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Pediatric-onset multiple sclerosis (POMS) is a rare neuroinflammatory and neurodegenerative disease that has a significant impact on long-term physical and cognitive patient outcomes. A small percentage of multiple sclerosis (MS) diagnoses occur before the age of 18 years. Before treatment initiation, a careful differential diagnosis and exclusion of other similar acquired demyelinating syndromes such as anti-aquaporin-4-associated neuromyelitis optica spectrum disorder (AQP4-NMOSD) and myelin oligodendrocyte glycoprotein antibody spectrum disorder (MOGSD) is warranted. The recent 2017 changes to the McDonald criteria can successfully predict up to 71% of MS diagnoses and have good specificity of 95% and sensitivity of 71%. Additional measures such as the presence of T1-weighted hypointense lesions and/or contrast-enhancing lesions significantly increase the accuracy of diagnosis. In adults, early use of disease-modifying therapies (DMTs) is instrumental to a better long-term prognosis, including lower rates of relapse and disability worsening, and numerous FDA-approved therapies for adult-onset MS are available. However, unlike their adult counterparts, the development, testing, and regulatory approval of POMS treatments have been significantly slower and hindered by logistic and/or ethical considerations. Currently, only two MS DMTs (fingolimod and teriflunomide) have been tested in large phase III trials and approved by regulatory agencies for use in POMS. First-line therapies not approved by the FDA for use in children (interferon-β and glatiramer acetate) are also commonly used and result in a significant reduction in inflammatory activity when compared with non-treated POMS patients. An increasing number of POMS patients are now treated with moderate efficacy therapies such as dimethyl fumarate and high-efficacy therapies such as natalizumab, anti-CD20 monoclonal antibodies, anti-CD52 monoclonal antibodies, and/or autologous hematopoietic stem cell transplantation. These high-efficacy DMTs generally provide additional reduction in inflammatory activity when compared with the first-line medications (up to 62% of relapse-rate reduction). Therefore, a number of phase II and III trials are currently investigating their efficacy and safety in POMS patients. In this review, we discuss potential changes in the regulatory approval process for POMS patients that are recommended for DMTs already approved for the adult MS population, including smaller sample size for pharmacokinetic/pharmacodynamic studies, MRI-centered primary outcomes, and/or inclusion of teenagers in the adult trials.

Background and Purpose: To assess whether initial imaging characteristics independently predict 1-year neurological outcomes in childhood arterial ischemic stroke patients. Methods: We used prospectively collected demographic and clinical data, imaging data, and 1-year outcomes from the VIPS study (Vascular Effects of Infection in Pediatric Stroke). In 288 patients with first-time stroke, we measured infarct volume and location on the acute magnetic resonance imaging studies and hemorrhagic transformation on brain imaging studies during the acute presentation. Neurological outcome was assessed with the Pediatric Stroke Outcome Measure. We used univariate and multivariable ordinal logistic regression models to test the association between imaging characteristics and outcome. Results: Univariate analysis demonstrated that infarcts involving uncinate fasciculus, angular gyrus, insular cortex, or that extended from cortex to the subcortical nuclei were significantly associated with poorer outcomes with odds ratios ranging from 1.95 to 3.95. All locations except the insular cortex remained significant predictors of poor outcome on multivariable analysis. When infarct volume was added to the model, the locations did not remain significant. Larger infarct volumes and younger age at stroke onset were significantly associated with poorer outcome, but the strength of the relationships was weak. Hemorrhagic transformation did not predict outcome. Conclusions: In the largest pediatric arterial ischemic stroke cohort collected to date, we showed that larger infarct volume and younger age at stroke were associated with poorer outcomes. We made the novel observation that the strength of these associations was modest and limits the ability to use these characteristics to predict outcome in children. Infarcts affecting specific locations were significantly associated with poorer outcomes in univariate and multivariable analyses but lost significance when adjusted for infarct volume. Our findings suggest that infarcts that disrupt critical networks have a disproportionate impact upon outcome after childhood arterial ischemic stroke.

