The Fainting Assessment Inventory: A 10-Item Instrument Developed to Screen for Psychogenic Nonsyncopal Collapse Among Youth Referred for Syncope.

The conversion disorder that appears like syncope is common but poorly recognized. The study aimed to develop and validate a brief, clinician-administered screening tool to discriminate psychogenic nonsyncopal collapse (PNSC) among young patients referred for fainting. Consecutive patients with PNSC and with syncope (15.4 ± 2.2 years) completed a 92-item inventory highlighting features typical of PNSC and neurally mediated syncope (n = 35, each cohort). Fourteen items were retained and revised and then administered to new cohorts ultimately diagnosed with PNSC or syncope (n = 40, each cohort). Further revision led to a 10-item Fainting Assessment Inventory (FAI-10). Scoring the syncope ratings positively and the PNSC ratings negatively, median scores differed between cohorts with PNSC and with syncope (-6 vs. 7; p < 0.001). Diagnostic sensitivity (0.95), specificity (0.875), positive predictive value (0.889), negative predictive value (0.93), and area under the curve (0.973) were calculated. The FAI-10 furthers clinicians' ability to distinguish various forms of transient loss of consciousness.

Signs of autonomic arousal precede tilt-induced psychogenic nonsyncopal collapse among youth.

Characterizing the physiologic changes leading up to psychogenic nonsyncopal collapse (PNSC) may help to elucidate the processes that cause paroxysmal functional neurological symptom disorders and to clinically distinguish PNSC from syncope. Thus, we aimed to characterize preictal sweat rate, heart rate, and systolic blood pressure changes among patients with tilt-induced PNSC compared to patients with tilt-induced neurally mediated syncope. The presence of increased preictal sweating was compared between groups. Heart rates and systolic blood pressures were compared from the recumbent and tilted baselines to the periods 120 s and 30 s prior to PNSC and syncope. Patients with PNSC (n = 44) were more likely than patients with syncope (n = 44) to have preictal increases in sweating, n = 31 (70.5%) versus n = 21 (47.7%), p = 0.03, although all patients with syncope eventually developed a sweat response. Comparing the recumbent baseline to the period 30 s prior to PNSC, blood pressure (112 ±â€¯9 versus 129 ±â€¯13 mmHg, p < 0.001) and heart rate (76 ±â€¯12 versus 119 ±â€¯22 bpm, p < 0.001) increased. Similarly, comparing the tilted baseline to the period 30 s prior to PNSC, blood pressure (118 ±â€¯12 versus 129 ±â€¯13 mmHg, p < 0.001) and heart rate (95 ±â€¯15 versus 119 ±â€¯22 bpm, p < 0.001) increased. Preictal blood pressure and heart rate differed significantly between patients with PNSC and patients with syncope. In conclusion, signs of autonomic arousal (increased sweating, heart rate, and blood pressure) often precede tilt-induced PNSC. Sweating prior to fainting may not be useful in distinguishing PNSC from neurally mediated syncope.

Lightheadedness After Concussion: Not All Dizziness is Vertigo.

