Clinical presentation of new onset refractory status epilepticus in children (the pSERG cohort)

被引：27

作者：

Sculier, Claudine ^{[1
,2
]}

Barcia Aguilar, Cristina ^{[1
,3
]}

Gaspard, Nicolas ^{[2
,4
]}

Gainza-Lein, Marina ^{[1
,5
,6
]}

Sanchez Fernandez, Ivan ^{[1
,7
]}

Amengual-Gual, Marta ^{[1
,8
]}

Anderson, Anne ^{[9
]}

Arya, Ravindra ^{[10
]}

Burrows, Brian T. ^{[11
,12
]}

Brenton, James N. ^{[13
]}

Carpenter, Jessica L. ^{[14
]}

Chapman, Kevin E. ^{[15
,16
]}

Clark, Justice ^{[1
]}

Gaillard, William D. ^{[14
]}

Glauser, Tracy A. ^{[10
]}

Goldstein, Joshua L. ^{[17
]}

Goodkin, Howard P. ^{[13
]}

Gorman, Mark ^{[1
]}

Lai, Yi-Chen ^{[9
]}

McDonough, Tiffani L. ^{[18
]}

Mikati, Mohamad A. ^{[19
]}

Nayak, Anuranjita ^{[9
]}

Peariso, Katrina ^{[10
]}

Riviello, James ^{[9
]}

Rusie, Allison ^{[16
]}

Sperberg, Katherine ^{[14
]}

Stredny, Coral M. ^{[1
]}

Tasker, Robert C. ^{[20
]}

Tchapyjnikov, Dmitry ^{[18
]}

Vasquez, Alejandra ^{[1
,21
]}

Wainwright, Mark S. ^{[22
]}

Wilfong, Angus A. ^{[23
]}

Williams, Korwyn ^{[23
]}

Loddenkemper, Tobias ^{[1
]}

机构：

[1] Harvard Med Sch, Boston Childrens Hosp, Div Epilepsy & Clin Neurophysiol, Dept Neurol, Boston, MA 02115 USA

[2] Free Univ Brussels, Erasmus Hosp, Dept Neurol, Brussels, Belgium

[3] Autonomous Univ Madrid, La Paz Univ Hosp, Dept Child Neurol, Madrid, Spain

[4] Yale Univ Sch Med, Neurol Dept, Comprehens Epilepsy Ctr, New Haven, CT USA

[5] Univ Austral Chile, Inst Pediat, Fac Med, Valdivia, Chile

[6] Univ Chile, San Borja Arriaran Clin Hosp, Childrens Neuropsychiat Serv, Santiago, Chile

[7] Univ Barcelona, SJD Barcelona Childrens Hosp, Dept Child Neurol, Barcelona, Spain

[8] Univ Balearic Isl, Son Espases Univ Hosp, Dept Pediat, Pediat Neurol Unit, Palma De Mallorca, Spain

[9] Baylor Coll Med, Dept Pediat, Sect Neurol & Dev Neurosci, Houston, TX 77030 USA

[10] Univ Cincinnati Coll Med, Cincinnati Childrens Hosp Med Ctr, Dept Pediat, Div Pediat Neurol, Cincinnati, OH USA

[11] Univ Arizona Sch Med, Phoenix Childrens Hosp, Barrows Neurol Inst, Dept Pediat, Phoenix, AZ USA

[12] Mayo Clin, Dept Neurol, Scottsdale, AZ USA

[13] Univ Virginia Hlth Syst, Dept Neurol & Pediat, Charlottesville, VA USA

[14] George Washington Univ, Sch Med & Hlth Sci, Natl Childrens Hosp, Ctr Neurosci, Washington, DC 20052 USA

[15] Univ Colorado Sch Med, Dept Pediat, Aurora, CO USA

[16] Univ Colorado Sch Med, Dept Neurol, Aurora, CO USA

[17] Northwestern Univ Feinberg, Sch Med, Ruth D Ken M Davee Pediat Neurocrit Care Program, Chicago, IL USA

[18] Northwestern Univ Feinberg Sch Med, Ann & Robert H Lurie Childrens Hosp Chicago, Dept Pediat, Div Neurol & Epilepsy, Chicago, IL USA

[19] Duke Univ Med Ctr, Duke Univ, Div Pediat Neurol, Durham, NC USA

[20] Harvard Med Sch, Boston Childrens Hosp, Dept Anesthesiol, Dept Neurol, Boston, MA 02115 USA

[21] Mayo Clin, Mayo Clin Sch Med, Div Child & Adolescent Neurol, Rochester, MN USA

[22] Univ Washington, Dept Neurol, Div Pediat Neurol, Seattle, WA 98195 USA

[23] Univ Arizona Coll Med, Phoenix Childrens Hosp, Dept Child Hlth, Div Pediat Neurol, Phoenix, AZ USA

来源：

EPILEPSIA | 2021年 / 62卷 / 07期

关键词：

clinical neurology; epilepsy; febrile infection-related epilepsy syndrome; new onset refractory status epilepticus; pediatric; refractory status epilepticus; status epilepticus; EPILEPSY SYNDROME FIRES; CLAUSTRUM DAMAGE; ENCEPHALOPATHY; CHILDHOOD; NORSE; MULTICENTER; MORTALITY; SEIZURES; OUTCOMES;

D O I：

10.1111/epi.16950

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Objective We aimed to characterize the clinical profile and outcomes of new onset refractory status epilepticus (NORSE) in children, and investigated the relationship between fever onset and status epilepticus (SE). Methods Patients with refractory SE (RSE) between June 1, 2011 and October 1, 2016 were prospectively enrolled in the pSERG (Pediatric Status Epilepticus Research Group) cohort. Cases meeting the definition of NORSE were classified as "NORSE of known etiology" or "NORSE of unknown etiology." Subgroup analysis of NORSE of unknown etiology was completed based on the presence and time of fever occurrence relative to RSE onset: fever at onset (<= 24 h), previous fever (2 weeks-24 h), and without fever. Results Of 279 patients with RSE, 46 patients met the criteria for NORSE. The median age was 2.4 years, and 25 (54%) were female. Forty (87%) patients had NORSE of unknown etiology. Nineteen (48%) presented with fever at SE onset, 16 (40%) had a previous fever, and five (12%) had no fever. The patients with preceding fever had more prolonged SE and worse outcomes, and 25% recovered baseline neurological function. The patients with fever at onset were younger and had shorter SE episodes, and 89% recovered baseline function. Significance Among pediatric patients with RSE, 16% met diagnostic criteria for NORSE, including the subcategory of febrile infection-related epilepsy syndrome (FIRES). Pediatric NORSE cases may also overlap with refractory febrile SE (FSE). FIRES occurs more frequently in older children, the course is usually prolonged, and outcomes are worse, as compared to refractory FSE. Fever occurring more than 24 h before the onset of seizures differentiates a subgroup of NORSE patients with distinctive clinical characteristics and worse outcomes.

引用

页码：1629 / 1642

页数：14

共 40 条

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