Background and ObjectivesSpinal muscular atrophy (SMA) was added to the Recommended Uniform Screening Panel in July 2018 largely on the basis of the availability and efficacy of newly approved disease-modifying therapies. New York State (NYS) started universal newborn screening for SMA in October 2018. The authors report the findings from the first 3 years of screening.MethodsStatewide neonatal screening was conducted using DNA extracted from dried blood spots using a real-time quantitative PCR assay. Retrospective follow-up data were collected from 9 referral centers across the state on 34 infants.ResultsIn the first 3 years since statewide implementation, nearly 650,000 infants have been screened for SMA. Thirty-four babies screened positive and were referred to a neuromuscular specialty care center. The incidence remains lower than previously predicted. The majority (94%), including all infants with 2–3 copies of survival motor neuron (SMN) 2, have received treatment. Among treated infants, the overwhelming majority (94%; 30/32) have received gene replacement. All infants in this cohort with 3 copies of SMN2 are clinically asymptomatic posttreatment based on early clinical follow-up data. Infants with 2 copies of SMN2 are more variable in their outcomes. Electrodiagnostic outcomes data obtained from a subgroup of patients (n = 11) demonstrated either improvement or no change in compound muscle action potential (CMAP) amplitude at last clinical follow-up compared with pretreatment baseline. Most infants were treated before 6 weeks of age (median = 34.5 days of life; range 11–180 days). Delays and barriers to treatment identified by treating clinicians followed 2 broad themes: medical and nonmedical. Medical delays most commonly reported were the presence of AAV9 antibodies and elevated troponin I levels. Nonmedical barriers included delays in obtaining insurance and insurance policies regarding specific treatment modalities.DiscussionThe findings from the NYS cohort of newborn screen-identified infants are consistent with other reports of improved outcomes from early diagnosis and treatment. Additional biomarkers of motor neuron health including EMG can potentially be helpful in detecting preclinical decline.

Spinal muscular atrophy (SMA) was added to the Recommended Uniform Screening Panel (RUSP) in July 2018, following FDA approval of the first effective SMA treatment, and demonstration of feasibility of high-throughput newborn screening using a primary molecular assay. SMA newborn screening was implemented in New York State (NYS) on 1 October 2018. Screening was conducted using DNA extracted from dried blood spots with a multiplex real-time quantitative polymerase chain reaction (qPCR) assay targeting the recurrent SMN1 exon 7 gene deletion. During the first year, 225,093 infants were tested. Eight screened positive, were referred for follow-up, and confirmed to be homozygous for the deletion. Infants with two or three copies of the SMN2 gene, predicting more severe, earlier-onset SMA, were treated with antisense oligonucleotide and/or gene therapy. One infant with ≥4 copies SMN2 also received gene therapy. Newborn screening permits presymptomatic SMA diagnosis, when treatment initiation is most beneficial. At 1 in 28,137 (95% confidence interval [CI]: 1 in 14,259 to 55,525), the NYS SMA incidence is 2.6- to 4.7-fold lower than expected. The low SMA incidence is likely attributable to imprecise and biased estimates, coupled with increased awareness, access to and uptake of carrier screening, genetic counseling, cascade testing, prenatal diagnosis, and advanced reproductive technologies.

Alice in Wonderland syndrome is a disorienting neurological condition that affects human perception to the senses of vision, hearing, touch, sensation, and the phenomenon of time. Individuals affected with Alice in Wonderland syndrome can experience alterations in their perception of the size of objects or their own body parts, known as metamorphopsias. It is known to occur in conditions including migraine, epilepsy, and certain intoxicants and infectious diseases. The name refers to Lewis Carrol's well-known children's book Alice's Adventures in Wonderland, in which the title character experiences alterations of sensation in which she felt that her body had grown too tall or too small, or parts of her body were changing shape, size, or relationship to the rest of her body. The syndrome was described in 1952 by Caro Lippman, and given its name in 1955 by John Todd. The metamorphopsias characteristic of this condition are also sometimes referred to as Lilliputian hallucinations, a reference to the fictional island of Lilliput in the novel Gulliver's Travels, written by Jonathan Swift in 1726. As such, many literary and medical publications have roots in the description of this syndrome. The purpose of this review is to summarize the literary and historical significance of Alice in Wonderland syndrome, as well as to provide the reader with a medical overview of the condition.