OBJECTIVE: To characterize the presence and degree of postconcussion lightheadedness in relation to postconcussion vertigo and dizziness, and to determine whether lightheadedness influences overall symptom duration. DESIGN: Prospective, cohort design. SETTING: Nationwide Children's Hospital, Sports Concussion Clinic. PARTICIPANTS: Five hundred ten patients (9-19 years of age) within 30 days of concussion injury. MAIN OUTCOME MEASURES: Patient ratings (scale 0-6) of multiple postconcussion symptoms including lightheadedness, vertigo, and dizziness, reported from the day of clinic evaluation and recalled from the day of concussion. RESULTS: Postconcussion lightheadedness occurred commonly relative to vertigo. Lightheadedness was recalled more than vertigo (70.8% vs 48.6%, P < 0.001) on concussion day and reported more than vertigo (47.1% vs 24.1%, P < 0.001) on the day of clinic evaluation. Principal component analysis demonstrated strong correlations among lightheadedness, vertigo, balance difficulties, difficulty concentrating, mental fogginess, and difficulty remembering, relative to other postconcussion symptoms. When present on the day of clinic evaluation, these highly correlated symptoms predicted prolonged concussion recovery [P = 0.028; Hazard Ratio (HR) = 1.2]. Female sex (P = 0.04; HR = 1.23), emotional symptoms recalled from the day of concussion (P = 0.007; HR = 1.23), and cephalalgic symptoms (P < 0.001; HR = 1.34) reported on the day of clinic evaluation also predicted prolonged concussion recovery. CONCLUSIONS: Not all postconcussion dizziness is vertigo. Postconcussion lightheadedness is common and, when present at the time of clinic evaluation, can influence concussion recovery. CLINICAL RELEVANCE: Distinguishing postconcussion lightheadedness from vertigo may help to inform clinical treatment and concussion research design. This study adds to the growing body of evidence that supports an association between concussion and autonomic dysfunction.

Utilization of conventional neuroimaging following youth concussion.

BACKGROUND/OBJECTIVE: Conventional neuroimaging is not recommended for the routine diagnosis of concussion, but some patients with concussion undergo computed tomography (CT) or magnetic resonance imaging (MRI). The objective of this study was to explore the clinical factors that predict neuroimaging utilization in concussion. METHODS: Concussion-related CT and MRI data were analysed from 1953 patients, aged 10-19 years, who presented to a sports concussion clinic within 30 days of injury. RESULTS: The majority of CT scans (n = 193) were obtained during the acute concussion period (mean = 2.7 days post-concussion), whereas MRI scans (n = 134) were ordered later during recovery (mean = 39.4 days post-concussion). Predictors of CT utilization included loss of consciousness, amnesia and vomiting (all p < 0.001). Prior concussion (p = 0.002) and continued participation in activity after injury (p = 0.03) predicted greater MRI utilization. Neuroimaging with either CT (p = 0.024, hazard ratio = 1.2) or MRI (p < 0.001, hazard ratio = 2.75) was associated with prolonged symptoms. Only 3.1% of CTs and 1.5% of MRIs demonstrated signs of traumatic brain injury. CONCLUSION: Several clinical factors predict neuroimaging utilization in patients with concussion. CT is generally used acutely, while MRI is used in the sub-acute and chronic post-concussion periods. In a sports concussion clinic, delayed neuroimaging has limited clinical yield.

Specific Factors Influence Postconcussion Symptom Duration among Youth Referred to a Sports Concussion Clinic.

OBJECTIVE: To identify the clinical factors that influence the duration of postconcussion symptoms among youth referred to a sports concussion clinic. STUDY DESIGN: A retrospective cohort study was conducted to evaluate several potential predictors of symptom duration via a Cox proportional hazards analyses. The individual postconcussion symptom scores were highly correlated, so these symptoms were analyzed in the statistical model as coefficients derived from principal component analyses. RESULTS: Among 1953 youth with concussion, 1755 (89.9%) had dates of reported symptom resolution. The remainder (10.1%) were lost to follow-up and censored. The median time to recovery was 18 days (range 1-353 days). By 30 days, 72.6% had recovered; by 60 days, 91.4% had recovered; and by 90 days, 96.8% had recovered. Several variables in a multivariate Cox model predicted postconcussion symptom duration: female sex (P < .001, hazard ratio [HR] = 1.28), continued activity participation (P = .02, HR = 1.13), loss of consciousness (P = .03, HR = 1.18), anterograde amnesia (P = .04, HR = 1.15), premorbid headaches (P = .03, HR = 1.15), symptom components from the day of concussion (emotion, P = .03, HR = 1.08), and the day of clinic evaluation (cognitive-fatigue, P < .001, HR = 1.22; cephalalgic, P < .001, HR = 1.27; emotional, P = .05, HR = 1.08; arousal-stimulation, P = .003, HR = 1.1). In univariate analyses, greater symptom scores generally predicted longer symptom durations. Worsening of symptoms from the day of concussion to the day of clinic evaluation also predicted longer recovery (P < .001, HR = 1.59). CONCLUSIONS: Several factors help to predict protracted postconcussion symptom durations among youth referred to a sports concussion clinic.

