011), whereas prolonged pVEP latency in children with MS occurred independent of ON history. CONCLUSIONS: OCT and pVEPs provide complementary but distinct insights. OCT is sensitive to retinal changes in the context of clinical ON, whereas pVEPs are useful to detect disseminated lesions of the visual pathway in children with MS.

Binocular low-contrast letter acuity and the symbol digit modalities test improve the ability of the Multiple Sclerosis Functional Composite to predict disease in pediatric multiple sclerosis.

BACKGROUND: Outcome measures to capture disability, such as the Multiple Sclerosis Functional Composite (MSFC), were developed to enhance outcome measurements for clinical trials in adults with multiple sclerosis (MS). The MSFC initially included three components: a timed 25-foot walk [T25FW], 9-hole peg test [9HPT], and the Paced Auditory Serial Addition Task [PASAT]. Modifications to the original MSFC, such as adding binocular low-contrast letter acuity (LCLA) or substituting the symbol digit modalities test (SDMT) for the PASAT, improved the capacity to capture neurologic impairment in adults. Similar outcome scales for pediatric MS have not yet been established. OBJECTIVE: To determine whether the three-component MSFC or a modified MSFC with LCLA and the SDMT better identifies neurological deficits in pediatric MS. METHODS: We evaluated 5 measures (T25FW, 9HPT, Children's PASAT [ChiPASAT], SDMT, and binocular LCLA [Sloan charts, 1.25% contrast]) in children with MS (disease onset <18 years) and healthy controls. To be able to compare measures whose scores have different scales, Z-scores were also created for each test based on the numbers of standard deviations from a control group mean, and these individual scale scores were combined to create composite scores. Logistic regression models, accounting for age, were used to determine whether the standard 3-component MSFC or modified versions (including 4 or 5 metrics) best distinguished children with MS from controls. RESULTS: Twenty pediatric-onset MS subjects, aged 6-21 years, and thirteen healthy controls, aged 6-19 years, were enrolled. MS subjects demonstrated worse scores on the 9HPT (p=0.004) and SDMT (p=0.001), but not the 25FTW (adjusted for height, p=0.63) or the ChiPASAT (p=0.10): all comparisons adjusted for age. Decreased (worse) binocular LCLA scores were associated with MS (vs. control status, p=0.03, logistic regression; p=0.08, accounting for age). The MSFC composite score for the traditional 3 components did not differ between the groups (p=0.28). Replacing the ChiPASAT with the SDMT (OR 0.72, p=0.05) better distinguished MS from controls. A modified MSFC-4 with the SDMT replacing the ChiPASAT and including binocular 1.25% LCLA had the greatest capacity to distinguish pediatric MS from controls (OR 0.89, p=0.04, logistic regression). Including all 5 metrics as a composite MSFC-5 did not improve the model (p=0.18). CONCLUSIONS: A modified MSFC (25FTW, 9HPT, SMDT, and binocular 1.25% LCLA) is more sensitive than the traditional MSFC or its components to capture the subtle impairments that characterize pediatric MS and should be validated in order to be considered for future pediatric MS trials.

Hospital admission rates for pediatric multiple sclerosis in the United States using the Pediatric Health Information System (PHIS).

BACKGROUND: The onset of multiple sclerosis (MS) during childhood or adolescence is increasingly recognized in the United States. Administrative databases quantify healthcare utilization as measured by hospital admissions, providing insight into the impact of MS in the pediatric population. OBJECTIVE: We examine the frequency of hospital admissions for pediatric MS in the US using the Pediatric Health Information System (PHIS) database. METHODS: Data was extracted from the PHIS database using the ICD-9 code for MS (340.00) and reviewed to verify case ascertainment. Mean, median, and range values were determined for the number of inpatient hospitalizations per patient, number of days in the hospital, and cost of each encounter. A trend analysis was performed to evaluate the annual frequency of MS-related admissions over the study period. RESULTS: After case verification, the PHIS database extraction reported 2068 hospital inpatient encounters for 1422 unique pediatric MS patients between 2004 and 2013. The median number of hospitalizations per patient was 2 with a median hospital stay of 4 days. Admission rates for MS increased from 2.37 per 10,000 in 2004 to 4.13 per 10,000 in 2013. CONCLUSION: The number of admissions due to pediatric MS has increased since the start of the PHIS database collection, concurrent with increased disease awareness and the establishment of dedicated pediatric MS centers.

"Alice in wonderland" syndrome: presenting and follow-up characteristics.

BACKGROUND: We investigated the distribution of symptoms and etiologies of patients with "Alice in Wonderland" syndrome (visual perception of change in one's body size) and "Alice in Wonderland"-like syndrome (extrapersonal illusions) at presentation and to determine their prognosis. DESIGN: Retrospective chart review and telephone interview. METHODS: Charts of children diagnosed with "Alice in Wonderland" syndrome by a pediatric neuro-ophthalmologist between July 1993 and July 2013 were reviewed. Patients seen before 2012, or their parents, were contacted for follow-up information. RESULTS: A total of 48 patients (average age 8.1 years) diagnosed with "Alice in Wonderland" syndrome or "Alice in Wonderland"-like syndrome were identified. Common visual symptoms were micropsia (69%), teleopsia (50%), macropsia (25%), metamorphopsia (15%), and pelopsia (10%). Magnetic resonance imaging and electroencephalography were unrevealing in 21 of 21 and 23 of 23 cases, respectively. The etiology was infection in 33% of patients and migraine and head trauma in 6% each. No associated conditions were found in 52%. Of the 15 patients with follow-up, 20% had a few more events of "Alice in Wonderland" syndrome or "Alice in Wonderland"-like syndrome, which eventually stopped after the initial diagnosis; 40% had no more events, and 40% were still having "Alice in Wonderland" syndrome or "Alice in Wonderland"-like syndrome symptoms at the time of the interview, while four patients (27%) developed migraines and one patient (7%) seizures since the diagnosis. CONCLUSION: "Alice in Wonderland" syndrome and "Alice in Wonderland"-like syndrome typically affect young children, and the most common visual complaints are micropsia and teleopsia. The most common associated condition is infection, but half of these individuals have no obvious trigger. Magnetic resonance imaging and electroencephalography are not helpful. The symptoms of "Alice in Wonderland" syndrome and "Alice in Wonderland"-like syndrome usually resolve, but in more than one third of the cases, they continue. One quarter of patients without a history of migraine may subsequently develop migraine.