To characterize behavior of 2-year-old children based on the severity of bronchopulmonary dysplasia (BPD).We studied children born at 22-26 weeks of gestation and assessed at 22-26 months of corrected age with the Child Behavior Checklist (CBCL). BPD was classified by the level of respiratory support at 36 weeks of postmenstrual age. CBCL syndrome scales were the primary outcomes. The relationship between BPD grade and behavior was evaluated, adjusting for perinatal confounders. Mediation analysis was performed to evaluate whether cognitive, language, or motor skills mediated the effect of BPD grade on behavior.Of 2310 children, 1208 (52%) had no BPD, 806 (35%) had grade 1 BPD, 177 (8%) had grade 2 BPD, and 119 (5%) had grade 3 BPD. Withdrawn behavior (P .001) and pervasive developmental problems (P .001) increased with worsening BPD grade. Sleep problems (P = .008) and aggressive behavior (P = .023) decreased with worsening BPD grade. Children with grade 3 BPD scored 2 points worse for withdrawn behavior and pervasive developmental problems and 2 points better for externalizing problems, sleep problems, and aggressive behavior than children without BPD. Cognitive, language, and motor skills mediated the effect of BPD grade on the attention problems, emotionally reactive, somatic complaints, and withdrawn CBCL syndrome scales (P values .05).BPD grade was associated with increased risk of withdrawn behavior and pervasive developmental problems but with decreased risk of sleep problems and aggressive behavior. The relationship between BPD and behavior is complex. Cognitive, language, and motor skills mediate the effects of BPD grade on some problem behaviors.

This is a prospective study of children with Lennox-Gastaut syndrome receiving clobazam as adjunctive therapy. This pilot study aims to examine medication effectiveness as it relates to seizure reduction, as well as improvement in parent-reported behavior and quality of life (QOL).Ten patients with Lennox-Gastaut syndrome aged 3-11 years were enrolled in this 6-week, 4 phase study. Seizure frequency, QOL, and Aberrant Behavior Checklist questionnaires were evaluated at 0, 2, 6, and 10 weeks during the study.Patients showed improvement on indices of QOL, including physical activities (62.5%), well-being (37.5%), cognition (87.5%), social activities (37.5%), behavior (87.5%), general health (50%), and overall QOL (87.5%). The Quality of Life in Childhood Epilepsy (QOLCE) questionnaire revealed significant improvement in cognition (There was improvement in all patients with documented baseline seizures (8/10), with 5 showing significant improvement (95%-100% reduction) and 3 showing minor improvement (7%-23% reduction). Statistically significant improvement in areas of cognition, social activities, behavior, and overall QOL were seen. An overall trend toward a positive well-being was seen in our patients with clobazam, as adjunct therapy for Lennox-Gastaut syndrome in children.

Objective To investigate if magnetic resonance imaging (MRI) is an accurate predictor for death or moderate-severe disability at 18-22 months of age among infants with neonatal encephalopathy in a trial of cooling initiated at 6-24 hours. Study design Subgroup analysis of infants ≥36 weeks of gestation with moderate-severe neonatal encephalopathy randomized at 6-24 postnatal hours to hypothermia or usual care in a multicenter trial of late hypothermia. MRI scans were performed per each center's practice and interpreted by 2 central readers using the Eunice Kennedy Shriver National Institute of Child Health and Human Development injury score (6 levels, normal to hemispheric devastation). Neurodevelopmental outcomes were assessed at 18-22 months of age. Results Of 168 enrollees, 128 had an interpretable MRI and were seen in follow-up (n = 119) or died (n = 9). MRI findings were predominantly acute injury and did not differ by cooling treatment. At 18-22 months, death or severe disability occurred in 20.3%. No infant had moderate disability. Agreement between central readers was moderate (weighted kappa 0.56, 95% CI 0.45-0.67). The adjusted odds of death or severe disability increased 3.7-fold (95% CI 1.8-7.9) for each increment of injury score. The area under the curve for severe MRI patterns to predict death or severe disability was 0.77 and the positive and negative predictive values were 36% and 100%, respectively. Conclusions MRI injury scores were associated with neurodevelopmental outcome at 18-22 months among infants in the Late Hypothermia Trial. However, the results suggest caution when using qualitative interpretations of MRI images to provide prognostic information to families following perinatal hypoxia–ischemia. Trial registration Clinicaltrials.gov: NCT00614744 .