Post-traumatic headaches correlate with migraine symptoms in youth with concussion.

OBJECTIVE: The term "post-traumatic migraine" (PTM) has been used to describe post-traumatic headaches (PTHs) that have associated migraine features, but studies of this relationship are lacking. The objective of the present study was to determine whether PTH correlates strongly with migraine symptoms among youth with concussion. METHODS: Twenty-three symptoms were analyzed from a retrospective cohort of 1953 pediatric patients with concussion. A principal component analysis (PCA) with oblique Promax rotation was conducted to explore underlying symptom relationships in the full cohort and in subcohorts stratified by the presence (n = 414) or absence (n = 1526) of premorbid headache. RESULTS: The mean patient age was 14.1 years; 63% were male. Headache was the most common postconcussion symptom, acknowledged by 69.4% of patients. When considering the full cohort, the PCA demonstrated clustering of headache with photophobia, phonophobia, nausea, dizziness, and neck pain. Similar clustering was present among patients without premorbid headaches. Repeating the analysis in the patients with preconcussion headaches led to elimination of neck pain from the cluster. CONCLUSIONS: PTH correlates strongly with other migraine symptoms among youth with concussion, regardless of premorbid headaches. This clustering of migraine symptoms supports the existence of PTM as a distinct clinical entity in some patients.

Comparison of semiologies between tilt-induced psychogenic nonsyncopal collapse and psychogenic nonepileptic seizures.

We sought to characterize the clinical features of tilt-induced psychogenic nonsyncopal collapse (PNSC) from a cohort of young patients and to compare the semiologies between PNSC and EEG-confirmed psychogenic nonepileptic seizures (PNES). A PNSC diagnosis was made if a clinical event occurred during tilt-table testing that the patient regarded as fainting, but neither hypotension nor EEG changes were present. A diagnosis of PNSC was made in 17.6% of all patients referred during the 15-month study period. Cohorts with psychogenic nonsyncopal collapse (n=40) and PNES (n=40) did not differ in age (15.5±2.2 versus 14.6±2.7, p=.11) or female gender (80% versus 72.5%, p=.43). Psychogenic nonsyncopal collapse events were briefer than PNES events (median: 45 versus 201.5s, p<.001). Negative motor signs (head drop, body limpness) predominated in PNSC (85% versus 20%, p<.001), while the positive motor signs of convulsion occurred more often with PNES (90% versus 30%, p<.001). Behavioral arrest (25% versus 32.5%, p=.46) and eye closure (85% versus 72.5%, p=.21) did not differ between PNSC and PNES. Patients with PNSC were more likely to be tearful before (30% versus 7.5%, p=.02) and after (62.5% versus 7.5%, p<.001) an event. In conclusion, although overlap exists, the features of PNSC generally appear similar to neurally mediated syncope, while the features of PNES generally appear similar to epileptic seizures. Psychogenic nonsyncopal collapse and PNES likely represent similar disorders that differ primarily by clinical semiologies and referral patterns.

Sweat patterns differ between tilt-induced reflex syncope and tilt-induced anxiety among youth.

