Publications by authors named "Sarah A Kelley"

10 Publications

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Treatment Practices and Outcomes in Continuous Spike and Wave During Slow Wave Sleep (CSWS): A Multicenter Collaboration.

J Pediatr 2021 Jan 20. Epub 2021 Jan 20.

Department of Pediatrics & Neurology, University of Colorado, Aurora, CO.

Objectives: To determine how Continuous Spike and Wave during Slow Wave Sleep (CSWS) is currently managed and to compare the effectiveness of current treatment strategies using a database from 11 pediatric epilepsy centers in the United States.

Study Design: This retrospective study gathered information on baseline clinical characteristics, CSWS etiology, and treatment(s) in consecutive patients seen between 2014-2016 at 11 epilepsy referral centers. Treatments were categorized as benzodiazepines, steroids, other antiseizure medications (ASMs), or other therapies. Two measures of treatment response [clinical improvement as noted by the treating physician; and EEG improvement] were compared across therapies, controlling for baseline variables.

Results: 81 children underwent 153 treatment trials during the study period (68 trials of benzodiazepines, 25 of steroids, 45 of ASMs, 14 of other therapies). Children most frequently received benzodiazepines (62%) or ASMs (27%) as first line therapy. Treatment choice did not differ based on baseline clinical variables, nor did these variables correlate with outcome. After adjusting for baseline variables, children had a greater odds of clinical improvement with benzodiazepines (OR 3.32, 95%CI 1.57-7.04, P = .002) or steroids (OR 4.04, 95%CI 1.41-11.59, p=0.01) than with ASMs and a greater odds of EEG improvement after steroids (OR 3.36, 95% CI 1.09-10.33, p=0.03) than after ASMs.

Conclusions: Benzodiazepines and ASMs are the most frequent initial therapy prescribed for CSWS in the United States. Our data suggests that ASMs are inferior to benzodiazepines and steroids and support earlier use of these therapies. Multicenter prospective studies that rigorously assess treatment protocols and outcomes are needed.
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http://dx.doi.org/10.1016/j.jpeds.2021.01.032DOI Listing
January 2021

Diagnosing and managing childhood absence epilepsy by telemedicine.

Epilepsy Behav 2021 02 13;115:107404. Epub 2020 Dec 13.

Division of Pediatric Neurology, Department of Neurology, The Johns Hopkins University School of Medicine, Baltimore, MD, United States of America.

The diagnosis of childhood absence epilepsy (CAE) is typically based on history and description of spells, supported by an office-based positive hyperventilation test and confirmed by routine electroencephalography (EEG). In the current coronavirus disease 2019 (COVID-19) pandemic, many pediatric neurologists have switched to telemedicine visits for nonemergent outpatient evaluations. We present a series of children diagnosed as having CAE on the basis of a positive hyperventilation test performed during remote televisits. Several of these children were begun on treatment for CAE prior to obtaining an EEG, with significant seizure reduction. Our series documents the feasibility of CAE diagnosis and management by telemedicine.
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http://dx.doi.org/10.1016/j.yebeh.2020.107404DOI Listing
February 2021

The onset of pediatric refractory status epilepticus is not distributed uniformly during the day.

Seizure 2019 Aug 18;70:90-96. Epub 2019 Jun 18.

Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA. Electronic address:

Purpose: To evaluate whether the onset of pediatric refractory status epilepticus (rSE) is related to time of day.

Method: We analyzed the time of day for the onset of rSE in this prospective observational study performed from June 2011 to May 2019 in pediatric patients (1 month to 21 years of age). We evaluated the temporal distribution of pediatric rSE utilizing a cosinor analysis. We calculated the midline estimating statistic of rhythm (MESOR) and amplitude. MESOR is the estimated mean number of rSE episodes per hour if they were evenly distributed. Amplitude is the difference between MESOR and maximum rSE episodes/hour, or between MESOR and minimum rSE episodes/hour. We also evaluated the temporal distribution of time to treatment.

Results: We analyzed 368 patients (58% males) with a median (p - p) age of 4.2 (1.3-9.7) years. The MESOR was 15.3 (95% CI: 13.9-16.8) and the amplitude was 3.2 (95% CI: 1.1-5.3), p = 0.0024, demonstrating that the distribution is not uniform, but better described as varying throughout the day with a peak in the morning (11am-12 pm) and trough at night (11 pm-12 am). The duration from rSE onset to application of the first non-benzodiazepine antiseizure medication peaked during the early morning (2am-3 am) with a minimum during the afternoon (2 pm-3 pm) (p = 0.0179).