Background Acute disseminated encephalomyelitis (ADEM) is a demyelinating disorder that is usually self-limited. Recent studies have suggested ongoing neurological deficits and neurocognitive impairment in these patients. Little information on the correlation of clinical and neuroimaging markers in ADEM is available. We examined potential clinical factors (e.g., age of onset, acute symptom duration, magnetic resonance imaging [MRI] lesions) and their relation to neurocognitive and psychosocial outcomes. Methods This is a retrospective chart review of consecutive pediatric patients diagnosed with ADEM between 2006 and 2012. Patients were evaluated with standard neurological assessment, MRI of the brain, and neuropsychological evaluation. Results Twenty-three patients with ADEM with average age at neuropsychological assessment of 10.1 years (±3.50) were included. Five (22.7%) patients were impaired on three or more neurocognitive measures. Psychosocial problems were reported in 20%-40% of patients. Earlier age of onset was correlated with poorer sustained attention and psychosocial problems, whereas acute symptom duration and Expanded Disability Status Scale were not. MRI outcomes were correlated with psychosocial outcomes but not neuropsychological findings. Conclusions Our findings suggest lingering cognitive and psychosocial deficits in children with a history of ADEM. Clinical features and MRI findings correlated more strongly with psychosocial outcomes than cognitive functioning. Further studies are needed to confirm relationships and other possible contributing factors to lingering deficits.

Background and Purpose— Although arteriopathies are the most common cause of childhood arterial ischemic stroke, and the strongest predictor of recurrent stroke, they are difficult to diagnose. We studied the role of clinical data and follow-up imaging in diagnosing cerebral and cervical arteriopathy in children with arterial ischemic stroke. Methods— Vascular effects of infection in pediatric stroke, an international prospective study, enrolled 355 cases of arterial ischemic stroke (age, 29 days to 18 years) at 39 centers. A neuroradiologist and stroke neurologist independently reviewed vascular imaging of the brain (mandatory for inclusion) and neck to establish a diagnosis of arteriopathy (definite, possible, or absent) in 3 steps: (1) baseline imaging alone; (2) plus clinical data; (3) plus follow-up imaging. A 4-person committee, including a second neuroradiologist and stroke neurologist, adjudicated disagreements. Using the final diagnosis as the gold standard, we calculated the sensitivity and specificity of each step. Results— Cases were aged median 7.6 years (interquartile range, 2.8–14 years); 56% boys. The majority (52%) was previously healthy; 41% had follow-up vascular imaging. Only 56 (16%) required adjudication. The gold standard diagnosis was definite arteriopathy in 127 (36%), possible in 34 (9.6%), and absent in 194 (55%). Sensitivity was 79% at step 1, 90% at step 2, and 94% at step 3; specificity was high throughout (99%, 100%, and 100%), as was agreement between reviewers (κ=0.77, 0.81, and 0.78). Conclusions— Clinical data and follow-up imaging help, yet uncertainty in the diagnosis of childhood arteriopathy remains. This presents a challenge to better understanding the mechanisms underlying these arteriopathies and designing strategies for prevention of childhood arterial ischemic stroke.