PURPOSE: Profound sweating can occur with reflex-syncope and with emotional distress, but little is known about the similarities and differences between these sweat responses when they occur during orthostatic challenge. We sought to characterize and compare the sweat patterns related to tilt-induced syncope, presyncope, anxiety, and normal tilt testing. METHODS: In a prospective observational study, quantitative sweat rate was measured from the abdomen, forearm, ankle, and thigh during head-upright tilt. Sweat characteristics were compared across tilt diagnoses of syncope, presyncope, anxiety, and normal testing. When anxiety and syncope/presyncope occurred during the same study (separated by ≥6 min), both were diagnosed. RESULTS: Our cohort comprised150 patients (15.1 ± 2.3 years; 82.9 % female) with 156 diagnoses: 76 with reflex-syncope, 31 with presyncope, 23 with anxiety, and 26 with normal results. All syncope/presyncope patients and 20 (87 %) of the anxiety patients had corresponding sweat responses. Minimal or negligible sweating occurred among patients with normal tests. Neither basal sweat (19.4 ± 4.7 versus 18.3 ± 3.7 versus 18.5 ± 3.7 nL/min/cm(2)) nor peak sweat (171 ± 47.4 versus 149.4 ± 64.4 versus 154.4 ± 59.2 nL/min/cm(2)) differed between patients with syncope, presyncope, or anxiety, p = .32 and p = .12, respectively. However, the qualitative sweat patterns related to syncope/presyncope (diffuse, smoothly contoured, symmetrical, single peaks) differed considerably from the sweat patterns related to anxiety (heterogeneous, asymmetrical, roughly contoured single-peak, multi-peak, or progressive sweat changes). CONCLUSIONS: The sweat patterns related to syncope/presyncope are distinguishable from the sweat patterns related to anxiety. Recognition of the different sweat patterns can inform how signs and symptoms are interpreted during clinical orthostatic challenge.

Orthostatic Intolerance and Autonomic Dysfunction in Youth With Persistent Postconcussion Symptoms: A Head-Upright Tilt Table Study.

OBJECTIVE: To explore head-upright tilt table (HUT) signs of autonomic dysfunction in a cohort of youth with persistent postconcussion symptoms (PCSs) that include light-headedness and to correlate repeat tilt table results with symptom improvements for those patients found to have postural tachycardia syndrome (POTS) on initial testing. DESIGN: Prospective cohort design. SETTING: Nationwide Children's Hospital, Neurology Clinic. PARTICIPANTS: Thirty-four patients (13-18 years of age) with persistent PCSs. MAIN OUTCOME MEASURES: All patients underwent at least 1 tilt table test. The PCS Interview (PCS-I) and patient ratings of light-headedness and vertigo were used to measure symptom burden. Patients found to have POTS were asked to repeat tilt table testing when PCSs improved or 3 to 6 months after the initial test if symptoms persisted. RESULTS: Twenty-four of the 34 (70.6%) patients had abnormal tilt table results with patients categorized as normal (n = 10), isolated syncope (n = 10), and POTS (n = 14). Patients with POTS had higher PCS-I scores than normal patients (P < 0.001) and higher ratings of light-headedness than both normal patients (P = 0.015) and syncope patients (P = 0.04). Twelve POTS patients underwent repeat tilt table testing, and 9 of 12 (75%) no longer met POTS diagnostic criteria. All patients with resolution of POTS had corresponding improvements in PCSs, including light-headedness and vertigo. CONCLUSIONS: Our study demonstrates a high rate of tilt table abnormalities among youth with persistent PCSs. Several patients with POTS had normalization of tilt table testing when PCSs improved. These findings warrant further research of autonomic dysfunction related to concussion. CLINICAL RELEVANCE: Our study is the first to prospectively characterize autonomic dysfunction in patients with persistent PCSs using HUT testing and to show that the tilt test abnormalities normalize in some patients as PCSs improve.

High school principals' resources, knowledge, and practices regarding the returning student with concussion.