Conclusions: The distribution of rSE onset is not uniform during the day. rSE onset shows a 24-h distribution with a peak in the mid-morning (11am-12 pm) and a trough at night (11 pm-12am).
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http://dx.doi.org/10.1016/j.seizure.2019.06.017DOI Listing
August 2019

Characterization of the Basal Ganglia Using Diffusion Tensor Imaging in Children with Self-Injurious Behavior and Tuberous Sclerosis Complex.

J Neuroimaging 2019 07 6;29(4):506-511. Epub 2019 May 6.

Le Bonheur Children's Hospital and Boling Center for Developmental Disabilities, Memphis, TN.

Background And Purpose: Tuberous sclerosis complex (TSC) is a rare, genetic disease that is associated with multiple manifestations including epilepsy and autism. Self-injurious behaviors (SIBs) also occur in a subset of patients. This study used diffusion tensor imaging (DTI) in children with TSC for quantitative and volumetric analysis of brain regions that have been associated with SIB in other genetic conditions.

Methods: We used DTI to compare 6 children with TSC-associated SIB and 10 children with TSC without SIB. Atlas-based analysis of DTI data and calculation of number of voxels; fractional anisotropy (FA); and mean, axial, and radial diffusivity were performed for multiple regions; DTI measures were summarized using medians and interquartile ranges and were compared using Wilcoxon rank sum tests and false discovery rates (FDRs).

Results: Analysis showed that children with TSC and SIB had reduced numbers of voxels (median) in the bilateral globus pallidus (right: 218 vs. 260, P = .008, FDR = .18; left: 222 vs. 274, P = .002, FDR = .12) and caudate nucleus (right: 712 vs. 896, P = .01, FDR = .26; left: 702 vs. 921, P = .03, FDR = .44) and reduced FA in the bilateral globus pallidus (right: .233 vs. .272, P = .003, FDR = .12; left: .223 vs. .247, P = .004, FDR = .12) and left caudate nucleus (.162 vs. .186, P = .03, FDR = .39) versus children without SIB. No other statistically significant differences were found.

Conclusions: These data support a correlation between lower volumes of the globus pallidus and caudate with SIB in children with TSC.
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http://dx.doi.org/10.1111/jon.12628DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6618151PMC
July 2019

Child Life Services in an Epilepsy Monitoring Unit.

Clin Pediatr (Phila) 2018 10 18;57(11):1269-1274. Epub 2018 Apr 18.

1 Johns Hopkins Hospital, Baltimore, MD, USA.

The goal of this study was to determine the value of a certified child life specialist (CCLS) on the patient and staff experiences in an epilepsy monitoring unit (EMU). We integrated a CCLS into the EMU for all children as well as adults with intellectual disability. We surveyed families to determine the impact of child life services on their stay. EMU staff completed questionnaires to determine perceived impact to their job performance from the integration of the CCLS. All of the families (pediatric and adult patients) who responded to the survey reported the presence of the CCLS improved their hospital experience. Staff reported that the CCLS improved their daily work by allowing them to focus on their assigned medical duties. This preliminary pilot study suggests that CCLS can have a strong impact on the experience of patients and staff in an EMU.
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http://dx.doi.org/10.1177/0009922818769469DOI Listing
October 2018

Bottom-of-sulcus focal cortical dysplasia presenting as epilepsia partialis continua multimodality characterization including 7T MRI.

Childs Nerv Syst 2018 06 14;34(6):1267-1269. Epub 2018 Feb 14.

Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, 1800 Orleans Street, Zayed Tower, Room 4174, Baltimore, MD, 21287, USA.

Introduction: Bottom-of-sulcus focal cortical dysplasias are an under recognized, surgically treatable cause of focal epilepsy. Resection can dramatically reduce the seizure burden for children with refractory epilepsy, or eliminate seizures altogether.

Material And Methods: We report the case and present the results of multimodality evaluation of a 15-year-old young man who presented with long-standing partial epilepsy affecting his right leg, which over the years became refractory to therapy.

Results: High-resolution 3T MRI images acquired as a dedicated epilepsyprotocol were initially interpreted as unremarkable. On further review by an experienced specialist aware of clinical and electroencephalographic findings, a subtle focal cortical dysplasia was identified at the bottom of a sulcus near the medial aspect of the left precentral gyrus. After confirmation of the extent of the lesion with PET and ultra-high field 7T MRI, the patient underwent cortical mapping and focal resection and remains free of seizures.