SUMMARY Pediatric-onset multiple sclerosis is relatively rare and research regarding disease characteristics is in its infancy. Regardless, there are a growing number of studies finding early cognitive deficits in this population. There are some similarities in outcomes to those seen in the adult-onset multiple sclerosis population, but also several important differences. With specific regard to cognitive functioning in pediatric-onset multiple sclerosis, there is evidence of deficit in nearly a third of patients, with impairment primarily in areas of processing speed, visual–spatial processing and language. There are additional findings of fatigue and depression that impact functional outcomes requiring further attention in assessment and treatment considerations. This paper also explores other areas requiring increased focus, including treatment and outcomes, neuroimaging and additional disease-modifying factors (comorbidities, socioeconomic status, race and so on).

This study evaluated the antiplatelet effects of clopidogrel (CPG) in patients sustaining acute ischemic stroke who were already receiving chronic outpatient aspirin therapy (81-325 mg/day). Platelet function was measured using 3 different "point-of-care" platelet function analyzers: the Thrombelastograph hemostasis system, the Accumetrics VerifyNow system, and the Chronolog 570VS impedance aggregometer. Platelet function was assessed before administration of a 300-mg CPG loading dose and again at 26 hours and 64 hours after this loading dose along with a 75-mg daily maintenance dose. All 3 instruments detected marked inhibition of platelet function at 26 hours and 64 hours after CPG administration. There were significant variations among the 3 instruments in monitoring antiplatelet responses to aspirin and CPG; however, these variations were eliminated when the platelet function results were corrected for baseline platelet variability. The percentage of patients who were poor responders to CPG after switching from aspirin depended on the measurement instrument used, but was higher at 26 hours after CPG administration than at 64 hours after CPG administration. Our findings indicate that poor response to antiplatelet agents in general, and to CPG in particular, is a function of the measuring instrument. The correction for baseline platelet variability results in similar levels of platelet inhibition measured by the 3 platelet function analyzers. Future studies are warranted to examine the association between ex vivo CPG-induced platelet inhibition and clinical outcomes in patients with ischemic stroke.

Purpose To study the usefulness of EEG in the diagnosis of lissencephaly, a rare cortical developmental disorder associated with abnormal cellular proliferation. Currently, the clinical emphasis is placed on the radiological and genetic aspects for the diagnosis of lissencephaly. Methods This is a retrospective review of consecutive EEG recordings and imaging data from 14 children, with the diagnosis of lissencephaly, who were admitted from January 1998 to January 2010. All EEG recordings were performed with the 10–20 system of electrode placement, in both awake and sleep states. All EEG recordings were reviewed using anterior-posterior bipolar and transverse montages and then they were interpreted blindly, with respect to the imaging and genetic investigations for each patient. Results All children showed one of the three characteristic EEG patterns reported in the literature of lissencephaly. The EEG pattern I, showed an anterior posterior gradient that corresponded to the severity of the imaging study abnormality. All patients were on two or more AEDs and reported to continue having active epilepsy. Conclusion In a child with clinical characteristics of lissencephaly, one of these three reported EEG patterns can prove useful in making the diagnosis very probable, preceding imaging and genetic testing.

Levetiracetam given via intravenous administration has been shown to be an effective alternative in adults with epilepsy when oral administration is not feasible. This study was a prospective single-arm, multicenter study to assess tolerability, safety, and pharmacokinetics of intravenous levetiracetam in children with epilepsy. Children with epilepsy ages 1 month to 16 years requiring intravenous levetiracetam were enrolled. Assessments included vital signs, electrocardiogram, hematology, chemistry, plasma concentrations of antiepileptic medications, weight, physical/neurological examinations, and pharmacokinetics. A total of 52 patients were enrolled. Mild to moderate treatment-emergent adverse events occurred in 63%, the most frequent being pyrexia and dry mouth. Most other treatment-emergent adverse events were considered unrelated to intravenous levetiracetam administration. Therefore, intravenous levetiracetam in the acute setting was overall well tolerated in children 1 month to 16 years.