OBJECTIVE: To determine high school principals' self-reported resources, knowledge, and practices regarding the management of students returning to school following concussion. STUDY DESIGN: A cross-sectional survey of public high school principals in the state of Ohio assessed respondent and school demographics, respondent concussion training, school resources, and monitoring and accommodation practices for students with concussion. RESULTS: Of the 695 eligible high school principals, 465 (66.9%) completed the survey. Over one-third of principals (37.2%) had some form of concussion training in the past year. Those with training were more likely to promote training of other school faculty (57.4% vs 30.6%, P < .001). Principals were asked to identify school personnel who are designated as case managers for students with concussion. Schools without a designated case manager were less likely to have an athletic trainer (P < .001) and had fewer students (median 424.5 vs 599) than schools with a case manager. Principals could list at least 1 faculty designee who communicates with health professionals more often for student-athletes than for nonathletes (P < .001). Most principals were willing to provide students with short-term academic accommodations, but 30.1% required a health professional's note prior to making any academic changes. Only 32% of principals reported providing families with a written academic plan following concussion. CONCLUSIONS: Schools differ in their resources and management strategies for students returning to school after concussion. Understanding these differences can help health professionals to overcome potential barriers in managing their school-aged patients with concussion.

The response to ACTH is determined early in the treatment of infantile spasms.

Although adrenocorticotropic hormone is the most commonly used treatment for infantile spasms in the United States, the optimal regimen for this indication is not known. The purpose of this study was to elucidate the optimal adrenocorticotropic hormone treatment duration. We conducted a retrospective chart review of response to adrenocorticotropic hormone among all patients with infantile spasms managed at our institution from January 2009 to September 2013. Treatment response was defined as clinical remission for greater than or equal to 28 days starting at any point within the adrenocorticotropic hormone course and remission of hypsarrhythmia (or definite EEG improvement if hypsarrhythmia was absent at baseline). For responders, the diagnostic and post-treatment EEG tracings were reviewed. Electroclinical remission was achieved in 21 of 39 patients (54%) receiving adrenocorticotropic hormone, including 11/25 (44%) receiving a long course (typically 12 weeks) and 10/14 (71%) receiving a short course (typically four weeks). The mean time to clinical remission was 5.8 days (median: 5 days; range: 1-20 days). Only one patient responded beyond two weeks of treatment. This study provides Class IV evidence that among patients with infantile spasms, the response to adrenocorticotropic hormone is most often determined early in the treatment course. Given the importance of rapid remission, clinicians should consider adding or changing treatment if infantile spasms do not resolve within two weeks of adrenocorticotropic hormone initiation. Further study is needed to determine the optimal adrenocorticotropic hormone regimen for infantile spasms.

How long is too long? The lack of consensus regarding the post-concussion syndrome diagnosis.

BACKGROUND/AIM: A standard definition of Post-concussion Syndrome (PCS) does not exist. The objective was to determine consensus regarding the definition of PCS among physician members of the American College of Sports Medicine (ACSM). METHODS: Physician members of the ACSM were sent an electronic survey to determine opinions regarding the PCS diagnosis. RESULTS: Five hundred and ninety-seven physicians completed the survey. When asked the minimum duration of symptoms required to diagnose PCS, respondents answered: <2 weeks (26.6%), 2 weeks to 1 month (20.4%), 1-3 months (33%) and >3 months (11.1%). Physicians who see ≥10% concussion patients in their practise, as well as physicians whose concussion population consists of >50% paediatric patients, were more likely to require >1 month of symptoms (p < 0.001). When asked the minimum number of symptoms required to diagnose PCS, responses varied: one symptom (55.9%), two symptoms (17.6%), three symptoms (14.6%) and four or more symptoms (3.2%). Respondents from the US were more likely than non-US respondents to require only one symptom for the PCS diagnosis (p = 0.01). CONCLUSIONS: There is a lack of consensus regarding the definition of PCS among physician members of the ACSM. A standard definition would improve consistency in concussion research and in clinical practise.

Abdominal and lower-extremity compression decreases symptoms of postural tachycardia syndrome in youth during tilt table testing.

