TY - JOUR
T1 - Review and standard operating procedures for collection of biospecimens and analysis of biomarkers in new onset refractory status epilepticus
AU - Hanin, Aurélie
AU - Cespedes, Jorge
AU - Pulluru, Yashwanth
AU - Gopaul, Margaret
AU - Aronica, Eleonora
AU - Decampo, Danielle
AU - Helbig, Ingo
AU - Howe, Charles L.
AU - Huttner, Anita
AU - Koh, Sookyong
AU - Navarro, Vincent
AU - Taraschenko, Olga
AU - Vezzani, Annamaria
AU - Wilson, Michael R.
AU - Xian, Julie
AU - Gaspard, Nicolas
AU - Hirsch, Lawrence J.
N1 - Publisher Copyright:
© 2023 International League Against Epilepsy.
PY - 2023/6
Y1 - 2023/6
N2 - New onset refractory status epilepticus (NORSE), including its subtype with a preceding febrile illness known as febrile infection-related epilepsy syndrome (FIRES), is one of the most severe forms of status epilepticus. The exact causes of NORSE are currently unknown, and there is so far no disease-specific therapy. Identifying the underlying pathophysiology and discovering specific biomarkers, whether immunologic, infectious, genetic, or other, may help physicians in the management of patients with NORSE. A broad spectrum of biomarkers has been proposed for status epilepticus patients, some of which were evaluated for patients with NORSE. Nonetheless, none has been validated, due to significant variabilities in study cohorts, collected biospecimens, applied analytical methods, and defined outcome endpoints, and to small sample sizes. The NORSE Institute established an open NORSE/FIRES biorepository for health-related data and biological samples allowing the collection of biospecimens worldwide, promoting multicenter research and sharing of data and specimens. Here, we suggest standard operating procedures for biospecimen collection and biobanking in this rare condition. We also propose criteria for the appropriate use of previously collected biospecimens. We predict that the widespread use of standardized procedures will reduce heterogeneity, facilitate the future identification of validated biomarkers for NORSE, and provide a better understanding of the pathophysiology and best clinical management for these patients.
AB - New onset refractory status epilepticus (NORSE), including its subtype with a preceding febrile illness known as febrile infection-related epilepsy syndrome (FIRES), is one of the most severe forms of status epilepticus. The exact causes of NORSE are currently unknown, and there is so far no disease-specific therapy. Identifying the underlying pathophysiology and discovering specific biomarkers, whether immunologic, infectious, genetic, or other, may help physicians in the management of patients with NORSE. A broad spectrum of biomarkers has been proposed for status epilepticus patients, some of which were evaluated for patients with NORSE. Nonetheless, none has been validated, due to significant variabilities in study cohorts, collected biospecimens, applied analytical methods, and defined outcome endpoints, and to small sample sizes. The NORSE Institute established an open NORSE/FIRES biorepository for health-related data and biological samples allowing the collection of biospecimens worldwide, promoting multicenter research and sharing of data and specimens. Here, we suggest standard operating procedures for biospecimen collection and biobanking in this rare condition. We also propose criteria for the appropriate use of previously collected biospecimens. We predict that the widespread use of standardized procedures will reduce heterogeneity, facilitate the future identification of validated biomarkers for NORSE, and provide a better understanding of the pathophysiology and best clinical management for these patients.
KW - biomarkers
KW - biospecimens
KW - new onset refractory status epilepticus
KW - standard operating procedures
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U2 - 10.1111/epi.17600
DO - 10.1111/epi.17600
M3 - Article
AN - SCOPUS:85152701067
SN - 0013-9580
VL - 64
SP - 1444
EP - 1457
JO - Epilepsia
JF - Epilepsia
IS - 6
ER -