Background: The ketogenic diet is an effective non-pharmacologic treatment for medically resistant epilepsy. The aim of this study was to identify any predictors that may influence the response of ketogenic diet. Methods: A retrospective chart review for all patients with medically resistant epilepsy was performed at a tertiary care epilepsy center from 1996 to 2012. Patient- and diet-related variables were evaluated with respect to seizure reduction at 1, 3, 6, 9 and 12-month intervals and divided into four possible outcome classes. Results: Sixty-three patients met inclusion. Thirty-seven (59%) reported >50% seizure reduction at 3 months with 44% and 37% patients benefiting at 6-month and 12-month follow up, respectively. A trend toward significant seizure improvement was noted in 48% patients with seizure onset >1 year at 12-month (p = 0.09) interval and in 62% patients with >10 seizure/day at 6-month interval (p = 0.054). An ordinal logistic regression showed later age of seizure to have higher odds of favorable response at 1-month (p = 0.005) and 3-month (p = 0.013) follow up. Patients with non-fasting diet induction were more likely to have a favorable outcome at 6 months (p = 0.008) as do females (p = 0.037) and those treated with higher fat ratio diet (p = 0.034). Conclusion: Our study reports the effectiveness of ketogenic diet in children with medically resistant epilepsy. Later age of seizure onset, female gender, higher ketogenic diet ratio and non-fasting induction were associated with better odds of improved seizure outcome. A larger cohort is required to confirm these findings.

Radiation optic neuropathy is a devastating form of vision loss that can occur months to years after radiation therapy for tumors and other lesions located in close proximity to the visual pathways. We present the case of a 24-year-old woman who underwent external beam radiation for treatment of a tectal pilocytic astrocytoma, and 5 years later she developed bilateral radiation optic neuropathy and radiation necrosis of the right temporal lobe. We opted to treat her with intravenous bevacizumab with 3 doses every 3 weeks, as well as dexamethasone and pentoxifylline. After the first infusion of bevacizumab, the patient noted improvement in vision and color vision, and a follow-up magnetic resonance imaging study showed that the previous enhancement of the optic nerves and chiasm was diminishing. Her vision improved dramatically and has remained stable over a 3-year period.

Little is known about the neurologic complications of the 2009 Influenza-A H1N1 epidemic in children. We present a retrospective analysis of children evaluated at a tertiary children’s hospital who tested positive for H1N1 with neurologic complications. A total of 164 children tested positive for H1N1. Thirty-one of these patients (19%) were evaluated and discharged from the emergency department. Thirty-nine (24%) were treated in the intensive care unit, the remaining 94 (57%) were treated in medical in-patient units. Six subjects died (3.7%). Neurologic complications identified included headache, encephalitis, polyneuropathy, seizures, and malignant hyperthermia. The rate of neurologic complications in this cohort of patients who tested positive for H1N1 was 19%. The incidence of serious neurologic complications was 3%, with another 3% of patients who experienced rapid clinical deterioration and subsequently died. Our observation of neurologic complications associated with 2009 influenza-A H1N1 epidemic suggests the need for clinical vigilance during future influenza epidemics.

A 2-week-old girl, who was born at term without complications, is brought to her primary pediatrician after developing diarrhea. According to the mother, she has been having increasingly frequent watery stools since yesterday. The pediatrician notes a small, 1-cm reddish-brown papule on the right deltoid area. This lesion is unchanged from the initial assessment of the girl in the newborn nursery. The infant is otherwise doing well and because she can tolerate oral hydration, she is sent home with instructions for oral rehydration. Two weeks later, the girl returns to the pediatrician's office because of recurrence of diarrhea. A chance finding during the physical examination helps to make the diagnosis. A term baby girl is born via cesarean section to a 31-year-old G2P1, now P2, woman. The cesarean section is performed because of failed induction. Results of the maternal medical history and all prenatal screening tests are negative. Maternal blood is A+ and antibody-negative. An older sibling had neonatal hyperbilirubinemia. At birth, the infant displays significant hypotonia and diminutive respiratory effort. Apgar scores are 7 and 9 at 1 and 5 minutes, respectively. Positive-pressure ventilation is applied for 30 seconds, at which point the infant cries spontaneously. The only finding of note on the newborn examination at approximately 5 hours after birth is mild facial icterus. At 9 hours after birth, the newborn's transcutaneous bilirubin (TcB) is 11.2 mg/dL (191.6 μmol/L). Because of this substantially elevated TcB value, a direct antiglobulin test (DAT), complete blood count (CBC) with differential count, total serum bilirubin (TsB), and blood type of the newborn are ordered. Triple phototherapy is initiated. The noteworthy laboratory findings include: blood type of O+, strongly positive (4+) DAT, hemoglobin of 13 g/dL (130 g/L), hematocrit of 37% (0.37), white blood cell count of 30×103/μL (30×109/L), …