Little is known about the effectiveness of abdominal and lower-extremity compression in treating postural tachycardia syndrome, particularly in younger patients. This repeated-measures study of 20 young patients with postural tachycardia syndrome demonstrates that compression during head-upright tilt table testing decreases tachycardia (P<.001) and effectively attenuates orthostatic symptoms.

Comparing patient and parent recall of 90-day and 30-day migraine disability using elements of the PedMIDAS and an Internet headache diary.

AIM: The aim of this article is to compare 90-day and 30-day recall of Pediatric Migraine Disability Assessment (PedMIDAS) elements and headache frequency against daily entries from an Internet headache diary among pediatric patients and their parents. METHODS: In a prospective cohort study, patients aged 10-18 years with episodic migraine or probable migraine completed a 90-day Internet-based headache diary that incorporated PedMIDAS questions. Following the 90-day diary period, patients and parents completed modified PedMIDAS instruments to assess 90-day and 30-day recall. Intraclass correlation coefficients (ICC) were calculated to measure recall reliability. The Kruskal-Wallis and Jonckheere-Terpstra tests were used to explore recall accuracy as it relates to each participant's self-reported confidence in recall and to patient age. RESULTS: Fifty-two subjects completed 90 consecutive diary entries. Comparing 30-day to 90-day recall of PedMIDAS elements, ICC scores improved by 26.2% (patients) and 17.5% (parents). Patients had better recall than their parents for all study measures. Self-reported confidence in recall and patient age had limited and inconsistent effects on recall accuracy. CONCLUSION: The optimal recall interval to assess migraine disability must balance recall accuracy with generalizability across a range of headache frequencies. When compared to daily diary entries, recall accuracy of PedMIDAS elements and headache frequency improves at 30 days compared to 90 days. Parent report of migraine disability should not be used as a replacement for patient report.

PedMIDAS-based scoring underestimates migraine disability on non-school days.

OBJECTIVE: The aim of this study is to compare daily Pediatric Migraine Disability Assessment (PedMIDAS)-based scores for headaches occurring on school days vs non-school days and during the school year vs the summer holiday. BACKGROUND: The PedMIDAS is the only instrument validated to assess migraine disability among school-aged children. However, the PedMIDAS may underestimate disability during prolonged school holidays. METHODS: In a prospective cohort study, migraine patients aged 10-18 years completed a 90-day Internet-based headache diary. For each headache day, they answered PedMIDAS-based questions and rated their headache intensity (scale 1-10). PedMIDAS-based scores, headache intensity ratings, and relative headache frequencies were compared for school days vs non-school days and for the school year vs the summer holiday. RESULTS: Fifty-two patients completed 4680 diary entries comprising 984 headache days. The headache frequencies and intensity ratings did not differ between time periods. However, the mean headache disability scores (as measured from PedMIDAS-based questions) were significantly different for school days (0.85) compared to non-school days (0.45), P < .001, and for the school year (0.73) compared to the summer holiday (0.46), P < .016. CONCLUSION: Given similar headache intensities and frequencies, daily PedMIDAS-based scores significantly underestimate headache disability on non-school days. Accordingly, PedMIDAS scoring during the school year may not be comparable to assessments done during the summer holiday. These potential differences must be considered when using the instrument as an outcome measure for clinical trials.

The diagnostic role for susceptibility-weighted MRI during sporadic hemiplegic migraine.

BACKGROUND: Hemiplegic migraine is a rare form of migraine with aura that includes motor weakness. Diagnosis during the first episode can be difficult to make and costly, especially with the sporadic form. CASES: Our study evaluates the ictal magnetic resonance imaging (MRI) features of four sequential pediatric patients during a first-time, sporadic hemiplegic migraine. Susceptibility-weighted imaging (SWI) revealed cerebral venous prominence and increased magnetic susceptibility affecting brain regions that corresponded with each patient's neurologic deficits. Repeat MRI (performed in three patients) following migraine recovery demonstrated resolution of all susceptibility abnormalities. CONCLUSION: When combined with conventional MRI sequences, SWI has diagnostic value in the acute setting of motor weakness and with clinical features consistent with hemiplegic migraine. The sequence may help to further characterize ictal cerebral blood flow changes during the hemiplegic migraine aura.

