Quality improvement in neurology: Child neurology quality measure set

Patel, Anup D.; Berg, Anne T.; Billinghurst, Lori; Fain, D.; Fecske, Erin; Feyma, Tim; Grinspan, Zachary M.; Houtrow, Amy J.; Kothare, Sanjeev V.; Kumar, Gogi; Lee, Erin; Monduy, Migvis; Morita, Diego; Szperka, Christina L.; Victorio, M. Cristina; Yeh, Ann; Buchhalter, Jeffrey

doi:10.1212/wnl.0000000000004806

Cited by 42 publications

(39 citation statements)

References 38 publications

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“…Effective treatment is not with standard antiseizure medications but with steroids (prednisone, prednisolone) or hormones (corticotropin [ACTH]) or vigabatrin. Guidelines, recommendations, and most recently, quality metrics provide strong support for rapid, optimum treatment of this condition . Early intervention may improve longer‐term developmental outcomes, as suggested in the United Kingdom Infantile Spasms Study trial although the underlying etiologies also have a substantial impact .…”

Section: Discussionmentioning

confidence: 99%

“…Guidelines, recommendations, and most recently, quality metrics provide strong support for rapid, optimum treatment of this condition. [14][15][16]33 Early intervention may improve longer-term developmental outcomes, as suggested in the United Kingdom Infantile Spasms Study trial, 34 although the underlying etiologies also have a substantial impact. 21,34 The potential for modifying disease progression through preemptive interventions has led to the pretreatment trial now underway in the tuberous sclerosis community, 3 and the Child Neurology Foundation's "STOP Spasms" campaign.…”

Section: Discussionmentioning

confidence: 99%

“…A Canadian study demonstrated the poor long-term outcomes in this group, with 24% mortality in the first 2-3 decades of life and refractory seizures and disability in the majority of the remainder. 7 West syndrome/infantile spasms (WS/IS), the most common of the severe epilepsies that occur in early life, has been the focus of considerable research, [8][9][10] interventional studies, [11][12][13] quality metrics, 14 guidelines and recommendations, 15,16 and an awareness campaign by the Child Neurology Foundation. 3 Although WS is defined by developmental delay, epileptic spasms, and hypsarrhythmia on electroencephalography (EEG), 17,18 the epileptic spasms are the focus of diagnostic and therapy recommendations because hypsarrhythmia may not be present and development may initially be normal.…”

Section: Introductionmentioning

confidence: 99%

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Evolution and course of early life developmental encephalopathic epilepsies: Focus on Lennox‐Gastaut syndrome

2018

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Objectives: Developmental encephalopathic epilepsies (DEE) are characterized by refractory seizures, disability, and early death. Opportunities to improve care and outcomes focus on West Syndrome/infantile spasms (WS/IS). Lennox-Gastaut syndrome (LGS) is almost as common but receives little attention. We examined initial presentations of DEEs and their evolution over time to identify risk and indicators of developing LGS. Methods: Data are from the Connecticut Study of Epilepsy, a prospective, longitudinal study of 613 children with newly-diagnosed epilepsy recruited in 1993–1997. Central review of medical records permitted classification of epilepsy syndromes at diagnosis and reclassification 2, 5, and 9 years later. DEEs were compared to other epilepsies for seizure and cognitive outcomes, and mortality. Analyses examined the evolution of DEE syndromes after initial presentation with specific comparisons made between WS/IS and LGS. Statistical analyses were performed with t-tests and chi-squares. Results: Fifty-eight (9.4%) had DEEs, median onset age=1.1y (interquartile range (IQR)= 0.3, 1.3) in DEEs and 6.0y (IQR=3.0, 9.0) in other epilepsies (p<0.001). DEEs versus other epilepsies had more pharmacoresistance (71% versus 18%), intellectual disability (84% versus 11%), and mortality (21% versus <1%; all p<0.001). During follow-up, the form of epilepsy evolved in 33 children. WS/IS was the most common initial diagnosis (N=23) and 5 children later evolved to WS/IS. LGS was diagnosed initially in 4 children (1 later revised) and in 22 by the end of follow-up, including 7 evolving from WS/IS and 12 from nonsyndromic generalized, focal or undetermined epilepsies. Evolution to LGS took a median of 1.9 years. LGS developed in 13% of infants, including 9% of those who did not initially present with WS/IS. Significance: DEEs account for disproportionate amounts of pharmacoresistance, disability, and early mortality. LGS often has a window between initial presentation and full expression. LGS, should become targeted for early detection and prevention.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Evolution and course of early life developmental encephalopathic epilepsies: Focus on Lennox‐Gastaut syndrome

2018

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“…13,[24][25][26] Thus, consensus quality improvement guidelines strongly recommend evaluation with prolonged EEG and treatment initiation as soon as possible, certainly within 1 to 2 weeks of presentation. [27][28][29][30] Take a Step-Wise Approach to the Etiologic Investigation-Opportunity 3…”