OBJECTIVE: To determine the outcome of preterm infants whose cystic periventricular leukomalacia “disappeared” on serial screening cranial imaging studies. STUDY DESIGN: Infants ≤26 weeks of gestation born between 2002 and 2012 who had cranial imaging studies at least twice, the most abnormal study at

Henoch-Schonlein purpura (HSP) is a vasculitic syndrome which commonly involves small vessels of the skin, joints, intestines, and kidney. Renal involvement is the greatest cause of long-term morbidity and mortality and is identified by proteinuria and/or hematuria. Hypertension is an uncommon complication of renal involvement in HSP. Its occurrence is almost universally associated with other evidence of nephritis. Hypertension resistant to b-adrenergic blockade, calcium channel blocking agents, and vasodilators may occur early in the course of HSP in the absence of other evidence of renal disease. We report two children with Henoch-Schonlein purpura who had significant hypertension resistant to treatment with B-adrenergic blocking agents and vasodilators, one of whom had no other evidence of renal disease. In one child, the hypertension responded to therapy with captopril, suggesting a reninangiotensin mediated mechanism for the hypertension. In both children, initiation of corticosteroid therapy resulted in prompt resolution of hypertension, permitting the discontinuation of antihypertensive medications.

1. Osman Farooq, MD* 2. Zebunnissa Memon, MD† 3. Nicholas Saikali, MD‡ 4. Thomas J. Langan, MD* 1. *Department of Pediatric Neurology, University of Buffalo, SUNY, Buffalo, NY. 2. †Department of Pediatrics, University of Buffalo, SUNY, Buffalo, NY. 3. ‡Department of Neurology, University of Buffalo, SUNY, Buffalo, NY. A 4-year-old boy presents to the emergency department (ED) with a 10-day history of headaches. He was seen in the same ED 1 week before for a 2- to 3-day history of headache. The patient's headache improved after he was given acetaminophen, and he was discharged from the ED with instructions to parents to administer acetaminophen on an as-needed basis. However, over the following week, the headaches increased in frequency and severity, and the patient was brought to his pediatrician complaining of global headaches, difficulty reading at school, and difficulty walking up and down stairs. The patient was referred for further evaluation. On assessment, vital signs are within normal limits. Physical examination is significant for generalized somnolence, right optic disc pallor, bitemporal hemianopsia, and a slight left-sided facial droop. Mild weakness is noted in the left upper extremity, both proximally and distally. Deep tendon reflexes are appropriate and symmetrical in all extremities. A computed tomography (CT) scan of the head (Fig 1) suggests the diagnosis. Figure 1. CT scan of the head of 4-year-old male with persistent headache. The CT scan of the head (Fig 1) revealed a midline hypodense cystic mass in the sellar region with areas of hyperdensity, suggestive of calcifications. Magnetic resonance imaging (MRI) of the brain further revealed an enhancing heterogeneous mass with solid and cystic components (Fig 2). Based on the location and appearance of the mass, the patient was strongly suspected of having a craniopharyngioma and scheduled for surgical resection. Figure 2. Axial, coronal, and sagittal views of MRI with contrast revealing a heterogeneously enhancing solid and cystic mass extending upward from …

We report a child who developed agitation and choreoathetoid movements with azithromycin therapy on 2 separate occasions. In both instances, the symptoms resolved when the antibiotic was discontinued. By means of the Naranjo adverse drug reaction probability scale, we classified this event as a probable adverse drug reaction (score of 6 points). To our knowledge, this is the first published case of azithromycin-induced agitation with choreoathetosis. Because this is a widely used medication for many common infectious conditions, including otitis media and pneumonia, this potential serious adverse reaction should be considered.

