TY - JOUR
T1 - New-onset refractory status epilepticus
T2 - A retrospective cohort study
AU - Gugger, James J.
AU - Husari, Khalil
AU - Probasco, John C.
AU - Cervenka, Mackenzie C.
N1 - Funding Information:
Dr. Cervenka receives grants from Nutricia, Vitaflo, BrightFocus Foundation, and Army Research Laboratory. Honoraria from American Epilepsy Society, The Neurology Center, Epigenix, LivaNova, and Nutricia. Royalties from Demos. Consulting for Nutricia, Sage Therapeutics.
Publisher Copyright:
© 2019 British Epilepsy Association
PY - 2020/1
Y1 - 2020/1
N2 - Purpose: To describe the clinical, laboratory, neuroimaging, electroencephalographic features, etiology, treatment, as well as short-term and long-term outcomes of adults with new-onset refractory status epilepticus (NORSE). Method: A retrospective, single institution cohort study (2010–2018) of consecutive adult patients with NORSE. Results: Among 20 patients with NORSE, nine (45 %) had prodromal febrile illness, 12 (60 %) had evidence of inflammation on CSF profile. Six patients (30 %) met criteria for definite autoimmune encephalitis (AE) while 8 patients (40 %) had probable AE. Eleven out of 13 (85 %) patients had an abnormal FDG-PET scan with the most common finding being regional hypermetabolism. Fourteen patients (70 %) received immunotherapy and ten (50 %) received the ketogenic diet (KD). Fifteen patients (75 %) progressed to super-refractory status epilepticus (SRSE) and seven patients (35 %) died in the hospital or within six months of discharge. Among the surviving patients, eight (40 %) had a good outcome (i.e., modified Rankin Scale score 0–2); 12 (80 %) received a diagnosis of epilepsy of which nine (75 %) developed drug-resistant epilepsy. Conclusions: New-onset refractory status epilepticus is a syndrome associated with multiple complications, high mortality, and subsequent intractable epilepsy. There are multiple causes, some of which are autoimmune encephalitides; however, in this series the majority of patients had no clear etiology identified after extensive evaluation. Prospective studies are needed to determine optimal evaluation and treatment.
AB - Purpose: To describe the clinical, laboratory, neuroimaging, electroencephalographic features, etiology, treatment, as well as short-term and long-term outcomes of adults with new-onset refractory status epilepticus (NORSE). Method: A retrospective, single institution cohort study (2010–2018) of consecutive adult patients with NORSE. Results: Among 20 patients with NORSE, nine (45 %) had prodromal febrile illness, 12 (60 %) had evidence of inflammation on CSF profile. Six patients (30 %) met criteria for definite autoimmune encephalitis (AE) while 8 patients (40 %) had probable AE. Eleven out of 13 (85 %) patients had an abnormal FDG-PET scan with the most common finding being regional hypermetabolism. Fourteen patients (70 %) received immunotherapy and ten (50 %) received the ketogenic diet (KD). Fifteen patients (75 %) progressed to super-refractory status epilepticus (SRSE) and seven patients (35 %) died in the hospital or within six months of discharge. Among the surviving patients, eight (40 %) had a good outcome (i.e., modified Rankin Scale score 0–2); 12 (80 %) received a diagnosis of epilepsy of which nine (75 %) developed drug-resistant epilepsy. Conclusions: New-onset refractory status epilepticus is a syndrome associated with multiple complications, high mortality, and subsequent intractable epilepsy. There are multiple causes, some of which are autoimmune encephalitides; however, in this series the majority of patients had no clear etiology identified after extensive evaluation. Prospective studies are needed to determine optimal evaluation and treatment.
KW - Autoimmune encephalitis
KW - FDG-PET
KW - Ketogenic diet
KW - New-onset refractory status epilepticus
KW - Status epilepticus
UR - http://www.scopus.com/inward/record.url?scp=85075981463&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85075981463&partnerID=8YFLogxK
U2 - 10.1016/j.seizure.2019.12.002
DO - 10.1016/j.seizure.2019.12.002
M3 - Article
C2 - 31830676
AN - SCOPUS:85075981463
SN - 1059-1311
VL - 74
SP - 41
EP - 48
JO - Seizure
JF - Seizure
ER -