Section: Epileptic Spasm (Es)mentioning

confidence: 99%

“…Currently, adrenocorticotropic hormone (ACTH), oral corticosteroids (OCS), and vigabatrin are the only identified "possibly" or "probably effective" therapies for IS (►Table 5). 28,30,37 Importantly, comparing treatment efficacy has been confounded by evolving dosing regimens and outcome measures, and most guidelines were published prior to several recent clinical studies. Although the 2012 American Academy of Neurology/Child Neurology Society guidelines found insufficient evidence to support short-term efficacy of OCS, they agreed with the ILAE consensus and Cochrane review that hormonal treatment with either ACTH or OCS improved long-term outcomes compared with vigabatrin, particularly for children with an unknown etiology.…”

Section: Offer An Effective Standard Treatment-opportunitymentioning

confidence: 99%

Infantile Spasms: Opportunities to Improve Care

Messer

Knupp

2020

Semin Neurol

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Infantile spasm (IS) is a distinct epilepsy syndrome characterized by epileptic spasms (the clinical seizure type) and hypsarrhythmia (the electrographic abnormality). IS is frequently accompanied by impaired neurodevelopment and is often associated with structural, genetic, or metabolic etiologies. Prompt treatment of this severe epileptic encephalopathy improves long-term outcomes but remains elusive in many situations. Despite common misconceptions, even patients with identified etiologies or preexisting developmental delay benefit from proven standard therapies, including adrenocorticotropic hormone (ACTH), oral corticosteroids, or vigabatrin. Treatment efficacy should be assessed with electroencephalography at 2 weeks, and an alternative therapy is indicated if epileptic spasms or hypsarrhythmia have not resolved. Collaboration with primary care providers is critical to mitigate the potentially serious adverse effects of standard treatments and also to provide developmental interventions. Although new approaches are on the horizon, addressing current challenges and opportunities now can dramatically improve patient outcomes.

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Hospital readmissions in children with new‐onset infantile epileptic spasms syndrome

et al. 2023

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Objective To describe inpatient resource use in the 2 years following infantile epileptic spasms syndrome (IESS) diagnosis, examine the association between clinical/demographic variables and incidence of readmission, and identify risk factors/reasons for frequent readmissions. Methods Retrospective cohort analysis of readmissions (scheduled/unscheduled) within the first 2 years following IESS diagnosis, details of readmissions (number/time between rehospitalizations, and length of stay), demographic/clinical variables, and reasons for readmissions were collected. Negative binomial regression analysis evaluated associations between incidence of readmissions (both scheduled/unscheduled and unscheduled alone) and demographic/clinical factors. Logistic regression assessed the risk of having recurrent readmissions (≥5 readmissions). Results Among 93 (60% males) new‐onset IESS patients, there were 394 readmissions (56% scheduled and 44% unscheduled) within 2‐years following IESS diagnosis. Mean length of stay was 3.5 days (SD: 5.9). Readmissions occurred in 82 patients (88%) and 37 (40%) experienced ≥5 readmissions. On multivariate regression analysis, readmissions were increased with use of multiple first‐line treatments for IESS (P = 0.006), technology assistance (P ≤ 0.001), and multispecialty care (P = 0.01); seizure freedom (P = 0.015) and known etiology (P = 0.011) lowered the incidence of readmissions. Examining unscheduled readmissions separately, increased readmissions occurred with public insurance (P = 0.013), technology use (P ≤ 0.0.001), and multispecialty care (P = 0.013); seizure freedom decreased unscheduled readmissions (P = 0.006). Technology assistance (G‐tube, NG tube, VP shunt, and tracheostomy use) increased the odds (P = 0.007) for recurrent readmissions. Reasons for readmissions included EEG monitoring (protocol driven for verification of IESS remission/characterization of events/EEG surveillance/presurgical monitoring) (51%), acute medical issues (21%), and seizure exacerbation (15%). Protocol‐driven readmissions declined an estimated 52% following protocol modification during the study. Significance In the 2 years following IESS diagnosis, there is substantial inpatient resource use with nearly 40% experiencing ≥5 readmissions (mostly epilepsy related). Since readmissions are increased by intrinsic patient characteristics such as medical complexity (technology use and multispecialty care) or epilepsy‐related issues, the preventability of readmissions is uncertain, except for protocol‐driven ones.

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Quality improvement in neurology: Child neurology quality measure set

Cited by 42 publications

References 38 publications

Evolution and course of early life developmental encephalopathic epilepsies: Focus on Lennox‐Gastaut syndrome

Evolution and course of early life developmental encephalopathic epilepsies: Focus on Lennox‐Gastaut syndrome

Infantile Spasms: Opportunities to Improve Care

Hospital readmissions in children with new‐onset infantile epileptic spasms syndrome

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