Coclusions: This case emphasizes the need for a multidisciplinary approach to the evaluation of refractory focal epilepsy in children and highlights the potential role of ultra-high field 7T MRI in identifying the often subtle causative anatomic abnormalities.
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http://dx.doi.org/10.1007/s00381-018-3749-2DOI Listing
June 2018

Author reply: To PMID 22744668.

Authors:
Sarah A Kelley

Neurology 2013 Jan;80(4):421

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http://dx.doi.org/10.1212/wnl.0b013e3182817f5fDOI Listing
January 2013

Comorbidity of migraine in children presenting with epilepsy to a tertiary care center.

Neurology 2012 Jul 27;79(5):468-73. Epub 2012 Jun 27.

Johns Hopkins University, Baltimore, MD, USA.

Objectives: Migraine and epilepsy are 2 of the most common neurologic disorders in children. In this cross-sectional study we investigated a population of children with epilepsy to determine if children with a greater seizure burden or certain epilepsy syndromes had a higher risk of migraines. We also examined how often migraine is addressed and treated in a pediatric epilepsy cohort.

Methods: Between January 2010 and March 2011 we distributed questionnaires regarding headache symptoms and treatment to consecutive children with epilepsy seen in clinic at Johns Hopkins Hospital (400 children were studied). Records were subsequently reviewed for seizure type, age at onset, and treatment.

Results: The prevalence of migraine in our pediatric epilepsy population was 25%, which is greater than reported for children without epilepsy (3%-23%). Migraine was more prevalent in children ≥10 years (p = 0.0009), children with benign epilepsy with centrotemporal spikes (BECTS) (p = 0.003), and children with juvenile myoclonic epilepsy (JME) (p = 0.008). Migraine onset was more likely to have occurred after epilepsy was diagnosed (p = 0.0002), but was not more prevalent in those with intractable epilepsy. Only 50% of patients with weekly or greater migraines had documented discussions regarding headaches with their neurologist.

Conclusion: Migraine was comorbid in one-quarter of children with epilepsy in a tertiary care center. Children who were older or who had BECTS or JME were more likely to have migraines. Migraines were infrequently addressed within the neurology clinic. It is imperative to address comorbid migraine in treating children with epilepsy.
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http://dx.doi.org/10.1212/WNL.0b013e3182617113DOI Listing
July 2012

Metabolic treatments for intractable epilepsy.

Semin Pediatr Neurol 2011 Sep;18(3):179-85

Johns Hopkins Hospital, Baltimore, MD 21287, USA.

When a child on anticonvulsant medications continues to have seizures, what other options should be considered? Over the past 100 years, dietary therapies for the treatment of intractable epilepsy have become more widely recognized, and their use has continued to expand throughout the world. An increasing number of studies has shown efficacy of these metabolic treatments in improving seizure control. Currently, 4 types of dietary therapy are available in the clinic: the classic long chain fatty acid "ketogenic" diet, the medium chain triglyceride diet, the modified Atkins diet, and the low glycemic index treatment. These therapies should be considered earlier in the treatment of intractable epilepsy because they offer a different approach to treatment that has proven efficacious, tolerable, and cost-effective.
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http://dx.doi.org/10.1016/j.spen.2011.06.004DOI Listing
September 2011

Doose syndrome (myoclonic-astatic epilepsy): 40 years of progress.

Dev Med Child Neurol 2010 Nov 16;52(11):988-93. Epub 2010 Aug 16.

Department of Neurology, The Johns Hopkins Hospital, Baltimore, MD, USA.

Doose syndrome, otherwise traditionally known as myoclonic-astatic epilepsy, was first described as a unique epilepsy syndrome by Dr Hermann Doose in 1970. In 1989, the International League Against Epilepsy classified it formally as a symptomatic generalized epilepsy, and 20 years later it was renamed 'epilepsy with myoclonic-atonic seizures'. In this review, we discuss the components of this unique disorder including its incidence, clinical features, and electroencephalographic findings. Recent evidence has suggested possible genetic links to the GEFS+ (generalized epilepsy with febrile seizures plus) family, and, additionally, some children with structural brain lesions can mimic the Doose syndrome phenotype. Treatment strategies such as corticosteroids, ethosuximide, and valproate have been described as only partially effective, but newer anticonvulsants, such as levetiracetam and zonisamide, may provide additional seizure control. The most effective treatment reported to date appears to be the ketogenic diet. Prognosis is quite varied in this disorder; however, many children can have a remarkably normal neurodevelopmental outcome.
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http://dx.doi.org/10.1111/j.1469-8749.2010.03744.xDOI Listing
November 2010