Symptoms predictive of postural tachycardia syndrome (POTS) in the adolescent headache patient.

OBJECTIVE: To identify symptoms that may predict postural tachycardia syndrome (POTS) among adolescent patients with headache and lightheadedness referred for tilt table testing. BACKGROUND: Individuals with POTS can have a variety of symptoms that impair quality of life. The specific symptoms that help to distinguish the POTS patient in an adolescent headache population have not been determined. METHODS: A group of symptoms was compared among 70 adolescent patients with headache and lightheadedness referred to a pediatric headache clinic for tilt table testing. Every patient completed a symptom questionnaire prior to the tilt table test. The chi-square test was used to compare questionnaire responses between patients found to have POTS and those who did not have POTS. Thirteen symptoms were analyzed. Symptoms that differed statistically between groups were further assessed for sensitivity, specificity, and diagnostic predictive values. RESULTS: Thirty-seven (53%) patients met diagnostic criteria for POTS. Several symptoms differed between the patients found to have POTS and those without POTS. Headache type was not predictive. Vertigo and evening exacerbation of headaches had P values <.05 but did not meet significance after a statistical correction for multiple variables, P ≤ .004 (0.05/13). New-onset motion sickness, dizziness as a headache trigger, and orthostatic headaches had P values <.004 and were relatively sensitive and/or specific for the POTS diagnosis. CONCLUSIONS: While no single clinical symptom or headache type reliably establishes the POTS diagnosis, several symptoms can help to distinguish the POTS patient in an adolescent headache population.

Predictors of cerebral arteriopathy in children with arterial ischemic stroke: results of the International Pediatric Stroke Study.

BACKGROUND: Cerebral arteriopathies, including an idiopathic focal cerebral arteriopathy of childhood (FCA), are common in children with arterial ischemic stroke and strongly predictive of recurrence. To better understand these lesions, we measured predictors of arteriopathy within a large international series of children with arterial ischemic stroke. METHODS AND RESULTS: Between January 2003 and July 2007, 30 centers within the International Pediatric Stroke Study enrolled 667 children (age, 29 days to 19 years) with arterial ischemic stroke and abstracted clinical and radiographic data. Cerebral arteriopathy and its subtypes were defined using published definitions; FCA was defined as cerebral arterial stenosis not attributed to specific diagnoses such as moyamoya, arterial dissection, vasculitis, or postvaricella angiopathy. We used multivariate logistic regression techniques to determine predictors of arteriopathy and FCA among those subjects who received vascular imaging. Of 667 subjects, 525 had known vascular imaging results, and 53% of those (n=277) had an arteriopathy. The most common arteriopathies were FCA (n=69, 25%), moyamoya (n=61, 22%), and arterial dissection (n=56, 20%). Predictors of arteriopathy include early school age (5 to 9 years), recent upper respiratory infections, and sickle cell disease, whereas prior cardiac disease and sepsis reduced the risk of arteriopathy. The only predictor of FCA was recent upper respiratory infection. CONCLUSIONS: Arteriopathy is prevalent among children with arterial ischemic stroke, particularly those presenting in early school age, and those with a history of sickle cell disease. Recent upper respiratory infection predicted cerebral arteriopathy and FCA in particular, suggesting a possible role for infection in the pathogenesis of these lesions.

PHACE syndrome: current knowledge, future directions.

On November 7-8, 2008, physicians gathered in Houston Texas for the first-ever workshop on PHACE syndrome, an important and recently described neurocutaneous syndrome. This article represents a summary of the discussions held at that workshop, which was attended by a broad range of medical specialists.