Paragonimiasis is a parasitic disease that typically produces a subacute to chronic inflammatory disease of the lung. Although rare in the United States, paragonimiasis is sporadically observed in the immigrant population. Rarely, paragonimiasis can affect the nervous system. This infection is even more unusual in the pediatric population, and therefore can be challenging to diagnose.Here we present a child with cerebral paragonimiasis. She presented with new onset seizures in the setting of a febrile illness. Magnetic resonance imaging of the brain with contrast revealed a ring-enhancing lesion within the right frontal lobe and a second lesion in the left parietal lobe extending from the cortex to the centrum semiovale. Extensive evaluation including stool ova and parasite analysis confirmed the diagnosis of Paragonimus westermani. She was treated with praziquantel and prednisone and improved both clinically and radiographically.Cerebral paragonimiasis is diagnosable and treatable and therefore is important to consider in the differential of immigrants presenting with cavitary lung lesions and central nervous system findings.

1. Osman Farooq, MD* 2. Ayesha Kirmani, MBBS† 3. Nitin Agarwal, MD* 4. Hema Purushotham, MD* 5. Amit Kandel, MD‡ 1. *Department of Pediatric Neurology, University of Buffalo, The State University of New York, Buffalo, NY. 2. †Liaqat University of Medical and Health Sciences, Jamshoro, Pakistan. 3. ‡Department of Neurology, University of Buffalo, The State University of New York, Buffalo, NY. * Abbreviations: ACTH: : adrenocorticotropin hormone ECMO: : extracorporeal membrane oxygenation ED: : emergency department EEG: : electroencephalography A 6-month-old boy with a history of seizures presents to the emergency department (ED) with new “strange body movements.” His mother first noticed this behavior 2 weeks ago, when his arms and legs would be held in a flexed position, lasting 1 to 2 seconds at a time (Figure 1). She said that these did not look like his typical seizures, and he remained alert and responsive during the episodes. After first witnessing these episodes 2 weeks ago, she did not notice them again until 1 week before presentation, at which time they began occurring every other day, increasing in frequency to 3 times per day, at times with full body flexing. Figure 1. Click here to view the video. On physical examination, his vital signs are within normal limits. He is smiling and playful. On motor examination, spontaneous movements of all extremities are seen but are slightly decreased on the left compared with the right. Bilateral fisting is noted, with increased tone in the left upper and lower extremities. Axial tone is mildly decreased for age. His history was notable for a cardiorespiratory arrest at 1 month of age secondary to respiratory syncytial virus bronchiolitis. At that time, he was resuscitated for 30 minutes, then given extracorporeal membrane oxygenation (ECMO). While receiving ECMO, he was noted to have episodes of left eyelid and arm twitching along with lip smacking. Electroencephalography (EEG) revealed status epilepticus. His seizures were pharmacologically controlled with phenobarbital and fosphenytoin. The patient was weaned off ECMO after 7 days. Subsequent EEGs revealed nonspecific abnormalities but no seizure activity. Magnetic resonance imaging, performed 12 days after the …

Osman Farooq1*, David Hojnacki2, Lucia Balos3and Bianca Weinstock-Guttman2 1Women & Children Hospital of Buffalo, SUNY University of Buffalo, USA 2Jacobs MS Center, SUNY University at Buffalo, USA 3Department of Pathology, SUNY University at Buffalo, USA *Corresponding author: Osman Farooq MD, Women & Children Hospital of Buffalo, Department of Neurology, 219 Bryant Street, Buffalo, NY 14222, USA, Tel: 716-878-7840; E-mail: ofarooq3@buffalo.edu

A previously healthy 13-year-old boy presented with left arm and face weakness. Brain MRI (figure 1, axial T1 postcontrast) revealed an enhancing mass within the right centrum semiovale, and a second non-enhancing lesion in the left occipital region (not shown). Lumbar puncture revealed five oligoclonal bands, giving a working diagnosis of a demyelinating disorder. Intravenous methylprednisolone …